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Elhalaby I, Koura O, Elhalaby R, Zeina W, Shareef M, Elhalaby E. Inflammatory myofibroblastic tumors of the colon in pediatrics: clinical presentation, management, and outcomes-A case report and systematic review of literature. Int J Colorectal Dis 2025; 40:94. [PMID: 40234278 PMCID: PMC12000112 DOI: 10.1007/s00384-025-04869-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 03/20/2025] [Indexed: 04/17/2025]
Abstract
PURPOSE Inflammatory myofibroblastic tumors (IMTs) of the colon represent an exceptionally rare entity in the pediatric population. This systematic review aims to comprehensively analyze the clinical presentation, diagnostic workup, management strategies, and outcomes of colorectal IMTs in children. METHODS A systematic literature review was conducted across multiple electronic databases (inception to January 2025), including MEDLINE (via PubMed), Embase, Cochrane, Web of Science, and Google Scholar. Two independent reviewers screened abstracts, reviewed studies, and extracted data on all reported cases of colorectal IMTs in the pediatric population, including one previously unreported case from our institution. RESULTS Including our case, 53 pediatric patients with colorectal IMTs were identified from 39 studies. The mean age at diagnosis was 7 years (range: 5 months-17 years) with a slight female preponderance. The IMTs comprised a wide range of anatomic locations with rectum (27%) and ascending colon (24%) being the most common. Abdominal pain (54%), gastrointestinal bleeding (29%), and fever (21%) were the predominant symptoms. Anemia was the most common laboratory abnormality (62%). Surgical resection was the primary treatment modality in 98% of cases. After a mean follow-up of 38 months ( range: 3-181 months), the local recurrence rate was 11%, with no distant metastases reported. CONCLUSION Colorectal IMTs in children present diagnostic and therapeutic challenges. While complete surgical resection remains the gold standard treatment, emerging therapies such as ALK inhibitors and NSAIDs warrant further investigation. The potential for late recurrence mandates long term follow-up.
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Affiliation(s)
- Ismael Elhalaby
- Department of Surgery, Faculty of Medicine, Tanta University, Tanta, Egypt.
- Kenanah Children's Medical Center, Tanta, Egypt.
| | - Omar Koura
- Department of Surgery, Faculty of Medicine, Tanta University, Tanta, Egypt
- Kenanah Children's Medical Center, Tanta, Egypt
| | - Rofyda Elhalaby
- Department of Pathology, Faculty of Medicine, Tanta University, Tanta, Egypt
| | - Wael Zeina
- Kenanah Children's Medical Center, Tanta, Egypt
- Benha Children Hospital, Benha, Egypt
| | - Mohamed Shareef
- Department of Pathology, Faculty of Medicine, Tanta University, Tanta, Egypt
| | - Essam Elhalaby
- Department of Surgery, Faculty of Medicine, Tanta University, Tanta, Egypt
- Kenanah Children's Medical Center, Tanta, Egypt
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Lange A, Dominiak N, Petros FG. Rare Site of an Inflammatory Myofibroblastic Tumor at the Epididymis: A Case Report. Cancer Manag Res 2025; 17:193-196. [PMID: 39897092 PMCID: PMC11786596 DOI: 10.2147/cmar.s481940] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/16/2024] [Accepted: 01/13/2025] [Indexed: 02/04/2025] Open
Abstract
Background Inflammatory myofibroblastic tumors (IMT) are uncommon with an estimated 150-200 people being diagnosed in the US annually. We describe a healthy adult male who presented with an enlarging, painless scrotal mass. Based on current literature, only nine epididymal inflammatory myofibroblastic tumors have been reported and in this case report we describe the tenth. Case Presentation A 40-year-old Caucasian male presented with an enlarging mass near his right testicle with no associative symptoms or obvious etiology. Scrotal ultrasound showed a solid heterogenous mass with internal vascularity. Tumor markers were unremarkable for lactate dehydrogenase (LDH), beta-human chorionic gonadotropin (b-hCG), and alpha-fetoprotein (AFP). A right inguinal approach was performed. A 2-3 cm round mass adjacent to the tail of the epididymis was excised with clear margins and sent to pathology. Histology confirmed a 2.4 cm inflammatory myofibroblastic tumors with scattered positivity for smooth muscle actin, negative pancytokeratin, and negative anaplastic lymphoma kinase. Patient recovered well with no reoccurrence at this time. Conclusion Inflammatory myofibroblastic tumors recurrence rate is <2%; however, some literature suggests higher depending on location and immunohistochemical profile. The expertise of pathologists, utilization of morphology, and immunohistological profile are all crucial in accurate diagnoses of these lesions. Literature reports some lesions have demonstrated metastatic tendencies and therefore complete excision of the mass is the recommended therapy of choice. This case highlights the increasing need to include IMT in differential diagnoses for patients presenting with painless lumps even in unlikely locations. While there is little data on epididymal tail mass recurrence rate potential, we report no current recurrence after complete excision of the mass.
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Affiliation(s)
- Alyssa Lange
- Department of Urology, College of Medicine and Life Sciences, The University of Toledo, Toledo, OH, USA
| | - Nicole Dominiak
- Department of Pathology, College of Medicine and Life Sciences, The University of Toledo, Toledo, OH, USA
| | - Firas G Petros
- Department of Urology, College of Medicine and Life Sciences, The University of Toledo, Toledo, OH, USA
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3
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Amro AM, Almassri T, Albandak M, Elqadi M, Bannoura S, Asafrah AA, Abu Asbeh Y. Successful management of an unusual case of pediatric inflammatory myofibroblastic tumor: a case report and literature review. Ann Med Surg (Lond) 2025; 87:407-412. [PMID: 40109596 PMCID: PMC11918599 DOI: 10.1097/ms9.0000000000002870] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/31/2024] [Accepted: 12/03/2024] [Indexed: 03/22/2025] Open
Abstract
Introduction and importance Inflammatory myofibroblastic tumor (IMT) is a rare neoplastic condition that primarily affects children and young adults. This case report highlights the challenges in diagnosing and treating pediatric IMT, emphasizing the importance of tailored interventions. Case presentation An 8-year-old boy presented with respiratory symptoms and was diagnosed with an IMT located in the left main bronchus. Imaging studies revealed a soft tissue mass and lymph node enlargement. Bronchoscopy and biopsy confirmed the diagnosis. The patient underwent bronchoscopic debulking procedures followed by lobectomy and bronchoplasty due to persistent disease. Clinical discussion IMTs pose diagnostic challenges due to their varied clinical presentation and similarities with other neoplasms. A multidisciplinary approach involving pathologists, radiologists, and surgeons is crucial for accurate diagnosis and optimal treatment planning. The pathogenesis of IMTs is not fully understood, but theories suggest an inflammatory response or involvement of the ALK gene. IMTs can affect various organs, each with distinct symptoms. Imaging modalities lack specificity, emphasizing the importance of histopathological examination. Conclusion IMTs require individualized treatment approaches based on the location and extent of the tumor. Long-term follow-up is essential for monitoring recurrence and metastasis. Further research is needed to enhance our understanding of IMT biology and develop targeted therapies to improve patient outcomes. This case report underscores the importance of tailored interventions in pediatric IMT cases and highlights the challenges in diagnosis and treatment.
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Affiliation(s)
- Alhareth M Amro
- Medical Research Club, Faculty of Medicine, Al-Quds University, Jerusalem, Palestine
| | | | - Maram Albandak
- Medical Research Club, Faculty of Medicine, Al-Quds University, Jerusalem, Palestine
| | | | - Sami Bannoura
- Department of Pathology, Al-Ahli Hospital, Hebron, Palestine
| | - Anas A Asafrah
- Department of Thoracic Surgery, Al-Ahli Hospital, Affiliated to Al-Quds University School of Medicine, Hebron, Jerusalem, Palestine
| | - Yousef Abu Asbeh
- Department of Thoracic Surgery, Al-Ahli Hospital, Affiliated to Al-Quds University School of Medicine, Hebron, Jerusalem, Palestine
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Khurana E, Mody S, Shah T, Bouffard JP, Pedemonte M, Holover G, Lee JS, Jacob G, Scheid S, Morin R, Mazzola C. Pediatric skull inflammatory myofibroblastic tumor: a rare case report and literature review. Childs Nerv Syst 2024; 40:3829-3835. [PMID: 38918263 DOI: 10.1007/s00381-024-06512-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/16/2024] [Accepted: 06/19/2024] [Indexed: 06/27/2024]
Abstract
Inflammatory myofibroblastic tumors (IMTs) represent rare neoplasms, particularly infrequent in the pediatric skull. We present a novel case of a newborn male with a 5 cm right temporal mass and discuss current diagnostic and treatment options for IMTs. A multidisciplinary effort to surgically remove the lesion was successful, and the patient's skull defect healed without neurological deficits. The etiology of IMTs remains elusive, with proposed associations with chromosomal mutations in the anaplastic lymphoma kinase (ALK) gene. Surgical excision remains the primary treatment for IMTs. Promising pharmacological treatments, like Crizotinib, warrant further research into understanding potential alternatives in IMT management.
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Affiliation(s)
| | - Shaan Mody
- NJ Craniofacial Center, Morristown, NJ, 07960, USA
| | - Tanisha Shah
- NJ Craniofacial Center, Morristown, NJ, 07960, USA
| | | | - Maria Pedemonte
- Department of Pathology, Atlantic Health System, Summit, NJ, 07960, USA
| | | | | | - Gregg Jacob
- NJ Craniofacial Center, Morristown, NJ, 07960, USA
| | - Sara Scheid
- NJ Craniofacial Center, Morristown, NJ, 07960, USA
| | - Robert Morin
- NJ Craniofacial Center, Morristown, NJ, 07960, USA
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Maqbool N, Pal KMB, Uddin Z, Safdar F. Inflammatory myofibroblastic tumor: A rare cause of intestinal obstruction: Case report. Int J Surg Case Rep 2024; 124:110438. [PMID: 39437499 PMCID: PMC11532891 DOI: 10.1016/j.ijscr.2024.110438] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/19/2024] [Revised: 10/07/2024] [Accepted: 10/09/2024] [Indexed: 10/25/2024] Open
Abstract
INTRODUCTION AND IMPORTANCE Inflammatory myofibroblastic tumor IMT1 is a rare neoplasm with diverse clinical presentations, needing histological confirmation for diagnosis. The tumor often mimics malignant conditions, making accurate diagnosis challenging. CASE PRESENTATION We report a case of a 29-year-old male presenting with long standing recurrent abdominal pain, with more recent increasing frequency and associated weight loss. Imaging revealed a heterogenous soft tissue lesions causing mechanical small bowel obstruction, prompting surgical intervention. Histology analysis confirmed the diagnosis of IMT. CLINICAL DISCUSSION This case highlights the importance of considering IMT in the differential diagnosis of neoplastic mechanical small bowel obstruction, particularly in patients presenting with atypical symptoms and image findings. Despite its rarity, IMT should be considered, as it can present similarly to more common malignancies. Further research is warranted to unravel the pathogenesis and refine management strategies for this intriguing neoplasm. CONCLUSION IMT is a rare and intriguing neoplasm that presents significant diagnostic challenge. This case emphasize the need for thorough histological evaluation to confirm rhe diagnosis and guide appropriate management strategies.
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Affiliation(s)
- Nargis Maqbool
- Department of Surgery, Aga Khan University Hospital, Karachi, Pakistan.
| | | | - Zeeshan Uddin
- Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan
| | - Fatima Safdar
- Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan
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Tully A, Lee ACH, Gorton A, Turkyilmaz M, Podbielski F. Double diagnostic deviance: Case report of chest wall liposarcoma mimicking inflammatory myofibroblastic tumor mimicking costochondritis. SAGE Open Med Case Rep 2024; 12:2050313X241289031. [PMID: 39474027 PMCID: PMC11520007 DOI: 10.1177/2050313x241289031] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/19/2023] [Accepted: 09/18/2024] [Indexed: 01/03/2025] Open
Abstract
Inflammatory myofibroblastic tumor and liposarcoma very rarely present as tumors of the chest wall. Never have both been reported together in the same lesion. We present a case wherein a 72-year-old man with a rapidly progressing lesion initially mistaken for a local infection underwent resection with diagnosis of inflammatory myofibroblastic tumor. He experienced recurrence less than 6 months before radical resection revealed well to dedifferentiated liposarcoma with areas of inflammatory myofibroblastic tumor. He is now doing well with over 4 years of follow-up.
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Affiliation(s)
- Andy Tully
- University of Illinois at Chicago Chicago, IL USA
| | | | - Andy Gorton
- University of Illinois at Chicago Chicago, IL USA
| | | | - Francis Podbielski
- Adventist Hinsdale Hospital, Hinsdale, IL, USA
- Department of Surgery, Presence St. Joseph Hospital, Chicago, IL, USA
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7
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Lassiter S, Smith ML, Siegert J, Nguyen T. Inflammatory Myofibroblastic Tumor of the urinary bladder: A case report. Urol Case Rep 2024; 54:102726. [PMID: 38799732 PMCID: PMC11126942 DOI: 10.1016/j.eucr.2024.102726] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/05/2023] [Revised: 03/17/2024] [Accepted: 03/23/2024] [Indexed: 05/29/2024] Open
Abstract
Inflammatory Myofibroblastic Tumors represent a rare subset of spindle cell tumors that can occur in the genitourinary tract, most commonly within the bladder. These tumors are proposed to fall in a spectrum of benign inflammatory pseudotumors to true sarcomas. This, along with their rarity makes diagnosis and treatment challenging to clinicians. We present a 51-year-old female diagnosed with IMT of the bladder following a presentation for hematuria. Treatment consisted of transurethral resection of the tumor. This case, and the accompanying review of the literature highlight the need for further research due to lack of clarity for diagnosis and treatment.
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Karibe J, Teranishi J, Kawahara T, Noguchi T, Takeshima T, Osaka K, Kumagai E, Sawazumi T, Fujii S, Uemura H. A diagnostically challenging case of inflammatory myofibroblastic tumor primary to the peritoneum. IJU Case Rep 2024; 7:206-209. [PMID: 38686080 PMCID: PMC11056253 DOI: 10.1002/iju5.12701] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/12/2023] [Accepted: 01/24/2024] [Indexed: 05/02/2024] Open
Abstract
Introduction Inflammatory myofibroblastic tumors are difficult to diagnose because of the lack of specific indicators. We describe a diagnostically challenging case of an inflammatory myofibroblastic tumor primary to the peritoneum. Case presentation The patient was a 25-year-old male who presented at our hospital with lower abdominal pain. Computed tomography revealed a mass lesion 80 mm in diameter just above the bladder. This was suspected to be a bleeding tumor of the urachus. Since malignancy could not be ruled out, surgery was planned. This revealed a fragile tumor arising from the peritoneum. Following its removal, the tumor was diagnosed by histopathological analysis as an inflammatory myofibroblastic tumor. Conclusion We describe a case of inflammatory myofibroblastic tumor primary to the peritoneum diagnosed by histopathology. Inflammatory myofibroblastic tumor should be considered in the differential diagnosis of abdominal wall and anterior bladder tumors.
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Affiliation(s)
- Jurii Karibe
- Department of Urology and Renal TransplantationYokohama City University Medical CenterYokohamaJapan
| | - Jun‐ichi Teranishi
- Department of Urology and Renal TransplantationYokohama City University Medical CenterYokohamaJapan
| | - Takashi Kawahara
- Department of Urology and Renal TransplantationYokohama City University Medical CenterYokohamaJapan
| | - Takeaki Noguchi
- Department of Urology and Renal TransplantationYokohama City University Medical CenterYokohamaJapan
| | - Teppei Takeshima
- Department of Urology and Renal TransplantationYokohama City University Medical CenterYokohamaJapan
| | - Kimito Osaka
- Department of Urology and Renal TransplantationYokohama City University Medical CenterYokohamaJapan
| | - Eita Kumagai
- Department of PathologyYokohama City University Medical CenterYokohamaJapan
| | - Tomoe Sawazumi
- Department of PathologyYokohama City University Medical CenterYokohamaJapan
| | - Satoshi Fujii
- Department of PathologyYokohama City University Medical CenterYokohamaJapan
- Department of PathologyYokohama City University HospitalYokohamaJapan
- Department of Molecular PathologyYokohama City University Graduate School of MedicineYokohamaJapan
| | - Hiroji Uemura
- Department of Urology and Renal TransplantationYokohama City University Medical CenterYokohamaJapan
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Tong D, Chisholm J, Madden B, Ahmed M. Anaplastic lymphoma kinase-positive pulmonary inflammatory myofibroblastic tumour: a case report. J Med Case Rep 2024; 18:167. [PMID: 38594735 PMCID: PMC11005263 DOI: 10.1186/s13256-024-04472-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/14/2023] [Accepted: 02/23/2024] [Indexed: 04/11/2024] Open
Abstract
BACKGROUND Pulmonary inflammatory myofibroblastic tumour (IMT) is a rare condition that usually presents in young individuals and is associated with anaplastic lymphoma kinase (ALK)-translocation. CASE PRESENTATION We report a case of an 18-year-old Caucasian man with ALK-translocated pulmonary IMT treated with multimodality therapy. The patient presented with breathlessness and was found to have a collapsed left lung. Further investigations revealed an ALK-translocated pulmonary IMT. This is usually treated with an ALK-inhibitor but patient declined after discussing potential side-effects and had repeated rigid bronchoscopic interventions for local disease control. Due to persistent local recurrence, patient received radical external beam radiotherapy (EBRT) with pulse steroids, and one year later started on Ibuprofen, a non-steroidal anti-inflammatory agent (NSAID). Following multimodality treatment, he developed a complete response. He remains treatment-free for the past seven years. Eleven years on from his diagnosis, he remains in remission with a ECOG performance status of zero. CONCLUSIONS Achieving long-term local control in pulmonary IMT can be challenging. Multimodality treatment is sometimes needed but the overall outlook remains good.
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Affiliation(s)
- Daniel Tong
- Lung Unit, The Royal Marsden NHS Foundation Trust, Sutton, UK.
| | - Julia Chisholm
- Children and Young People's Unit, Institute of Cancer Research, Royal Marsden Hospital, Sutton, SM2 5NG, UK
| | - Brendan Madden
- Department of Cardiothoracic Medicine, St Georges Hospital, Blackshaw Road, London, SW17 0QT, UK
| | - Merina Ahmed
- Lung Unit, The Royal Marsden NHS Foundation Trust, Sutton, UK
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Hage L, O’Donnell MA, Abou Chakra M, Kime A, Sibony M, Peyromaure M, Duquesne I. Inflammatory myofibroblastic tumor of the urinary bladder: A systematic review of the literature and report of a case. Indian J Urol 2024; 40:88-95. [PMID: 38725889 PMCID: PMC11078447 DOI: 10.4103/iju.iju_50_24] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/30/2024] [Revised: 03/07/2024] [Accepted: 03/08/2024] [Indexed: 05/12/2024] Open
Abstract
Introduction Inflammatory myofibroblastic tumors (IMTs) are intermediate-grade lesions that frequently recur and rarely metastasize. There are currently no guidelines on the management of bladder IMTs. This systematic review aims to describe the clinical presentation and compare the management options for bladder IMTs. Methods A PubMed/Medline search was conducted, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, using the following Mesh terms: ("inflammatory myofibroblastic") AND ("tumor") OR ("tumor") AND ("bladder") AND ("case report"). A total of 75 case reports were included in the analysis. Results The mean age of the patients was 36 years. 65% of the cases initially presented with hematuria. 68% of the tumors stained positive for anaplastic lymphoma kinase, and 20% invaded the muscularis. Patients underwent either transurethral resection of the bladder tumor (TURBT) only (34%), TURBT followed by complementary partial cystectomy (16%), or TURBT followed by radical cystectomy (4%). 36% and 9% of the cases underwent partial and radical cystectomy after the initial diagnosis, respectively. Cystectomies were performed using an open (74%), laparoscopic (14%), robotic-assisted (10%), or unknown (2%) approach. At a mean follow-up of 14 months, the recurrence and metastasis rates were about 9% and 4%, respectively. In addition, we present the case of a 49-year-old woman with a bladder IMT who underwent TURBT followed by laparoscopic partial cystectomy. The patient remains tumor free postoperatively (follow-up period of 12 months). Conclusion A complete surgical excision of the bladder IMT is crucial for the optimal management of these cases. Proper differentiation of this tumor from sarcoma or leiomyosarcoma leads to the best outcomes.
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Affiliation(s)
- Lory Hage
- Department of Urology, Cochin Hospital, Assistance Publique-Hopitaux de Paris, Universite Paris Cite, Paris, France
| | - Michael A O’Donnell
- Department of Urology, The University of Iowa Hospitals and Clinics, Iowa City, IA, USA
| | - Mohamad Abou Chakra
- Department of Urology, The University of Iowa Hospitals and Clinics, Iowa City, IA, USA
| | - Amel Kime
- Department of Pathology, Cochin Hospital, Assistance Publique-Hopitaux de Paris, Universite Paris Cite, Paris, France
| | - Mathilde Sibony
- Department of Pathology, Cochin Hospital, Assistance Publique-Hopitaux de Paris, Universite Paris Cite, Paris, France
| | - Michaël Peyromaure
- Department of Urology, Cochin Hospital, Assistance Publique-Hopitaux de Paris, Universite Paris Cite, Paris, France
| | - Igor Duquesne
- Department of Urology, Cochin Hospital, Assistance Publique-Hopitaux de Paris, Universite Paris Cite, Paris, France
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Lim JJ, Chen EY, Schaub SK, Wagner MJ. Reclassification of a spindle cell sarcoma after identification of a TFG-ROS1 fusion: A case demonstrating the clinical benefit of next-generation sequencing in sarcoma. Mol Genet Genomic Med 2024; 12:e2423. [PMID: 38622850 PMCID: PMC11019117 DOI: 10.1002/mgg3.2423] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2023] [Revised: 02/14/2024] [Accepted: 03/19/2024] [Indexed: 04/17/2024] Open
Abstract
BACKGROUND Inflammatory myofibroblastic tumors (IMTs) are rare mesenchymal soft tissue sarcomas that often present diagnostic challenges due to their wide and varied morphology. A subset of IMTs have fusions involving ALK or ROS1. The role of next-generation sequencing (NGS) for classification of unselected sarcomas remains controversial. METHODS AND RESULTS We report a case of a metastatic sarcoma in a 34-year-old female originally diagnosed as an unclassified spindle cell sarcoma with myofibroblastic differentiation and later reclassified as IMT after NGS revealed a TFG-ROS1 rearrangement. Histologically, the neoplasm had spindle cell morphology with a lobulated to focally infiltrative growth pattern with scant inflammatory cell infiltrate. Immunohistochemistry demonstrated focal desmin and variable smooth muscle actin staining but was negative for SOX10, S100, and CD34. Fluorescence in situ hybridization was negative for USP6 or ALK gene rearrangements. NGS revealed a TFG-ROS1 rearrangement and the patient was treated with crizotinib with clinical benefit. CONCLUSIONS We discuss the role of NGS as well as its potential benefit in patients with unresectable, ALK-negative metastatic disease. Considering this case and previous literature, we support the use of NGS for patients requiring systemic treatment.
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Affiliation(s)
- John J. Lim
- Division of Medical OncologyUniversity of WashingtonSeattleWashingtonUSA
| | - Eleanor Y. Chen
- Department of Laboratory Medicine and PathologyUniversity of WashingtonSeattleWashingtonUSA
| | | | - Michael J. Wagner
- Division of Medical OncologyUniversity of WashingtonSeattleWashingtonUSA
- Clinical Research DivisionFred Hutchinson Cancer CenterSeattleWashingtonUSA
- Present address:
Center for Sarcoma and Bone OncologyDana Farber Cancer InstituteBostonMassachusettsUSA
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Watanabe R, Ano S, Kikuchi N, Saegusa M, Shigemasa R, Kondo Y, Hizawa N. Inflammatory myofibroblastic tumor directly invading the right first rib treated with oral steroids: a case report. BMC Pulm Med 2024; 24:67. [PMID: 38308319 PMCID: PMC10835977 DOI: 10.1186/s12890-024-02873-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/13/2023] [Accepted: 01/20/2024] [Indexed: 02/04/2024] Open
Abstract
BACKGROUND We present a case of an inflammatory myofibroblastic tumor cured with a short period of steroid administration, a treatment previously unreported for such cases. CASE PRESENTATION A 49-year-old man had a chief complaint of chest pain for more than 3 days. Computed tomography (CT) revealed a tumoral lesion suspected to have infiltrated into the right first rib and intercostal muscles, with changes in lung parenchymal density around the lesion. The maximal standardized uptake value on 18 F-fluorodeoxyglucose positron emission tomography/computed tomography was high (16.73), consistent with tumor presence. CT-guided biopsy revealed an inflammatory myofibroblastic tumor with no distant metastases. Surgery was indicated based on the disease course. However, he had received an oral steroid before the preoperative contrast-enhanced CT scan due to a history of bronchial asthma, and subsequent CT showed that the tumor shrank in size after administration; he has been recurrence-free for more than a year. CONCLUSIONS Surgery is still the first choice for inflammatory myofibroblastic tumors, as the disease can metastasize and relapse; however, this condition can also be cured with a short period of steroid therapy.
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Affiliation(s)
- Ryo Watanabe
- Department of Respiratory Medicine, National Hospital Organization Kasumigaura Medical Center, 2-7-14 Shimotakatsu, 300-8585, Tsuchiura, Ibaraki, Japan
| | - Satoshi Ano
- Department of Respiratory Medicine, National Hospital Organization Kasumigaura Medical Center, 2-7-14 Shimotakatsu, 300-8585, Tsuchiura, Ibaraki, Japan.
- Department of Respiratory Medicine, University of Tsukuba, Tsukuba, Japan.
| | - Norihiro Kikuchi
- Department of Respiratory Medicine, National Hospital Organization Kasumigaura Medical Center, 2-7-14 Shimotakatsu, 300-8585, Tsuchiura, Ibaraki, Japan
| | - Michiko Saegusa
- Department of Respiratory Medicine, National Hospital Organization Kasumigaura Medical Center, 2-7-14 Shimotakatsu, 300-8585, Tsuchiura, Ibaraki, Japan
| | - Rie Shigemasa
- Department of Respiratory Medicine, National Hospital Organization Kasumigaura Medical Center, 2-7-14 Shimotakatsu, 300-8585, Tsuchiura, Ibaraki, Japan
| | - Yuzuru Kondo
- Department of Diagnostic Pathology, National Hospital Organization Kasumigaura Medical Center, Tsuchiura, Japan
| | - Nobuyuki Hizawa
- Department of Respiratory Medicine, University of Tsukuba, Tsukuba, Japan
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Wang QA, Chen HW, Wu RC, Wu CE. Update of Diagnosis and Targeted Therapy for ALK + Inflammation Myofibroblastic Tumor. Curr Treat Options Oncol 2023; 24:1683-1702. [PMID: 37938503 PMCID: PMC10781869 DOI: 10.1007/s11864-023-01144-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/08/2023] [Indexed: 11/09/2023]
Abstract
OPINION STATEMENT: Inflammatory myofibroblastic tumor (IMT), characterized by intermediate malignancy and a propensity for recurrence, has presented a formidable clinical challenge in diagnosis and treatment. Its pathological characteristics may resemble other neoplasms or reactive lesions, and the treatment was limited, taking chemotherapies as the only option for those inoperable. However, discovering anaplastic lymphoma kinase (ALK) protein expression in approximately 50% of IMT cases has shed light on a new diagnostic approach and application of targeted therapies. With the previous success of combating ALK+ non-small-cell lung cancers with ALK tyrosine kinase inhibitors (TKIs), crizotinib, a first-generation ALK-TKI, was officially approved by the U.S. Food and Drug Administration in 2020, to treat unresectable ALK+ IMT. After the approval of crizotinib, other ALK-TKIs, such as ceritinib, alectinib, brigatinib, and lorlatinib, have proven their efficacy on ALK+ IMT with sporadic case reports. The sequential treatments of targeted therapies in may provide the insight into the choice of ALK-TKIs in different lines of treatment for unresectable ALK+ IMT.
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Affiliation(s)
- Qi-An Wang
- School of Medicine, College of Medicine, Chang Gung University, Taoyuan, Taiwan
| | - Huan-Wu Chen
- Division of Emergency and Critical Care Radiology, Department of Medical Imaging and Intervention, Chang Gung Memorial Hospital, Chang Gung University, Linkou, Taiwan
| | - Ren-Chin Wu
- Department of Pathology, Chang Gung Memorial Hospital at Linkou and Chang Gung University, Taoyuan, Taiwan
| | - Chiao-En Wu
- Division of Hematology-Oncology, Department of Internal Medicine, Chang Gung Memorial Hospital at Linkou, Chang Gung University College of Medicine, Taoyuan, Taiwan.
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14
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Kavirayani V, Pai NG, Nayal B, Prabhu S. Infantile inflammatory myofibroblastic tumour of the sigmoid colon: a diagnostic dilemma. BMJ Case Rep 2023; 16:e256505. [PMID: 37832973 PMCID: PMC10583037 DOI: 10.1136/bcr-2023-256505] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/15/2023] Open
Abstract
An inflammatory myofibroblastic tumour (IMT) is an uncommon neoplasm composed of inflammatory cells and myofibroblasts in a fibrous stroma. They are mostly seen in the lungs and rarely involve the gastrointestinal tract. An 8-month-old infant presented with a history of lower abdominal lump for 2 months. Her CT scan confirmed a large, lobulated mass in the retroperitoneum arising from the pelvis. The mass was found to be arising from the sigmoid colon on laparotomy which was excised. Histopathology showed a cellular tumour composed of spindle cells and inflammatory lymphocytic infiltrate. Immunohistochemistry revealed positive staining for anaplastic lymphoma kinase and smooth muscle actin, confirming the diagnosis of IMT. The patient is doing well at her 6-month follow-up. Ours is the youngest case of sigmoid IMT among the only other series of eight cases reported in the literature indicating its rarity.
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Affiliation(s)
- Vaishnavi Kavirayani
- Pediatric Surgery, Kasturba Medical College Manipal, Manipal academy of Higher education, Manipal, Karnataka, India
| | - Nitin G Pai
- Pediatric Surgery, Kasturba Medical College Manipal, Manipal academy of Higher education, Manipal, Karnataka, India
| | - Bhavna Nayal
- Pathology, Kasturba Medical College Manipal, Manipal academy of Higher education, Manipal, Karnataka, India
| | - Santosh Prabhu
- Pediatric Surgery, Kasturba Medical College Manipal, Manipal academy of Higher education, Manipal, Karnataka, India
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15
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Liu X, Duan Y, Wang G, Zhu P. A novel TPD52L2-ROS1 gene fusion expanding the molecular alterations in inflammatory myofibroblastic tumor: case report and literature review. Diagn Pathol 2023; 18:105. [PMID: 37735390 PMCID: PMC10512592 DOI: 10.1186/s13000-023-01382-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/19/2022] [Accepted: 08/13/2023] [Indexed: 09/23/2023] Open
Abstract
BACKGROUND Inflammatory myofibroblastic tumor (IMT) is a distinctive tumor composed of spindle cells accompanied by mixed inflammatory cells, and immunohistochemical positivity for ALK (anaplastic lymphoma kinase protein) can be detected in half of IMTs. The diagnosis of ALK-negative IMT could be a challenge. Recently, the fusions of some kinase genes, such as RET, NTRK1, ROS1, etc., are revealed in ALK-negative IMT. CASE PRESENTATION A 19-year-old woman presented with swelling of the left upper arm. Magnetic resonance imaging (MRI) scan revealed a tumor in the left postbrachium extended to the left axillary, serratus anterior muscle, and latissimus dorsi muscle. Histopathologically, the irregular-circumscribed tumor was composed of dense spindle-shaped cells with eosinophilic abundant cytoplasm and hyalinized mesenchyme in an inflammatory background. Immunohistochemically (IHC), tumor cells were positive for SMA, MDM2, and p16; the cells were negative for desmin, MyoD1, Myogenin, pan-cytokeratin, S100, SOX10, HMB45, Malen-A, CD34, CD31, CD99, and ALK. By RNA-based NGS, a novel fusion between TPD52L2 3' end of exon 1-4 and ROS1 5' end of exon 36-43 was revealed. ROS1 IHC staining was negative. The final diagnosis of IMT with TPD52L2-ROS1 fusion was made. Subsequently, the patient experienced a good clinical response to Crizotinib, and clinical follow-up showed stable disease after 9 months. CONCLUSION This report expands the spectrum of ROS1 gene rearrangements in the IMT and highlights the importance of molecular analysis of IMT for getting a diagnostic clue and determining potential therapeutic strategies.
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Affiliation(s)
- Xuguang Liu
- Department of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China
| | - Yaqi Duan
- Department of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China
- Department of Pathology, School of Basic Medical Science, Tongji Medical College, Huazhong University of Science and Technology, No. 13 Hangkong Road, Wuhan, 430030, China
| | - Guoping Wang
- Department of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China
- Department of Pathology, School of Basic Medical Science, Tongji Medical College, Huazhong University of Science and Technology, No. 13 Hangkong Road, Wuhan, 430030, China
| | - Pengcheng Zhu
- Department of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
- Department of Pathology, School of Basic Medical Science, Tongji Medical College, Huazhong University of Science and Technology, No. 13 Hangkong Road, Wuhan, 430030, China.
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16
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Chandramouli R, Sowmya YS, Raghava Ks A, Gochhait D, Jagadesan P. Inflammatory Myofibroblastic Tumor of the Testis in a Patient With Cecal Carcinoma. Cureus 2023; 15:e44573. [PMID: 37662511 PMCID: PMC10474877 DOI: 10.7759/cureus.44573] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/02/2023] [Indexed: 09/05/2023] Open
Abstract
Inflammatory myofibroblastic tumors (IMTs) are uncommon tumors that can be classified among fibroinflammatory disorders. IMTs are diagnosed after excluding all other entities, which can be considered differential diagnoses of IMTs. Microscopic examination of IMTs shows multiple myofibroblastic spindle cells, which are surrounded by inflammation. IMTs have lesser chances of progression to malignancy. The case defined below is one of the few cases in the literature that reports the presentation of IMT and another malignancy. We describe a 72-year-old man who was found to have cecal carcinoma and later diagnosed with an IMT of the testis. IMTs are generally benign tumors with a tendency for local recurrence. Patients affected by IMTs usually get diagnosed only after more than one biopsy. IMT is diagnosed only after ruling out other differential diagnoses. They rarely show invasiveness and metastasize. The presence of metastasis, recurrence, and other malignancies probably indicate poor prognosis and poor survival. The course of IMTs is usually benign, with good outcomes after surgery. IMTs have been known to recur, invade, or metastasize in sites such as paranasal sinuses, mediastinum, and the abdomen. In the case that we researched, vimentin and smooth muscle actin were strongly positive in the spindle-shaped cells, whereas anaplastic lymphoma kinase-1 was negative.
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Affiliation(s)
- R Chandramouli
- Radiation Oncology, Krishna Cancer Institute, Cuddalore, IND
| | - Y Sree Sowmya
- Radiation Oncology, Great Eastern Medical School and Hospital, Ragolu, IND
| | | | - Debasis Gochhait
- Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND
| | - Pandjatcharam Jagadesan
- Radiation Oncology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND
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17
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Nguyen H, Pham DH, Luong TH. Successful surgical management of an aggressive IgG4-related sclerosing esophageal inflammatory pseudotumor: a case report and review of literature. J Cardiothorac Surg 2023; 18:215. [PMID: 37403075 DOI: 10.1186/s13019-023-02317-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/11/2022] [Accepted: 06/28/2023] [Indexed: 07/06/2023] Open
Abstract
BACKGROUND Inflammatory pseudotumor (IPT) of the esophagus is a very rare benign lesions which clinical presentation is not clear and difficult to make a definitive diagnosis preoperatively. CASE PRESENTATION In this report, we presented a case of a 24-year-old female with signs of severe malnutrition state due to dysphagia increasing gradually and losing 10 kg in weight for 2 months. Comprehensive preoperative radiologic investigations were proceeded with a circumferential severe stricture caused smooth submucosal swelling in the esophagus under 23 cm from the upper dental arch and two times of negative biopsy. Due to the aggressive clinical symptoms and gross lesion characteristics, the patient underwent laparoscopic-thoracoscopic esophagectomy and reconstruction with a gastric tube. Histopathological examination showed that the squamous epithelium of the esophagus had a small, benign nucleus, the submucosal layer and the smooth muscle layer increased fibrous, with infiltrating many lymphocytes, plasma cells, and macrophages. Immunohistochemical staining was negative for CD68, CD34, Desmin and ALK markers, and there was an increase in the number of IgG4-positive plasma cells. The final diagnosis was an aggressive IgG4-related sclerosing esophageal inflammatory pseudotumor. CONCLUSIONS Inflammatory pseudotumor of the esophagus is an extremely rare benign lesion but could led to aggressive clinical presentation. The gold standard of diagnosis is histopathological examination of surgically removed specimens. Radical resection is still the most efficient treatment method.
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Affiliation(s)
- Hoang Nguyen
- Department of Gastrointestinal and Hepatobiliary surgery, Hanoi Medical University Hospital, Hanoi, Vietnam
- Department of Surgery, Hanoi Medical University, 1st Ton That Tung Street, Dong Da, Ha Noi, Hanoi, 11521, Vietnam
| | - Duc Huan Pham
- Center for Gastroenterology - Hepatobiliary - Urology - Vinmec Times City International Hospital, Hanoi, Vietnam
| | - Tuan Hiep Luong
- Department of Surgery, Hanoi Medical University, 1st Ton That Tung Street, Dong Da, Ha Noi, Hanoi, 11521, Vietnam.
- Department of Gastrointestinal and Hepato-pancreato-biliary surgery, Bach Mai Hospital, Hanoi, Vietnam.
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18
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Zhang Y, Liu J. Inflammatory myofibroblastic tumor of the thyroid gland. Front Endocrinol (Lausanne) 2023; 14:1156117. [PMID: 37255972 PMCID: PMC10225677 DOI: 10.3389/fendo.2023.1156117] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/01/2023] [Accepted: 05/01/2023] [Indexed: 06/01/2023] Open
Abstract
Inflammatory myofibroblastic tumor (IMT) is a mesenchymal tumor with low incidence, which is extremely rare in the thyroid. At present, there is a lack of understanding regarding the etiology, pathogenesis, diagnosis and treatment of thyroid IMT. To improve the understanding of the disease, this article reviews the pathogenesis, clinical manifestations, pathology and immunohistochemistry, diagnosis, therapy and prognosis of thyroid IMT.
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19
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Prijovic N, Santric V, Babic U, Stankovic B, Acimovic M, Cekerevac M, Nikolic G, Cegar B. Inflammatory Myofibroblastic Tumour of the Urinary Bladder in a Middle-Aged Man-A Case Report of an Unusual Localization of a Rare Tumour. MEDICINA (KAUNAS, LITHUANIA) 2023; 59:medicina59040791. [PMID: 37109749 PMCID: PMC10144372 DOI: 10.3390/medicina59040791] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/20/2023] [Revised: 04/04/2023] [Accepted: 04/06/2023] [Indexed: 04/29/2023]
Abstract
Inflammatory myofibroblastic tumour (IMT) is a rare tumour with an intermediate biological behaviour. It usually occurs in children and adolescents, primarily in the abdomen or lungs. Histopathologically, IMT consists of spindle cells, i.e., myofibroblasts, and a variable inflammatory component. Localization in the urinary bladder is rare. We are presenting a rare case of IMT in the bladder in a middle-aged man treated by partial cystectomy. A 62-year-old man consulted a urologist because of haematuria and dysuric disturbances. A tumorous mass was detected by an ultrasound in the urinary bladder. CT urography described the tumorous mass at the dome of the urinary bladder measuring 2 × 5 cm. A smooth tumorous mass was cystoscopically observed at the dome of the urinary bladder. Transurethral resection of the bladder tumour was performed. Histopathological analysis of the specimen identified spindle cells with a mixed inflammatory infiltrate; immunohistochemical findings showed positivity for anaplastic lymphoma kinase (ALK), smooth muscle actin (SMA) and vimentin. A histopathological diagnosis of IMT was established. It was decided that the patient would undergo a partial cystectomy. A complete excision of the tumour from the dome of the urinary bladder with surrounding healthy tissue was performed. Histopathological and immunohistochemical findings of the sample confirmed the diagnosis of IMT, without the presence of the tumour at the surgical margins. The postoperative course went smoothly. IMT is a rare tumour in adults, especially localised in the urinary bladder. IMT of the urinary bladder is difficult to distinguish from urinary bladder malignancy both clinically and radiologically, as well as histopathologically. If the location and size of the tumour allow it, bladder-preserving surgeries such as partial cystectomy represent a reasonable modality of operative treatment.
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Affiliation(s)
- Nebojsa Prijovic
- Clinic of Urology, University Clinical Center of Serbia, 11000 Belgrade, Serbia
| | - Veljko Santric
- Clinic of Urology, University Clinical Center of Serbia, 11000 Belgrade, Serbia
- Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia
| | - Uros Babic
- Clinic of Urology, University Clinical Center of Serbia, 11000 Belgrade, Serbia
- Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia
| | - Branko Stankovic
- Clinic of Urology, University Clinical Center of Serbia, 11000 Belgrade, Serbia
| | - Miodrag Acimovic
- Clinic of Urology, University Clinical Center of Serbia, 11000 Belgrade, Serbia
- Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia
| | - Milica Cekerevac
- Department of Pathology, University Clinical Centre of Serbia, 11000 Belgrade, Serbia
| | - Gorana Nikolic
- Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia
- Institute of Pathology, Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia
| | - Bojan Cegar
- Clinic of Urology, University Clinical Center of Serbia, 11000 Belgrade, Serbia
- Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia
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20
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Desai G, Parikh DM, Wagle PK. A Giant Solid-Cystic Gastric Inflammatory Myofibroblastic Tumor: A Case Report and Literature Review. Cureus 2023; 15:e37167. [PMID: 37168160 PMCID: PMC10166389 DOI: 10.7759/cureus.37167] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/05/2023] [Indexed: 05/13/2023] Open
Abstract
An inflammatory myofibroblastic tumor (IMT) is a very rare tumor of mesenchymal origin with unclear etiopathogenesis, no unique diagnostic features, and no specific management protocol. It is often confused with inflammatory pseudotumor in literature, and the distinction needs further study. The average size, recurrence risk, and metastatic potential differ as per the site of origin. The abdomen is a very rare site for IMTs. Hepatic IMTs (H-IMTs) are reported to be solid tumors with sizes ranging from 1 cm to 20 cm in literature, and gastric IMTs (G-IMTs) range from 3 cm to 10 cm in size and can be solid-cystic. We report here a case of a 36-year-old gentleman with a 34x27x17 cm solid-cystic lesion in the lesser sac with loss of fat planes with stomach and left hemi-liver. The patient was managed by complete surgical resection of the lesion with wedge gastrectomy and wedge hepatectomy and recovered uneventfully. To our knowledge and based on our literature review, this case presents the largest reported and solid-cystic G-IMT with the involvement of left hemi-liver in a young gentleman and discusses its management as well as the relevant literature on this rare entity. This clinical presentation of G-IMT should be kept in the differential diagnosis in a relevant case presenting in the future. Immunohistochemistry is a must to establish the diagnosis, and surgical resection to negative margins is the management option of choice in resectable cases.
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Affiliation(s)
- Gunjan Desai
- Gastrointestinal Surgery, Asian Cancer Institute and ACI Cumballa Hill Hospital, Mumbai, IND
- Gastrointestinal Surgery, Lilavati Hospital and Research Centre, Mumbai, IND
| | - Deepak M Parikh
- Head and Neck Surgery, Asian Cancer Institute and ACI Cumballa Hill Hospital, Mumbai, IND
- Head and Neck Surgery, Lilavati Hospital and Research Centre, Mumbai, IND
| | - Prasad K Wagle
- Gastrointestinal Surgery, Asian Cancer Institute and ACI Cumballa Hill Hospital, Mumbai, IND
- Gastrointestinal Surgery, Lilavati Hospital and Research Centre, Mumbai, IND
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21
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Rispoli C, Marra E, Tufo A, Marte G, D’Avino R, Quassone P, Tamburrini S, Armellino MF. Laparoscopic excision of inflammatory pseudotumors: A case report. G Chir 2023; 43:e03. [DOI: 10.1097/ia9.0000000000000019] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
Abstract
Inflammatory pseudotumors (IPs) are rare incidental benign lesions which can occur in any anatomical site. They have been reported by a variety of names, including inflammatory myofibroblastic tumor, histiocytoma, xanthoma, and plasma cell granuloma. Their knowledge is important because of the differential diagnosis with others true malignancies. Only 30 case reports are reported in the literature. Surgical resection is the mainstay of treatment with excellent outcomes and low recurrence rates. When anatomical site is suitable, a laparoscopic approach should be performed to minimize morbidity and expedite recovery. Here, we present a case report of IPs of antimesenteric bowel treated with a laparoscopic surgical approach.
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Affiliation(s)
- Corrado Rispoli
- Department of Surgery, Azienda Ospedaliera dei Colli Monaldi, Naples, Italy
| | - Ester Marra
- Department of Surgery, Ospedale S.G. Moscati di Aversa, Caserta, Italy
| | - Andrea Tufo
- Department of Surgery, Ospedale del Mare, ASL NA1 Centro, Naples, Italy
| | - Gianpaolo Marte
- Department of Surgery, Ospedale del Mare, ASL NA1 Centro, Naples, Italy
| | - Raffaele D’Avino
- Department of Surgery, Ospedale del Mare, ASL NA1 Centro, Naples, Italy
| | - Pasquale Quassone
- Department of Radiology, Università degli Studi della Campania Luigi Vanvitelli, Naples, Italy
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22
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Luke ND, Gottlieb S, Brothers J, Winikoff S. Inflammatory Myofibroblastic Tumor: A Rare Case Report. Cureus 2023; 15:e36579. [PMID: 37095797 PMCID: PMC10122435 DOI: 10.7759/cureus.36579] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/22/2023] [Indexed: 04/26/2023] Open
Abstract
Inflammatory myofibroblastic tumors (IMTs) are rare benign tumors that can occur anywhere in the body, most commonly in the pediatric and young adult populations. The gold standard treatment is surgical resection, possibly along with chemotherapy and/or radiotherapy. IMTs have a high recurrence rate and may present with secondary symptoms, such as hemoptysis, fever, and stridor. We present a 13-year-old male patient with hemoptysis for one month who was subsequently diagnosed with an obstructing IMT of the trachea. The preoperative assessment showed the patient was not in acute distress and could protect his airway, even when lying flat. The treatment plan was discussed with the otolaryngologist, to keep the patient spontaneously breathing throughout the case. Anesthesia was induced with boluses of midazolam, remifentanil, propofol, and dexmedetomidine. Doses were adjusted as needed. Glycopyrrolate was also given to limit the patient's secretions before initiating the surgical procedure. The FiO2 was kept under 30% as tolerated to reduce the risk of airway fire. During surgical resection, the patient was kept spontaneously breathing, and paralytics were avoided. Due to high tumor vascularity and inability to obtain hemostasis, the patient was kept intubated and on ventilation post-operatively until definitive treatment could be performed. On postoperative day 3, the patient returned to the operating room due to a worsening condition. He was found to have a partial obstruction of the right mainstem bronchus by the tumor. More of the tumor was debulked, and he remained intubated above the level of the debulked mass. The patient was then transferred to a higher acuity institution for advanced care. After the transfer, the patient underwent a carinal resection on cardiopulmonary bypass. This case provides insight into successfully sharing the airway during tracheal tumor resection, emphasizing minimizing the risk of airway fire and constant communication with the surgeon.
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Affiliation(s)
- Nicholas D Luke
- Medicine, St. George's University School of Medicine, True Blue, GRD
| | | | - Julia Brothers
- Anesthesiology, St. Joseph's Regional Medical Center, Paterson, USA
| | - Stephen Winikoff
- Anesthesiology, St. Joseph's Regional Medical Center, Paterson, USA
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23
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Chanthong S, Sathitsamitphong L, Natesirinilkul R, Charoenkwan P, Suwansirikul S, Choed-Amphai C. Treatment modalities of ALK-positive relapsed/refractory inflammatory myofibroblastic tumor of the brain and lungs in 7-year-old girl: case-based reviews. Childs Nerv Syst 2023; 39:331-342. [PMID: 36515740 DOI: 10.1007/s00381-022-05789-w] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/14/2022] [Accepted: 12/08/2022] [Indexed: 12/15/2022]
Abstract
PURPOSE Inflammatory myofibroblastic tumor (IMT) belongs to mesenchymal neoplasm of intermediate malignancy in WHO classification. Primary CNS disease or CNS metastases (CNS-IMT) occur in minority. We describe a case of relapsed/refractory IMT of lungs with multiple brain metastases in young child who achieved long-term complete response after alectinib. This systematic review also summarizes treatment modalities and outcome of children and adolescent with CNS-IMT. METHODS PRISMA 2020 guideline was applied to select an article from PubMed, Scopus, and Cochrane databases without time limits. This review focused on children and adolescent 0-24 years of age with CNS-IMT or inflammatory pseudotumor (CNS-IPT). The clinical characteristics and treatment outcome were explored. RESULTS A total of 51 patients in 49 publications were identified. Median age of patients with CNS-IMT/IPT was 15-year-old and 60.8% were male. The most common location of tumor was cerebral cortex (54.9%). Complete resection of CNS-IMT/IPT was performed in 27 cases with 100% complete response and 18.5% recurrence. Nearly half of patients who received partial resection without adjuvant therapy experienced progressive disease, while the contrast group totally achieved partial response. Overall responses in 7 patients treating with ALK inhibitors were 57.1% durable complete response and 42.9% transient partial response. CONCLUSION First-line treatment of CNS-IMT/IPT is complete resection. Patients who received partial tumor removal might have benefit from adjuvant therapy. ALK inhibitors reveal a promising result in unresectable CNS-IMT/IPT. Our case has shown a success in treating relapsed and refractory CNS-IMT as well as the primary site using 2nd-generation ALK inhibitor.
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24
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Oh JS, Kim HW, Park SB, Kang DH, Choi CW, Kim SJ, Nam HS, Ryu DG. A large and pedunculated inflammatory pseudotumor with pseudosarcomatous change of the cecum mimicking a malignant polyp: a case report and literature review. Clin Endosc 2023; 56:119-124. [PMID: 34275257 PMCID: PMC9902694 DOI: 10.5946/ce.2021.081] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/24/2021] [Accepted: 04/05/2021] [Indexed: 02/04/2023] Open
Abstract
Inflammatory pseudotumor (IPT) is a rare benign tumor of unknown etiology that can occur in almost any organ system. It has neoplastic features such as local recurrence, invasive growth, and vascular invasion, leading to the possibility of malignant sarcomatous changes. The clinical presentations of colonic IPT may include abdominal pain, anemia, a palpable mass, and intestinal obstruction. A few cases of colonic IPT have been reported, but colonic IPT with pedunculated morphology is very rare. Furthermore, since it can mimic malignant polyps, understanding the endoscopic findings of colonic IPT is important for proper treatment. Herein, we present a case of colonic IPT with pseudosarcomatous changes, presenting as a large polyp, mimicking a malignant polyp in the cecum, along with a literature review.
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Affiliation(s)
- Jong Suk Oh
- Department of Internal Medicine, Pusan National University School of Medicine and Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Korea
| | - Hyung Wook Kim
- Department of Internal Medicine, Pusan National University School of Medicine and Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Korea,Correspondence: Hyung Wook Kim Department of Internal Medicine, Pusan National University Yangsan Hospital, Pusan National University School of Medicine and Research Institute for Convergence of Biomedical Science and Technology, 20 Geumo-ro, Mulgeum-eup, Yangsan 50612, Korea E-mail:
| | - Su Bum Park
- Department of Internal Medicine, Pusan National University School of Medicine and Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Korea
| | - Dae Hwan Kang
- Department of Internal Medicine, Pusan National University School of Medicine and Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Korea
| | - Cheol Woong Choi
- Department of Internal Medicine, Pusan National University School of Medicine and Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Korea
| | - Su Jin Kim
- Department of Internal Medicine, Pusan National University School of Medicine and Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Korea
| | - Hyeong Seok Nam
- Department of Internal Medicine, Pusan National University School of Medicine and Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Korea
| | - Dae Gon Ryu
- Department of Internal Medicine, Pusan National University School of Medicine and Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Korea
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25
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Abu-Salah AK, Brocken E, Mesa H, Collins K. Jejunal Intussusception Secondary to a Large Inflammatory Fibroid Polyp: A Case Report and Discussion of Differential Diagnosis. Case Rep Pathol 2023; 2023:9417141. [PMID: 37091748 PMCID: PMC10118902 DOI: 10.1155/2023/9417141] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/31/2022] [Revised: 03/06/2023] [Accepted: 03/28/2023] [Indexed: 04/25/2023] Open
Abstract
Inflammatory fibroid polyp (IFP), initially considered a reactive process, is now recognized as a benign mesenchymal neoplasm of the gastrointestinal tract. We report a case of a 68-year-old woman with medically refractory Crohn disease that presented with intussusception requiring surgical intervention. The resection revealed a jejunal mass consisting of a submucosal proliferation of bland spindle cells in a fibrous stroma infiltrated by numerous eosinophils. By immunohistochemistry, the lesion was positive for vimentin and negative for desmin, smooth muscle actin (SMA), S-100, CD117, DOG1, ALK (D5F3), Melan-A, HMB-45, CD34, and STAT6. Ki-67 proliferative index was low (<1%). The mass was classified as IFP by its characteristic morphology and associated eosinophilia. IFP should be considered in the differential diagnosis of adults with intussusception or bowel obstruction. Definitive treatment typically requires surgical resection of the involved bowel segment.
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Affiliation(s)
- Asma Khalid Abu-Salah
- Department of Pathology, Indiana University School of Medicine, Indianapolis, IN 46202, USA
| | - Eric Brocken
- Department of Pathology, Indiana University School of Medicine, Indianapolis, IN 46202, USA
| | - Hector Mesa
- Department of Pathology, Indiana University School of Medicine, Indianapolis, IN 46202, USA
| | - Katrina Collins
- Department of Pathology, Indiana University School of Medicine, Indianapolis, IN 46202, USA
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Su ZJ, Guo ZS, Wan HT, Hong XY. Inflammatory myofibroblastic tumor of the central nervous system: A case report. World J Clin Cases 2022; 10:12637-12647. [PMID: 36579095 PMCID: PMC9791513 DOI: 10.12998/wjcc.v10.i34.12637] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/30/2022] [Revised: 09/16/2022] [Accepted: 11/07/2022] [Indexed: 12/02/2022] Open
Abstract
BACKGROUND An inflammatory myofibroblastic tumor (IMT) occurring in the central nervous system is very rare, and thus its pathogenesis is unknown. This case report and literature review aimed to explore the pathogenesis, clinical features, imaging findings, pathological characteristics, immunohistochemical characteristics, diagnoses, treatments, and risks of postoperative recurrence of IMT in the central nervous system.
CASE SUMMARY A 67-year-old woman was admitted to the hospital with an exophthalmic protrusion and double vision in the left eye that had persisted for 3 mo. Magnetic resonance imaging (MRI) showed a 2.4 cm × 1.3 cm heterogeneous large mass in the bottom of the left anterior cranial fossa, which was closely related to the dura mater. Before surgery, we suspected the mass to be meningioma. The entire mass was successfully removed under neuronavigation and electrophysiological monitoring, and postoperative pathology indicated an IMT with extensive infiltration of chronic inflammatory cells and scattered multinucleated giant cells. Head MRI at the 3-mo follow-up showed that the tumor at the bottom of left anterior cranial fossa had been completely resected without recurrence.
CONCLUSION From the histological, immunohistochemical, and genetic analyses, the present case suggests that the pathogenesis of IMT-CNS is related to autoimmunity.
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Affiliation(s)
- Zhen-Jin Su
- Department of Neurosurgical Oncology, The First Hospital of Jilin University, Changchun 130000, Jilin Province, China
| | - Ze-Shang Guo
- Department of Neurosurgical Oncology, The First Hospital of Jilin University, Changchun 130000, Jilin Province, China
| | - Heng-Tong Wan
- Department of Neurosurgical Oncology, The First Hospital of Jilin University, Changchun 130000, Jilin Province, China
| | - Xin-Yu Hong
- Department of Neurosurgical Oncology, The First Hospital of Jilin University, Changchun 130000, Jilin Province, China
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Li YY, Zang JF, Zhang C. Laparoscopic treatment of inflammatory myofibroblastic tumor in liver: A case report. World J Clin Cases 2022; 10:11853-11860. [PMID: 36405255 PMCID: PMC9669864 DOI: 10.12998/wjcc.v10.i32.11853] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/11/2022] [Revised: 07/12/2022] [Accepted: 08/30/2022] [Indexed: 11/07/2022] Open
Abstract
BACKGROUND Inflammatory myofibroblastic tumor in the liver (IMTL) is a rare borderline mesenchymal tumor. Neither clinical symptoms nor laboratory tests have absolute specificity for the diagnosis of IMTL, and imaging also lacks obvious specificity. Although there are sporadic reports of recurrence after surgical treatment, surgical resection is the mainstay of treatment.
CASE SUMMARY A 29-year-old man complained of general weakness, slight discomfort in the upper abdomen, with a history of upper respiratory tract infection for 1 wk before admission. Plain and enhanced upper abdominal magnetic resonance imaging showed a mass in liver segments II and III (48 mm × 53 mm). He was treated by laparoscopic left lateral segmentectomy. Postoperative pathological examination with hematoxylin and eosin staining suggested that the mass in liver segments II and III was IMTL. During 21 mo postoperative follow-up, no obvious residual or recurrent lesions were observed.
CONCLUSION There is a risk of malignant degeneration in IMTL. The principal choice of treatment is laparoscopic left lateral segmentectomy.
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Affiliation(s)
- Yang-Yang Li
- Department of Hepatobiliary Surgery, Taizhou People’s Hospital, The Fifth Affiliated Hospital of Medical School of Nantong University, Taizhou 225300, Jiangsu Province, China
| | - Jin-Feng Zang
- Department of Hepatobiliary Surgery, Taizhou People’s Hospital, The Fifth Affiliated Hospital of Medical School of Nantong University, Taizhou 225300, Jiangsu Province, China
| | - Chi Zhang
- Department of Hepatobiliary Surgery, Taizhou People’s Hospital, The Fifth Affiliated Hospital of Medical School of Nantong University, Taizhou 225300, Jiangsu Province, China
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28
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Narihiro S, Teramura K, Kitaguchi D, Ikeda K, Hasegawa H, Tsukada Y, Nishizawa Y, Ito M. Transanal total mesorectal excision for treating a lower rectal inflammatory myofibroblastic tumor: A case report. Asian J Endosc Surg 2022; 15:841-845. [PMID: 35665471 DOI: 10.1111/ases.13090] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/06/2022] [Revised: 05/16/2022] [Accepted: 05/18/2022] [Indexed: 11/29/2022]
Abstract
Rectal inflammatory myofibroblastic tumors are extremely rare, with no reports of their preoperative diagnosis. A 17-year-old woman who presented with low-grade fever, repeated diarrhea, constipation, and a 1-month history of anal pain was referred to our hospital. Rectal examination revealed a palpable hard mass with a smooth surface at the posterior wall 4 cm from the anal verge. Colonoscopy revealed a 4.0-cm submucosal tumor in the upper edge of the anal canal. Computed tomography and magnetic resonance imaging revealed a 5.0 × 4.0 cm-sized well-defined tumor contacting the rectum. Computed tomography-guided biopsy was performed, and an inflammatory myofibroblastic tumor was diagnosed. There have been no reports of surgery for a rectal inflammatory myofibroblastic tumor using transanal total mesorectal excision. We preoperatively diagnosed the patient with an inflammatory myofibroblastic tumor in the lower rectum and achieved anorectal preservation and curative resection with transanal total mesorectal excision, providing good view of the deep pelvis.
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Affiliation(s)
- Satoshi Narihiro
- Department of Colorectal Surgery, National Cancer Center Hospital East, Kashiwa, Japan
| | - Koichi Teramura
- Department of Colorectal Surgery, National Cancer Center Hospital East, Kashiwa, Japan
| | - Daichi Kitaguchi
- Department of Colorectal Surgery, National Cancer Center Hospital East, Kashiwa, Japan
| | - Koji Ikeda
- Department of Colorectal Surgery, National Cancer Center Hospital East, Kashiwa, Japan
| | - Hiro Hasegawa
- Department of Colorectal Surgery, National Cancer Center Hospital East, Kashiwa, Japan
| | - Yuichiro Tsukada
- Department of Colorectal Surgery, National Cancer Center Hospital East, Kashiwa, Japan
| | - Yuji Nishizawa
- Department of Colorectal Surgery, National Cancer Center Hospital East, Kashiwa, Japan
| | - Masaaki Ito
- Department of Colorectal Surgery, National Cancer Center Hospital East, Kashiwa, Japan
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29
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Setiawan D, Sari PP, Adibrata ASP, Nugraha HG, Soetikno RD, Hernowo B. Inflammatory Myofibroblastic Tumor in Bladder with Multiple Vesicocutaneous Fistula in Pediatric Patient: A Rare Case Report. Open Access Maced J Med Sci 2022. [DOI: 10.3889/oamjms.2022.9414] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
Inflammatory myofibroblastic tumor (IMT) is a rare tumor that occurs at any age from childhood to late adulthood and may have a slight male predilection. A 7-year-old female presented with gross hematuria and a lump in the lower abdomen since 1 month prior to admission. The complaints were also accompanied by weight loss, abdominal pain and anemia. Ultrasound examination, Cystography, CT-Scan and MRI showed that mass in the bladder. Histopathology examination confirmed diagnosis for IMT, thus, radiology plays an important role in supporting histologic examination for diagnosis and evaluation of IMT.
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30
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Durham C, Clemons M, Alias A, Konduri K. Locally Advanced Inflammatory Myofibroblastic Tumor Treated With Targeted Therapy: A Case Report and Literature Review. Cureus 2022; 14:e27223. [PMID: 36035049 PMCID: PMC9400374 DOI: 10.7759/cureus.27223] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/25/2022] [Indexed: 11/30/2022] Open
Abstract
Inflammatory myofibroblastic tumors (IMTs) are known to be associated with anaplastic lymphoma kinase (ALK) gene rearrangements. Other molecular alterations such as ROS proto-oncogene 1, receptor tyrosine kinase (ROS1), neurotrophic tyrosine receptor kinase (NTRK), and platelet-derived growth factor receptor (PDGFR) have also been identified in IMTs. Although there are no randomized controlled clinical trials comparing chemotherapy, tyrosine kinase inhibitors (TKIs), or other systemic therapies, the literature demonstrates the use of ALK-targeted TKIs as an effective strategy for the treatment of locally advanced or metastatic ALK-rearranged IMTs. This case report describes a patient with an ALK-rearranged locally advanced pulmonary IMT who was treated with neoadjuvant-intent crizotinib. The patient had a very favorable response to therapy, and surgery was declined. It is difficult to determine the duration and sequencing of TKI use in these settings as there is little published data to guide decisions. This report also includes a comprehensive compilation of published IMT cases with molecular alterations treated with systemic therapy, which also highlighted the duration of therapies and clinical outcomes.
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Lourdesamy Anthony AI, Satnam Singh TK, Ng KL, Abdul Rahaman JA. Endotracheal inflammatory myofibroblastic tumour: A rare cause of central airway occlusion in adults. Respirol Case Rep 2022; 10:e0984. [PMID: 35702693 PMCID: PMC9174595 DOI: 10.1002/rcr2.984] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/07/2022] [Accepted: 05/19/2022] [Indexed: 11/29/2022] Open
Abstract
Inflammatory myofibroblastic tumour (IMT) is an unusual myofibroblastic spindle cell neoplasm that is rarely discovered in the airway of adults. Previously, it was regarded as a reactive lesion and was infamously known as inflammatory pseudotumour before recent insights revealed that significant majority of cases harboured neoplastic genes. Diagnosis is difficult as clinical presentation and imaging findings are non-specific. Diagnosis and a favourable prognosis require the complete resection of the tumour. Detection of the anaplastic lymphoma kinase expression via immunohistochemistry expedites diagnosis. We report a young adult with an endotracheal mass occluding the central airway. The patient successfully underwent bronchoscopic resection using interventional techniques. IMT was diagnosed. No recurrence was seen after a year of surveillance. Due to the rarity of the disease, the recurrence rates for large airway disease is unknown. Recurrence rates for pulmonary lesions is reported to be lower compared to extrapulmonary IMTs and recurrence is unlikely if compete surgical excision is achieved.
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Affiliation(s)
| | | | - Khai Lip Ng
- Department of MedicineHospital MelakaMelakaMalaysia
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Seyedi S, Saeidinia A, Dehghanian P. Pulmonary inflammatory myofibroblastic tumor in a male child: A case report. Clin Case Rep 2022; 10:e6003. [PMID: 35769237 PMCID: PMC9211773 DOI: 10.1002/ccr3.6003] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/06/2022] [Revised: 06/08/2022] [Accepted: 06/13/2022] [Indexed: 01/21/2023] Open
Abstract
Pulmonary inflammatory myofibroblastic tumor (IMT) is a rare condition in the normal population and specifically in the pediatric population. We reported a 9-year-old male child who presented with cough and intermittent fever and weight loss that was most suggestive of the infectious process. We reviewed the consideration of diagnosis and treatment.
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Affiliation(s)
- Seyed‐Javad Seyedi
- Pediatric Department, Faculty of MedicineMashhad University of Medical SciencesMashhadIran
| | - Amin Saeidinia
- Pediatric Department, Faculty of MedicineMashhad University of Medical SciencesMashhadIran
- Pharmaceutical Research CenterMashhad University of Medical SciencesMashhadIran
| | - Parisa Dehghanian
- Pathology Department, Akbar HospitalMashhad University of Medical SciencesMashhadIran
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Pire A, Orbach D, Galmiche L, Berrebi D, Irtan S, Boudjemaa S, Brisse HJ, Berteloot L, Moalla S, Mussini C, Philippe-Chomette P, Tilea B, Pierron G, Guerin F, Minard-Colin V, Sarnacki S. Clinical, pathologic, and molecular features of inflammatory myofibroblastic tumors in children and adolescents. Pediatr Blood Cancer 2022; 69:e29460. [PMID: 34854544 DOI: 10.1002/pbc.29460] [Citation(s) in RCA: 10] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/04/2021] [Revised: 10/01/2021] [Accepted: 10/18/2021] [Indexed: 01/09/2023]
Abstract
BACKGROUND Inflammatory myofibroblastic tumors (IMT) are rare, intermediate malignant tumors harboring frequent somatic molecular rearrangements. The management of IMT has not been standardized. METHODS A retrospective multicenter study was conducted on all pediatric patients treated for IMT between 2000 and 2019. RESULTS This series included 39 cases of IMT, with a median age at diagnosis of 7 years (range 20 days to 16 years). Tumor location included pelvis-abdomen (n = 16), thorax (n = 14), head and neck (n = 7), and limbs (n = 2). One patient had metastatic disease. Immunochemistry showed 21/39 (54%) anaplastic lymphoma kinase (ALK)-positive tumors. Somatic tyrosine kinase rearrangement was present in 31/36 (86%) of the tumors analyzed: 21 ALK, five ROS1, and five NTRK. Immediate surgery was performed in 24 patients (62%), with adjuvant therapy for three patients. Delayed surgery after neoadjuvant therapy was possible in 10 cases. Exclusive systemic therapy was delivered to four patients; one patient with orbital IMT was managed by watchful waiting. After a median follow-up of 33 months (range 5-124), eight (20%) recurrences/progressions occurred after surgery (seven after primary surgery and one after delayed surgery), after a median interval of 7 months (range 2-21), all in thoracic locations. The 3-year overall and disease-free survivals were 96.8% (95% CI: 79.2%-94.0%) and 77.4% (95% CI: 59.6%-88.1%), respectively. Relapses/progressions were more common in patients with a thoracic primary (p < .001) or after incomplete surgery with no adjuvant therapy (p = .027). CONCLUSION Surgery is effective in most cases of pediatric IMT. Systematic analysis of tyrosine kinase rearrangement is recommended. When the tumor is deemed only partially resectable to preserve organs and function, neoadjuvant therapy may be proposed to allow adequate conservative surgery.
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Affiliation(s)
- Aurore Pire
- Department of Paediatric Surgery and Abdominal Transplantation, ,Cliniques Universitaires Saint-Luc, Brussels, Belgium
| | - Daniel Orbach
- SIREDO Oncology Center (Care, Innovation and Research for Children and AYA with Cancer), PSL Research University, Institut Curie, Paris, France
| | - Louise Galmiche
- Department of pathology, University Hospital of Nantes, Nantes, France
| | | | - Sabine Irtan
- Department of Paediatric Surgery, Hopital Armand Trousseau, Paris, France
| | - Sabah Boudjemaa
- Department of Pathology, Hopital Armand Trousseau, Paris, France
| | | | | | - Salma Moalla
- Department of Imaging, Institut Gustave Roussy, Paris, France
| | - Charlotte Mussini
- Department of Pathology, Hopital Kremlin Bicêtre, Pathology, Paris, France
| | | | - Bogdana Tilea
- Department of Imaging, Hopital Robert Debré, Paris, France
| | - Gaelle Pierron
- Institut Curie, Unité de Génétique Somatique, Paris, France
| | - Florent Guerin
- Department of Paediatric Surgery, Hôpital Kremlin Bicêtre, Paris, France
| | - Véronique Minard-Colin
- Department of Paediatric and Adolescent Oncology, INSERM U1015, Gustave Roussy, Université Paris-Saclay, Villejuif, France
| | - Sabine Sarnacki
- Department of Paediatric Surgery, Hôpital Universitaire Necker-Enfants Malades, Université de Paris, Paris, France
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Lee CS, Kim JS, Rodriguez R, Krell RW. Anaplastic Lymphoma Kinase Positive Mesenteric Inflammatory Myofibroblastic Tumor in Adult Woman. Cureus 2022; 14:e24422. [PMID: 35637807 PMCID: PMC9126854 DOI: 10.7759/cureus.24422] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/23/2022] [Indexed: 11/25/2022] Open
Abstract
Inflammatory myofibroblastic tumors (IMTs) are rare mesenchymal neoplasms containing spindle cells and inflammatory components that can be locally aggressive. They have unclear biological behavior and may recur after resection. A 31-year-old woman presented with three months of cough, fatigue, weight loss, abdominal pain, anemia, and elevated inflammatory markers. CT showed a large well-circumscribed enhancing mass in the right colic mesentery. The patient underwent a laparoscopic right colectomy. Pathologic review showed fascicular spindle cells with admixed chronic inflammatory cells. Cells stained diffusely positive for SMA and anaplastic lymphoma kinase (ALK), diagnostic of an IMT. Post-operatively, the patient reported symptom resolution and had normalization of lab values. She remains disease-free at 20 months. IMT is rare in adults, accounting for 0.7%-1.0% of lung tumors. Up to 30% of patients present with elevated inflammatory markers. On imaging, IMTs are soft tissue masses with variable enhancement and fibrosis, often suspected to be malignant neoplasms. Up to 80% of IMTs are driven by altered tyrosine kinase signaling and half of IMTs express ALK, which may be treated in unresectable/recurrent cases using ALK-inhibitors. IMT may recur in 10%-15% of patients. The roles of adjuvant treatments are unclear given the rarity and unpredictable biological behavior. Long-term follow-up with regular radiologic and laboratory surveillance is recommended given possible local recurrence. IMTs are best managed in a multidisciplinary setting given their unpredictable nature. Surgery is the mainstay of IMT treatment with long-term control expected in >80% of adult patients.
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35
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Wang Z, Geng Y, Yuan LY, Wang MM, Ye CY, Sun L, Dai WP, Zang YS. Durable Clinical Response to ALK Tyrosine Kinase Inhibitors in Epithelioid Inflammatory Myofibroblastic Sarcoma Harboring PRRC2B-ALK Rearrangement: A Case Report. Front Oncol 2022; 12:761558. [PMID: 35237506 PMCID: PMC8882834 DOI: 10.3389/fonc.2022.761558] [Citation(s) in RCA: 19] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/20/2021] [Accepted: 01/24/2022] [Indexed: 12/21/2022] Open
Abstract
Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal neoplasm and patients with IMT tend to have a favorable outcome after complete surgical resection. However, some tumors of IMT cases have recurred and grown rapidly after successful surgery. Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a highly aggressive intra-abdominal IMT variant with epithelioid-to-round cell morphology. Currently, no standard therapy exists for recurrent or invasive IMTs and EIMS, but anaplastic lymphoma kinase (ALK) tyrosine kinase inhibitors (TKIs) are recommended for those harboring ALK gene rearrangements. We herein report the first case of PRRC2B-ALK fusion associated IMTs with clinical and pathological manifestation matched the diagnosis criteria of EIMS and the durable clinical response of the sequential use of ALK TKIs (crizotinib, alectinib, ceritinib, and lorlatinib). A female patient with EIMS of the greater omentum was suffering from a rapid recurrence after cytoreductive surgery was done. Crizotinib was administered when PRRC2B-ALK fusion was detected, and partial response was achieved. The progression-free survival (PFS) of crizotinib was 5 months. Alectinib was administered based on the results of second next-generation sequencing (NGS) analysis, which identified the secondary mutation ALK R1192P. The best overall response of alectinib treatment was a partial response (PR) and the PFS was 5.5 months. Ceritinib was prescribed as third-line therapy after alectinib resistance with ALK L1196M mutation. PR was achieved and the PFS of ceritinib was 6 months. The patient was taking lorlatinib after ceritinib resistance and achieved a stable disease at 2 months with the PFS more than 5 months. The overall survival was more than two years as of the time of manuscript preparation. We describe an EIMS of greater omentum caused by PRRC2B-ALK fusion gene and showed durable clinical response to the sequential use of ALK TKIs.
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Affiliation(s)
- Zhan Wang
- Department of Medical Oncology, Second Affiliated Hospital of Naval Medical University, Shanghai, China
| | - Yan Geng
- Department of Nursing, Shanghai Jing’an District Zhabei Central Hospital, Shanghai, China
| | - Ling-Yan Yuan
- Department of Medical Oncology, Second Affiliated Hospital of Naval Medical University, Shanghai, China
| | - Miao-Miao Wang
- Department of Medical Oncology, Second Affiliated Hospital of Naval Medical University, Shanghai, China
| | - Chen-Yang Ye
- Department of Medical Oncology, Second Affiliated Hospital of Naval Medical University, Shanghai, China
| | - Li Sun
- Department of Medical Oncology, Second Affiliated Hospital of Naval Medical University, Shanghai, China
| | - Wei-Ping Dai
- Department of Medical Oncology, Second Affiliated Hospital of Naval Medical University, Shanghai, China
| | - Yuan-Sheng Zang
- Department of Medical Oncology, Second Affiliated Hospital of Naval Medical University, Shanghai, China
- *Correspondence: Yuan-Sheng Zang, ; orcid.org/0000-0002-9488-7305
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Zhou P, Chen YH, Lu JH, Jin CC, Xu XH, Gong XH. Inflammatory myofibroblastic tumor after breast prosthesis: A case report and literature review. World J Clin Cases 2022; 10:1432-1440. [PMID: 35211580 PMCID: PMC8855174 DOI: 10.12998/wjcc.v10.i4.1432] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/05/2021] [Revised: 10/21/2021] [Accepted: 12/23/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Inflammatory myofibroblastic tumors (IMTs) are defined as tumors composed of differentiated myofibroblastic spindle cells, usually accompanied by numerous plasma cells and lymphocytes, and classified as intermediate (occasionally metastatic) by the World Health Organization. Its pathogenesis and biological behavior have not yet been elucidated. Breast IMT is extremely rare, and prosthesis implantation combined with IMT has not been reported. This study reports a case of IMT following resection of a malignant phyllodes tumor of the left breast and implantation of a prosthesis.
CASE SUMMARY A 41-year-old female presented to our hospital with a mass in the left breast for 3 mo. The patient had undergone resection of a large mass in her left breast pathologically diagnosed as a malignant phyllodes tumor and implantation of a prosthesis five years prior. Ultrasonic examination revealed an oval mass in the left breast, and the patient underwent left breast mass resection and prosthesis removal. Light microscopy revealed the spindle cells to be diffusely proliferated, with a large number of neutrophils, lymphocytes, and plasma cell infiltration. Immunohistochemical staining revealed that the spindle cells were partially positive for smooth muscle actin, which is positive for BCL-2 and cluster of differentiation (CD) 99 but were negative for anaplastic lymphoma kinase, cytokeratin, S-100 protein, desmin, and CD34. The final diagnosis was IMT. No recurrence or metastasis was observed during the 5-year postoperative follow-up.
CONCLUSION Prosthesis implantation may be one of the causes of IMT, but further investigation is necessary to prove it.
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Affiliation(s)
- Peng Zhou
- Department of Ultrasound, Shenzhen Second People’s Hospital, The First Affiliated Hospital of Shenzhen University, Shenzhen 518035, Guangdong Province, China
| | - Yi-Hao Chen
- Department of Ultrasound, Shenzhen Second People’s Hospital, The First Affiliated Hospital of Shenzhen University, Shenzhen 518035, Guangdong Province, China
- Graduate School, Guangdong Medical University, Zhanjiang 524023, Guangdong Province, China
| | - Jiang-Hao Lu
- Department of Ultrasound, Shenzhen Second People’s Hospital, The First Affiliated Hospital of Shenzhen University, Shenzhen 518035, Guangdong Province, China
| | - Chun-Chun Jin
- Department of Ultrasound, Shenzhen Second People’s Hospital, The First Affiliated Hospital of Shenzhen University, Shenzhen 518035, Guangdong Province, China
| | - Xiao-Hong Xu
- Graduate School, Guangdong Medical University, Zhanjiang 524023, Guangdong Province, China
- Department of Ultrasound, Guangdong Medical University Affiliated Hospital, Zhanjiang 524001, Guangdong Province, China
| | - Xue-Hao Gong
- Department of Ultrasound, Shenzhen Second People’s Hospital, The First Affiliated Hospital of Shenzhen University, Shenzhen 518035, Guangdong Province, China
- Graduate School, Guangdong Medical University, Zhanjiang 524023, Guangdong Province, China
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Zhu T, Gao P, Gao J, Liu X, Zhang X. A huge recurrent retroperitoneal inflammatory myofibroblastic tumor implicating renal and ureter: a case report. Transl Cancer Res 2022; 10:4565-4570. [PMID: 35116312 PMCID: PMC8798890 DOI: 10.21037/tcr-21-899] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2021] [Accepted: 07/30/2021] [Indexed: 12/05/2022]
Abstract
Inflammatory myofibroblastic tumor (IMT) is a rare sarcoma with unique molecular characteristics. It also has typical fasciitis-like, dense spindle cells and hypocellular fibrous histologic patterns and can occur at any age. The etiology of IMT is unknown and a complete treatment and monitoring plan for IMT have not been developed. We have found a rare case about a huge recurrent IMT implicating renal and ureter. The patient was accompanied by a history of a lesion sized 15.5 cm × 11.5 cm × 10.5 cm as a retroperitoneal IMT, and the first tumor resection was performed 12 months ago. The patient, who suffered from tumor recurrence within 1 year and whose recurrent tumor also invaded into the renal parenchymal was given a retroperitoneal neoplasm resection plus right nephrectomy. In order to clarify the pathological features, the histological specimens of recurrent retroperitoneal IMT were examined as well as primary retroperitoneal IMT, and immunohistochemical (IHC) stains showed that the spindle cells were positive for vimentin and negative for anaplastic lymphoma kinase (ALK). At 24 months follow-up, the patient recovered well and there was no evidence of tumor recurrence. This case report is conducive to understanding this rare tumor. When conditions permit, complete surgical resection is the recommended treatment for primary and recurrent IMT.
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Affiliation(s)
- Tianle Zhu
- Department of Urology, The First Affiliated Hospital of Anhui Medical University, Hefei, China
| | - Pan Gao
- Department of Urology, The First Affiliated Hospital of Anhui Medical University, Hefei, China
| | - Jingjing Gao
- Department of Urology, The First Affiliated Hospital of Anhui Medical University, Hefei, China
| | - Xi Liu
- Department of Urology, The First Affiliated Hospital of Anhui Medical University, Hefei, China
| | - Xiansheng Zhang
- Department of Urology, The First Affiliated Hospital of Anhui Medical University, Hefei, China
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Gross Hematuria in an Adolescent Secondary to a Rare Bladder Tumor: A Case Report and Review of Inflammatory Myofibroblastic Tumors of the Urinary Bladder. Urology 2022; 165:e39-e45. [DOI: 10.1016/j.urology.2022.01.034] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2021] [Revised: 01/13/2022] [Accepted: 01/23/2022] [Indexed: 11/19/2022]
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Park JW, Han SH, Kim DK. [Inflammatory Myofibroblastic Tumor Misdiagnosed as Intrahepatic Cholangiocarcinoma]. THE KOREAN JOURNAL OF GASTROENTEROLOGY 2022; 79:41-44. [PMID: 35086972 DOI: 10.4166/kjg.2021.146] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/26/2021] [Revised: 11/28/2021] [Accepted: 11/28/2021] [Indexed: 11/03/2022]
Abstract
An inflammatory myofibroblastic tumor (IMT) is a rare tumor that is currently classified as an intermediate cancer according to the World Health Organization classification system. The pathophysiology of its occurrence is still unknown. Imaging tests, such as CT or MRI, can be helpful in diagnosis, but the final diagnosis is confirmed by a pathological examination through a biopsy and immunohistochemistry stain. The patient, in this case, presented an asymptomatic intrahepatic mass discovered incidentally on an imaging examination. Initially, intrahepatic cholangiocarcinoma was suspected, but she was finally diagnosed with IMT through a histological examination after a liver resection.
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Affiliation(s)
- Ji Weon Park
- Department of Gastroenterology, Dong-A University Hospital, Dong-A University College of Medicine, Busan, Korea
| | - Song Hee Han
- Department of Pathology, Dong-A University Hospital, Dong-A University College of Medicine, Busan, Korea
| | - Dong Kyun Kim
- Department of Gastroenterology, Dong-A University Hospital, Dong-A University College of Medicine, Busan, Korea
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40
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Demir Ö, Onal O. Surgical treatment outcomes of pulmonary inflammatory myofibroblastic tumors. Ann Thorac Med 2022; 17:44-50. [PMID: 35198048 PMCID: PMC8809124 DOI: 10.4103/atm.atm_119_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/09/2021] [Revised: 06/25/2021] [Accepted: 07/03/2021] [Indexed: 11/04/2022] Open
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Albayrak HC, Gürler F, Sütçüoğlu O, Akyürek N, Özet A. "How long does crizotinib work? a rare case of recurrent inflammatory myofibroblastic tumor". Anticancer Drugs 2022; 33:109-111. [PMID: 34261914 DOI: 10.1097/cad.0000000000001137] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
Inflammatory myofibroblastic tumors (IMTs) are mesenchymal solid tumors, in which anaplastic lymphoma kinase (ALK) gene rearrangement might be detected. A 48-year-old female presented with IMT of lung, treated with surgery. After a 39-month disease-free survival metastatic recurrence was occurred involving soft tissues both infra- and supradiaphragmatic regions. The biopsies obtained from metastatic regions confirmed the recurrence with ALK rearrangement in immunohistochemistry. Initial partial response observed early in treatment course remained as a stable disease with crizotinib treatment. Although an excellent outcome with overall survival of 57 months was observed in our case, there is not enough information about survivals with crizotinib and the treatment options beyond progression. Therefore, every individual case has a unique value paving the way for more effective treatment.
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Affiliation(s)
| | | | | | - Nalan Akyürek
- Department of Pathology, Gazi University, Ankara, Turkey
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Perkins SJ, Gao R, Glazer TA, Zhao CX, Basura G, McKean EL. Treatment and Prognosis of Inflammatory Pseudotumor of the Skull Base. J Neurol Surg B Skull Base 2021; 83:e555-e563. [DOI: 10.1055/s-0041-1735558] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/12/2021] [Accepted: 07/25/2021] [Indexed: 10/20/2022] Open
Abstract
Abstract
Background Inflammatory pseudotumor (IPT) of the skull base is a rare, locally destructive lesion managed with a variety of treatments. We explore the impact of treatment on outcome and assess the prognosis of IPT.
Methods This is a retrospective review of IPT of the skull base at a tertiary academic medical center. The primary outcome was radiographic progression after treatment. Outcome versus tumor location was also examined and a prognostic model was developed using a logistic regression.
Results The demographics of 21 patients with IPT are reported. Treatment consisted of corticosteroids (in 80.1% of patients), disease modifying antirheumatic drugs (DMARDs; 33.3%), surgical resection (28.6%), radiation (23.8%), antibiotics (14.3%), chemotherapy (rituximab; 9.5%), and antivirals (4.8%). At 50.7 months, 50.8% had radiographic progression. Local therapy trended toward having a better response than systemic therapy (p = 0.60). IPT of the orbit required 2.4 treatment modalities, compared with 2.0 for pharyngeal IPT, and 1.3 for posterior skull base masses (p = 0.14). A total of 75% orbital IPT underwent radiographic progression, compared with 71% of pharyngeal IPT and 50% of posterior skull base masses (p = 0.62). Sixteen patients were used to create the logistic model of radiographic progression. The Cox–Snell R
2 was 0.71 (p = 0.03). No individual variables were statistically significant.
Conclusion To our knowledge, this is among the largest sample of cases describing the presentation, treatment, and prognosis of IPT of the skull base. Our data suggest that there may be an improved response with local therapy over systemic therapy and better prognosis among posterolateral skull base masses.
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Affiliation(s)
- Sidney J. Perkins
- Medical School, University of Michigan Medical School, Ann Arbor, Michigan, United States
| | - Rebecca Gao
- Department of Otolaryngology—Head and Neck Surgery, University of Michigan, Ann Arbor, Michigan, United States
| | - Tiffany A. Glazer
- Department of Surgery, Division of Otolaryngology-Head and Neck Surgery, University of Wisconsin, Madison, Wisconsin, United States
| | - Cher X. Zhao
- Department of Otolaryngology, Harvard Medical School, Pediatric Otolaryngology, Massachusetts Eye and Ear, Boston, MA 02114, United States
| | - Gregory Basura
- Department of Otolaryngology—Head and Neck Surgery, University of Michigan, Ann Arbor, Michigan, United States
| | - Erin L. McKean
- Department of Otolaryngology—Head and Neck Surgery, University of Michigan, Ann Arbor, Michigan, United States
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Farris N, Sampson M. Single-agent rituximab for treatment of multifocal and multiple relapsed pulmonary inflammatory myofibroblastic tumor in an adolescent patient. Pediatr Blood Cancer 2021; 68:e29131. [PMID: 34101979 DOI: 10.1002/pbc.29131] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/05/2021] [Revised: 04/28/2021] [Accepted: 05/07/2021] [Indexed: 11/07/2022]
Affiliation(s)
- Nicholas Farris
- Division of Pediatric Hematology and Oncology, Department of Pediatrics, Akron Children's Hospital, Akron, Ohio, USA
| | - Megan Sampson
- Division of Pediatric Hematology and Oncology, Department of Pediatrics, Akron Children's Hospital, Akron, Ohio, USA
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Oh HH, Joo YE. Rectal inflammatory myofibroblastic tumor: Case report and literature review. Medicine (Baltimore) 2021; 100:e27008. [PMID: 34414993 PMCID: PMC8376367 DOI: 10.1097/md.0000000000027008] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/14/2021] [Accepted: 08/05/2021] [Indexed: 01/04/2023] Open
Abstract
RATIONALE Rectal inflammatory myofibroblastic tumor (IMT) is an extremely rare mesenchymal tumor characterized by a mixture of spindle-shaped myofibroblasts or fibroblasts and inflammatory infiltration of lymphocytes and plasma cells. To date, only 8 cases of rectal IMT have been reported. Herein, we report an additional case of rectal IMT in a 28-year-old woman. PATIENT CONCERNS A 28-year-old woman presented with abdominal pain and hematochezia. DIAGNOSES Colonoscopy showed a 3.0-cm subepithelial tumor with central ulceration, covered by white exudate in the rectum. Rectal magnetic resonance imaging revealed a 4.0 × 3.0-cm-sized well-defined subepithelial tumor in the right wall of the rectum, with suspicious right perirectal fat infiltration. INTERVENTIONS Laparoscopic anterior resection was performed. Microscopic examination of the surgical specimen revealed bland-looking spindle cells intermingled with lymphoplasma cells. Immunohistochemistry and fluorescence in situ hybridization showed anaplastic lymphoma kinase positivity and anaplastic lymphoma kinase positivity rearrangement. Rectal IMT was confirmed based on histological, immunohistochemical, and fluorescence in situ hybridization findings. The patient was doing well without evidence of tumor recurrence 1 year after the surgery. LESSONS Rectal IMT, despite its rarity, should be considered in the differential diagnosis of rectal cancer. Second, an accurate histopathologic diagnosis and complete surgical resection can be the most important approaches to offer a chance for the cure of rectal IMT.
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45
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Mahajan P, Casanova M, Ferrari A, Fordham A, Trahair T, Venkatramani R. Inflammatory myofibroblastic tumor: molecular landscape, targeted therapeutics, and remaining challenges. Curr Probl Cancer 2021; 45:100768. [PMID: 34244015 DOI: 10.1016/j.currproblcancer.2021.100768] [Citation(s) in RCA: 29] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2021] [Accepted: 06/21/2021] [Indexed: 12/29/2022]
Abstract
Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor of intermediate malignant potential that predominantly affects children, adolescents and young adults. IMT has a predilection for the lung, abdomen, pelvis, and retroperitoneum, however, can affect any part of the body. IMT is typically localized, and multifocal or metastatic disease is uncommon. Complete surgical resection is the treatment of choice when feasible. There is no established standard of care for unresectable and advanced IMT. Approximately half of IMTs harbor anaplastic lymphoma kinase (ALK) gene rearrangements, and fusions involving ROS1, PDGFRβ, RET and NTRK have also been described. Given the molecular landscape of IMT, management of these tumors has evolved to include tyrosine kinase inhibitors and novel targeted therapeutics. This review highlights the molecular characteristics, evolution of targeted therapies and the remaining challenges in the management of IMT.
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Affiliation(s)
- Priya Mahajan
- Department of Pediatrics, Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, Houston, Texas
| | - Michela Casanova
- Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy
| | - Andrea Ferrari
- Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy
| | - Ashleigh Fordham
- Children's Cancer Institute, C25 Lowy Cancer Research Centre, UNSW Sydney New South Wales, Australia
| | - Toby Trahair
- Children's Cancer Institute, C25 Lowy Cancer Research Centre, UNSW Sydney New South Wales, Australia; Kids Cancer Centre, Sydney Children's Hospital, Randwick, New South Wales, Australia; School of Women's and Children's Health, UNSW Medicine, New South Wales, Australia
| | - Rajkumar Venkatramani
- Department of Pediatrics, Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, Houston, Texas.
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Jang EJ, Kim KW, Kang SH, Pak MG, Han SH. Inflammatory myofibroblastic tumors arising from pancreas head and peri-splenic area mimicking a malignancy. Ann Hepatobiliary Pancreat Surg 2021; 25:287-292. [PMID: 34053934 PMCID: PMC8180395 DOI: 10.14701/ahbps.2021.25.2.287] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/10/2021] [Revised: 03/04/2021] [Accepted: 03/04/2021] [Indexed: 11/17/2022] Open
Abstract
Inflammatory myofibroblastic tumors (IMTs) are a rare chronic inflammatory disease with unclear pathogenesis and pathological features that are not those of a malignant tumor. It is difficult to differentially diagnose them without surgical excision because of their unpredictable clinical behavior, which ranges from benign to locally invasive aggressiveness. We report two cases of IMTs that were diagnosed after surgery. In one case, the IMT originated in peri-splenic area in a 63-year-old female patient. The other case involved a 48-year-old female patient who suffered from an IMT of the head of the pancreas. Both of these cases did not require further treatment based on histological findings, and there has been no evidence of recurrence or metastasis so far. These cases show that the primary choice for the exact diagnosis and proper treatment of IMTs is complete surgical resection.
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Affiliation(s)
- Eun Jeong Jang
- Department of Surgery, Dong-A University Hospital, Dong-A University College of Medicine, Busan, Korea
| | - Kwan Woo Kim
- Department of Surgery, Dong-A University Hospital, Dong-A University College of Medicine, Busan, Korea
| | - Sung Hwa Kang
- Department of Surgery, Dong-A University Hospital, Dong-A University College of Medicine, Busan, Korea
| | - Min Gyoung Pak
- Department of Pathology, Dong-A University Hospital, Dong-A University College of Medicine, Busan, Korea
| | - Song Hee Han
- Department of Pathology, Dong-A University Hospital, Dong-A University College of Medicine, Busan, Korea
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Shah PA, Babu R, Ramaswamy V, G SK. Inflammatory myofibroblastic tumours of the duodenopancreaticobiliary tract. BMJ Case Rep 2021; 14:e240833. [PMID: 33958358 PMCID: PMC8103835 DOI: 10.1136/bcr-2020-240833] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/27/2021] [Indexed: 11/03/2022] Open
Abstract
Inflammatory myofibroblastic tumours (IMTs) are rare benign neoplasms of intermediate malignant potential that are found in the lungs and rarely at extrapulmonary sites common in children and young adults. IMTs tend to be locally invasive and have some amount of metastatic potential as well. We present two cases of IMTs involving the duodenum, pancreas and distal bile duct. The first case presented with extensive involvement of the first three parts of the duodenum and head of the pancreas, while the second presented with a pancreatic and biliary tree involvement. Upon examinations and investigations, these tumours mimicked malignant neoplasms. A Whipple procedure for surgical resection was undertaken in both cases. The histological findings showed fascicles of spindle cells with infiltration of lymphocytes and plasma cells. The inflammatory myofibroblastic tumour was diagnosed based on pathological grounds with immunohistochemistry. Preoperative diagnosis of IMTs is difficult and complete surgical resection is the primary treatment.
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Affiliation(s)
- Pritik A Shah
- Department of Hepato Pancreato Biliary(HPB) Surgery and Liver Transplant, HealthCare Global Enterprises Ltd, Bangalore, Karnataka, India
| | - Raghavendra Babu
- Department of Hepato Pancreato Biliary(HPB) Surgery and Liver Transplant, HealthCare Global Enterprises Ltd, Bangalore, Karnataka, India
| | - Veena Ramaswamy
- Department of Histopathology, Strand Life Sciences, Bangalore, Karnataka, India
| | - Subash K G
- Department of Hepato Pancreato Biliary(HPB) Surgery and Liver Transplant, HealthCare Global Enterprises Ltd, Bangalore, Karnataka, India
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A Conservative Approach to Inflammatory Myofibroblastic Tumor of the Bladder: A Case Report and Review of Literature. Case Rep Urol 2021; 2021:6660356. [PMID: 33763285 PMCID: PMC7964097 DOI: 10.1155/2021/6660356] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/15/2020] [Revised: 02/21/2021] [Accepted: 02/26/2021] [Indexed: 11/18/2022] Open
Abstract
Inflammatory myofibroblastic tumors (IMTs) are particularly rare tumors that have been described in various anatomic locations, of which the urinary bladder is the most common. These benign tumors are amendable to conservative therapy but are notoriously difficult to diagnose given their mimicry of malignant sarcomas and sarcomatoid carcinomas, making an accurate diagnosis paramount to spare a patient radical and unnecessary treatment. We hereby present the case of a 37-year-old female patient who was diagnosed with an IMT of the urinary bladder during workup for painless gross hematuria. Patient was successfully managed with a laparoscopic partial cystectomy and is free of recurrence 5 years after surgery. IMTs are rare benign tumors that share the same clinical presentation as malignant bladder tumors. Deep biopsy and experienced pathologist are crucial in establishing diagnosis and avoiding patient radical treatment. This case is a classical demonstration of a remarkably rare tumor that was adequately managed with conservative therapy, achieving excellent clinical outcomes.
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49
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Liang Y, Gao HX, Tian RC, Wang J, Shan YH, Zhang L, Xie CJ, Li JJ, Xu M, Gu S. Inflammatory myofibroblastic tumor successfully treated with metformin: A case report and review of literature. World J Clin Cases 2021; 9:429-435. [PMID: 33521112 PMCID: PMC7812890 DOI: 10.12998/wjcc.v9.i2.429] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/21/2020] [Revised: 11/20/2020] [Accepted: 11/29/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Inflammatory myofibroblastic tumor (IMT) is a distinct tumor with a low incidence rate, which can be diagnosed at any age with a predilection for children and adolescents. Although IMT is visible in any tissues and organs, it is more commonly found in the lungs. The clinical and radiological manifestations of IMT lack specificity, hence resulting in frequent misdiagnosis. Surgical resection is currently the main therapeutic approach for IMT. Only scarce cases of IMT treated with metformin have been reported. Here we report the case of an IMT patient with partial penile resection treated with metformin.
CASE SUMMARY A 1-year-old boy was born with a shorter penis, and his foreskin could not be completely turned over. When he was 6 month old, a well-circumscribed mass on the glans was found, while it did not attract the attention of his parents. The mass gradually increased in size over time before he was admitted to the hospital, where physical examination was performed. It was revealed that the glans hidden behind the foreskin had a mass with a diameter of about 4 cm surrounding the penis. The mass appeared to be hard with a smooth surface and poor mobility. The two testicles examined at the bottom of the scrotum were revealed to have a normal size. Magnetic resonance imaging showed a tumor with rich blood supply encircling the cavernosum with a size of 3.5 cm × 2.1 cm × 2.0 cm. A thick urinary line was found without urine dripping, urgency, and urodynia. Surgical treatment was performed. During the operation, it was observed that the mass had surrounded and invaded the cavernosum without obvious boundaries, and that the tumor occupied about one-half of the penis cross-section as well as infiltrated more than one-half of the glans. In addition, the tumor had caused urethral invasion and anterior urethrostenosis. With the intention of keeping the glans and cavernosum, the tumor at the anterior urethra was partially removed, leaving about 30% of the tumor mass. Pathology analysis demonstrated that the tumor was rich in spindle cells with infiltration of inflammatory cells. Immuno-histochemistry analysis indicated that the cells were positive for CD4, CD99, Ki67, BCL2, and CD68, and negative for ALK, MyoG, S100, SOX10, PR, and EMA. Hence, the tumor was diagnosed as IMT. Metformin was prescribed for the patient after the operation, following which an oral dose of 7 mg/kg was given three times a day after meals. Three months later, it was observed that the remaining tumor had completely disappeared and that the urination process from the urethra opening had resumed normal. In addition, there were no side effects observed. There was also no tumor recurrence. The growth and development of the boy were unaffected as a result of the treatment.
CONCLUSION The tumor was observed to have completely disappeared after treatment with metformin. Our finding is of great significance to facilitate future clinical treatment with IMT.
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Affiliation(s)
- Yu Liang
- Department of Surgery, Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China
| | - Hong-Xiang Gao
- Department of Surgery, Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China
| | - Rui-Cheng Tian
- Department of Surgery, Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China
| | - Jing Wang
- Department of Surgery, Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China
| | - Yu-Hua Shan
- Department of Surgery, Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China
| | - Lei Zhang
- Department of Surgery, Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China
| | - Chen-Jie Xie
- Department of Surgery, Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China
| | - Jing-Jing Li
- Department of Surgery, Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China
| | - Min Xu
- Department of Surgery, Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China
| | - Song Gu
- Department of Surgery, Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China
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Bompas E, Martin V, Meniai F, Toulmonde M, Marec-Berard P, Claude L, Ducimetiere F, Chargari C, Minard-Colin V, Corradini N, Laurence V, Piperno-Neumann S, Defachelles AS, Bernier V, Italiano A, Orbach D, Blay JY, Gaspar N, Berlanga P. Management of sarcomas in children, adolescents and adults: Interactions in two different age groups under the umbrellas of GSF-GETO and SFCE, with the support of the NETSARC+ network. Bull Cancer 2021; 108:163-176. [PMID: 33455736 DOI: 10.1016/j.bulcan.2020.10.009] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/11/2020] [Revised: 10/17/2020] [Accepted: 10/27/2020] [Indexed: 11/17/2022]
Abstract
Sarcomas are a rare heterogeneous group of malignant neoplasms that can arise in almost any anatomic site and any age. Close collaboration among adult and pediatric cancer specialists in the management of these tumors is of foremost importance. In this review, we present the current multidisciplinary organization in care of patients with sarcoma in France and we review the main advances made in the last decades in systemic and radiotherapy treatment in the main sarcoma types diagnosed in children, adolescents and young adults (AYA), thanks to the international collaboration.
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Affiliation(s)
- Emmanuelle Bompas
- Centre René Gauducheau, Medical Oncology Department, Saint-Herblain, France
| | - Valentine Martin
- Department of Radiation Oncology, Gustave Roussy, Villejuif, France
| | - Fatima Meniai
- University Lille, Inserm, U1189 - ONCO-THAI - Assisted Laser Therapy and Immunotherapy for Oncology, 59000 Lille, France
| | - Maud Toulmonde
- Institut Bergonié, Medical Oncology Department, Bordeaux, France
| | | | - Line Claude
- Centre Léon Bérard, Department of radiotherapy, Lyon, France
| | | | - Cyrus Chargari
- Department of Radiation Oncology, Gustave Roussy, Villejuif, France
| | | | - Nadège Corradini
- Centre Léon Bérard, Department of Pediatric Oncology, Lyon, France
| | | | | | | | - Valérie Bernier
- Oncology Radiotherapy Institut de Cancérologie de Lorraine, Vandœuvre-lès-Nancy, France
| | | | - Daniel Orbach
- Department of Pediatric Oncology, Institut Curie, Paris, France
| | - Jean-Yves Blay
- Medical Oncology Department, Centre Léon-Bérard, Lyon, France
| | - Nathalie Gaspar
- Department of Pediatric and Adolescent Oncology, Gustave Roussy, Villejuif, France
| | - Pablo Berlanga
- Department of Pediatric and Adolescent Oncology, Gustave Roussy, Villejuif, France.
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