|
1
|
Zhou L, Pan W, Huang R, Lu Z, You Z, Li Y. Intracranial Inflammatory Myofibroblastic Tumor: A Literature Review and a Rare Case Misdiagnosed as Acoustic Neuroma. Diagnostics (Basel) 2023; 13:2725. [PMID: 37685263 PMCID: PMC10486536 DOI: 10.3390/diagnostics13172725] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2023] [Revised: 08/11/2023] [Accepted: 08/12/2023] [Indexed: 09/10/2023] Open
Abstract
Inflammatory myofibroblastic tumor (IMT) stands as a rare neoplasm, initially documented by Bahadori and Liebow in 1973; however, its biological behavior and underlying pathogenesis continue to elude comprehensive understanding. Throughout the years, this tumor has been designated by various alternative names, including pseudosarcomatoid myofibroblastoma, fibromyxoid transformation, and plasma cell granuloma among others. In 2002, the World Health Organization (WHO) officially classified it as a soft tissue tumor and designated it as IMT. While IMT primarily manifests in the lungs, the common clinical symptoms encompass anemia, low-grade fever, limb weakness, and chest pain. The mesentery, omentum, and retroperitoneum are subsequent sites of occurrence with intracranial involvement being exceedingly rare. Due to the absence of specific clinical symptoms and characteristic radiographic features, diagnosing intracranial inflammatory myofibroblastic tumor (IIMT) remains challenging. Successful instances of pharmacological treatment for IIMT indicate that surgery may not be the sole therapeutic recourse, thus underscoring the imperative of an accurate diagnosis and apt treatment selection to improve patient outcomes.
Collapse
Affiliation(s)
- Le Zhou
- Department of Radiology, The First Affiliated Hospital of Soochow University, Suzhou 215000, China; (L.Z.); (R.H.); (Z.L.)
| | - Wanqian Pan
- Department of Cardiology, The First Affiliated Hospital of Soochow University, 188 Shizi Street, Suzhou 215006, China;
| | - Renjun Huang
- Department of Radiology, The First Affiliated Hospital of Soochow University, Suzhou 215000, China; (L.Z.); (R.H.); (Z.L.)
| | - Ziwei Lu
- Department of Radiology, The First Affiliated Hospital of Soochow University, Suzhou 215000, China; (L.Z.); (R.H.); (Z.L.)
| | - Zhiqun You
- Department of Pathology, The First Affiliated Hospital of Suzhou University, Suzhou 215000, China;
| | - Yonggang Li
- Department of Radiology, The First Affiliated Hospital of Soochow University, Suzhou 215000, China; (L.Z.); (R.H.); (Z.L.)
- Institute of Medical Imaging, Soochow University, Suzhou 215000, China
- National Clinical Research Center for Hematologic Diseases, The First Affiliated Hospital of Soochow University, Suzhou 215000, China
| |
Collapse
|
|
2
|
Ramos JG, Ochoa A, Cicutti S, Del Rio R, Lubienieki F. Inflammatory myofibroblastic tumor of the lung involving the central nervous system in pediatric patients. Childs Nerv Syst 2023; 39:1137-1145. [PMID: 37017800 DOI: 10.1007/s00381-023-05925-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/11/2023] [Accepted: 03/14/2023] [Indexed: 04/06/2023]
Abstract
INTRODUCTION The inflammatory myofibroblastic tumor (IMT) is a very rare lesion with an incidence of less than 0.1% of total neoplasms and with main affection in the lungs. Involvement in the central nervous system is extremely rare, but with a much more aggressive course than IMT diagnosed in the rest of the body. We report the 2 cases presented in our neurosurgery department to date; both were treated satisfactorily without intercurrences in 10 years of follow-up. HISTORICAL BACKGROUND The World Health Organization described the IMT as a distinctive lesion composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. CLINICAL PRESENTATION Clinical manifestations of patients with CNS IMT vary and may consist of headache, vomiting, seizures, and blindness. Seizures are the most common symptom in patients with focal lesions. DIAGNOSIS The true origin of this entity remains to be elucidated, but to date, etiologies ranging from chromosomal alterations to autoimmune or postinfectious mechanisms have been described. Due to its rarity and non-specificity in imaging, the final diagnosis of IMT in the brain parenchyma relies on pathological examination. MANAGEMENT Treatment options are controversial and include total or subtotal removal, high-dose steroids, and radiation therapy. In the last decade, the development of ALK Tyrosine Kinase Inhibitors allows the possibility of chemotherapy in those patients harboring ALK mutations. CONCLUSION IMT is a rare tumor that can exceptionally be found in the CNS. The cause is still unknown although the different studies focus on a neoplastic origin. The diagnosis is based in the use of different modalities of imaging and with histological confirmation. Optimal management is gross total resection whenever possible, is the only established curative treatment. Further research with longer follow-up is needed to clarify the natural history of this rare tumor.
Collapse
Affiliation(s)
- Javier Gonzalez Ramos
- Division of Pediatric Neurosurgery, Hospital de Pediatría Juan P. Garrahan, Combate de los Pozos 1881, ZC: 1245, Buenos Aires, Argentina.
| | - Adalberto Ochoa
- Division of Pediatric Neurosurgery, Hospital de Pediatría Juan P. Garrahan, Combate de los Pozos 1881, ZC: 1245, Buenos Aires, Argentina
| | - Santiago Cicutti
- Division of Pediatric Neurosurgery, Hospital de Pediatría Juan P. Garrahan, Combate de los Pozos 1881, ZC: 1245, Buenos Aires, Argentina
| | - Ramiro Del Rio
- Division of Pediatric Neurosurgery, Hospital de Pediatría Juan P. Garrahan, Combate de los Pozos 1881, ZC: 1245, Buenos Aires, Argentina
| | - Fabiana Lubienieki
- Division of Pathology, Hospital de Pediatría Juan P. Garrahan, Buenos Aires, Argentina
| |
Collapse
|
|
3
|
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.
Collapse
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
| |
Collapse
|
|
4
|
Tang XL, Huang WJ, Ma Q, Yi KM. Advanced MRI features of intraventricular inflammatory myofibroblastic tumor: a case report. BMC Neurol 2022; 22:448. [PMID: 36460992 PMCID: PMC9717528 DOI: 10.1186/s12883-022-02993-8] [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] [Received: 12/24/2021] [Accepted: 11/25/2022] [Indexed: 12/04/2022] Open
Abstract
BACKGROUND Inflammatory myofibroblastic tumor (IMT) is a rare central nervous system (CNS) tumor. We first report a rare case of IMT in the lateral ventricle and describe the magnetic resonance imaging (MRI) findings of the tumor with an emphasis on the advanced MRI features. CASE PRESENTATION A 49-year-old female patient with headaches and blurred vision for 2 months. Brain MRI revealed a well-circumscribed, lobulated mass occupying the left lateral ventricle trigone, with marked perilesional brain edema. The tumor showed heterogeneous significant hyperintensity on T2-weighted imaging (T2WI) and hypointensity on T1-weighted imaging (T1WI). After the administration of gadolinium, the mass exhibited marked contrast enhancement and the halo sign was observed. On advanced MRI, the lesion showed decreased perfusion on perfusion MRI and reduced diffusion on diffusion-weighted imaging (DWI). On susceptibility-weighted imaging (SWI), there was a punctate low signal intensity in the tumor. The patient underwent surgical resection of the mass and a pathological examination confirmed the lesion to be an inflammatory myofibroblastic tumor with negative expression of anaplastic lymphoma kinase (ALK). This patient had remained healthy without evidence of recurrence during a 20-month follow-up. CONCLUSIONS On MRI, marked perilesional brain edema, significant hyperintensity on T2WI, hypoperfusion on perfusion MRI but with an obvious enhancement, no diffusion restriction on DWI, and halo sign may be the characteristic findings of intraventricular IMT. The advanced MRI characteristics could provide abundant information to reflect the histological features and physiological metabolic characteristics of the tumor.
Collapse
Affiliation(s)
- Xiao-lin Tang
- grid.414048.d0000 0004 1799 2720Department of Radiology, Daping Hospital, Army Medical University, Chongqing, 400042 China ,Chongqing Clinical Research Centre of Imaging and Nuclear Medicine, Chongqing, 400042 China
| | - Wen-jun Huang
- Department of Radiology, Traditional Chinese Medicine Hospital of Banan District, Chongqing, 401320 China
| | - Qiang Ma
- grid.414048.d0000 0004 1799 2720Department of Pathology, Daping Hospital, Army Medical University, Chongqing, 400042 China
| | - Kun-ming Yi
- grid.414048.d0000 0004 1799 2720Department of Radiology, Daping Hospital, Army Medical University, Chongqing, 400042 China ,Chongqing Clinical Research Centre of Imaging and Nuclear Medicine, Chongqing, 400042 China
| |
Collapse
|
|
5
|
Malnik SL, Moor RF, Shin D, Laurent D, Trejo-Lopez J, Dodd W, Yachnis A, Ghiaseddin AP, Fox WC, Roper S. Inflammatory myofibroblastic tumor masquerading as an anterior choroidal artery fusiform aneurysm. Surg Neurol Int 2021; 12:297. [PMID: 34221627 PMCID: PMC8247755 DOI: 10.25259/sni_113_2021] [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: 02/06/2021] [Accepted: 03/16/2021] [Indexed: 11/12/2022] Open
Abstract
Background: Inflammatory myofibroblastic tumor is a rare, poorly understood tumor that has been found to occur in almost every organ tissue. Its location within the central nervous system is uncommon, and patients tend to present with nonspecific symptoms. Case Description: A female in her eighth decade presented to neurosurgery clinic with complaints of headache and dizziness. Initial imaging was consistent with a low-grade, benign brain lesion in the region of the left choroidal fissure. She was recommended for observation but returned 1 month later with progressive symptoms and doubling of the lesion size. She underwent surgical resection and was found to have an IMT arising from the wall of the left anterior choroidal artery. Conclusion: Intracranial IMT remains a rare and poorly understood entity. The present case demonstrates a novel presentation of IMT in an adult patient and exemplifies the heterogeneity of the disease presentation.
Collapse
Affiliation(s)
- Samuel Louis Malnik
- Lillian S. Wells Department of Neurosurgery, University of Florida, Gainesville, Florida, United States
| | - Rachel Freedman Moor
- Lillian S. Wells Department of Neurosurgery, University of Florida, Gainesville, Florida, United States
| | - David Shin
- Lillian S. Wells Department of Neurosurgery, University of Florida, Gainesville, Florida, United States
| | - Dimitri Laurent
- Lillian S. Wells Department of Neurosurgery, University of Florida, Gainesville, Florida, United States
| | - Jorge Trejo-Lopez
- Department of Neuropathology, Mayo Clinic, Rochester, Minnesota, United States
| | - William Dodd
- Lillian S. Wells Department of Neurosurgery, University of Florida, Gainesville, Florida, United States
| | - Anthony Yachnis
- Department of Pathology, University of Florida, Gainesville, United States
| | - Ashley P Ghiaseddin
- Lillian S. Wells Department of Neurosurgery, University of Florida, Gainesville, Florida, United States
| | - W Christopher Fox
- Department of Neurosurgery, Mayo Clinic, Jacksonville, Florida, United States
| | - Steven Roper
- Lillian S. Wells Department of Neurosurgery, University of Florida, Gainesville, Florida, United States
| |
Collapse
|
|
6
|
Wang X, Chen Y, Wu X, Zhang H. Intracranial Inflammatory Myofibroblastic Tumor with Negative Expression of Anaplastic Lymphoma Kinase: A Case Report and Review of the Literature. World Neurosurg 2019; 125:117-122. [PMID: 30735872 DOI: 10.1016/j.wneu.2019.01.155] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2018] [Revised: 01/24/2019] [Accepted: 01/25/2019] [Indexed: 12/12/2022]
Abstract
BACKGROUND Inflammatory myofibroblastic tumor (IMT) is an indolent tumor mainly affecting children and young adults. As a rare mesenchymal tumor with unknown etiology and pathogenesis, IMT has a predilection for the lung and abdominopelvic region. Previous literature featuring IMT in the central nervous system (IMT-CNS) is rare. The clinical symptoms and radiologic features of IMT-CNS are not specific; therefore, the diagnosis is predominately based on the histopathologic and immunohistochemical analysis of the specimen. CASE DESCRIPTION We herein present a case of a 21-year-old woman who complained of bilateral blurred vision for 15 days. Head magnetic resonance imaging demonstrated a round-shaped and irregular lesion located in the right frontal lobe. The boundary of the lesion was clear, and the lesion was homogeneously enhanced. Peripheral edema of the lesion was observed, and the mass effect was obvious. Supratentorial craniotomy tumor resection was performed. Histopathologic and immunohistochemical analysis revealed IMT, which had negative expression of anaplastic lymphoma kinase. CONCLUSIONS Remission of her symptoms was observed, and no recurrence was recorded during a 6-month follow-up.
Collapse
Affiliation(s)
- Xiaoke Wang
- Department of Neurosurgery, the Second Hospital of Jilin University, Jilin University, Changchun, China
| | - Yang Chen
- Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China
| | - Xiujuan Wu
- Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China
| | - Hongliang Zhang
- Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China.
| |
Collapse
|
|
7
|
Scarpante E, Palus V, Summers BA, Caine A, Cherubini GB. IMAGING DIAGNOSIS - MAGNETIC RESONANCE IMAGING OF INTRACRANIAL INFLAMMATORY FIBROSARCOMA IN A MIXED BREED DOG. Vet Radiol Ultrasound 2015; 57:E5-9. [PMID: 26304143 DOI: 10.1111/vru.12286] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2014] [Accepted: 03/27/2015] [Indexed: 11/28/2022] Open
Abstract
An 8-year-old mixed-breed dog presented with progressive behavioral changes and altered mentation. Magnetic resonance imaging (MRI) of the brain revealed an olfactory and frontal lobe extra-axial mass. The mass exhibited the following MRI signal intensity characteristics: T2W mixed, T1W iso- to hypointense, FLAIR hyperintense, and strong contrast enhancement. The mass was removed with cavitronic ultrasonic surgical aspirator (CUSA) assisted neurosurgery. Based on histopathological appearance and immunohistochemistry, the diagnosis of inflammatory fibrosarcoma was made. To our knowledge, this is the first report describing MRI characteristics of intracranial inflammatory fibrosarcoma in the veterinary literature.
Collapse
Affiliation(s)
- Elena Scarpante
- Dick White Referrals, London Road, Six Mile Bottom, Cambridgeshire, CB8 0UH
| | - Viktor Palus
- Dick White Referrals, London Road, Six Mile Bottom, Cambridgeshire, CB8 0UH
| | | | - Abby Caine
- Dick White Referrals, London Road, Six Mile Bottom, Cambridgeshire, CB8 0UH
| | | |
Collapse
|
|
8
|
Denis DJ, Elayoubi K, Weil AG, Berthelet F, Bojanowski MW. Inflammatory myofibroblastic tumors of the central nervous system that express anaplastic lymphoma kinase have a high recurrence rate. Surg Neurol Int 2013; 4:70. [PMID: 23776756 PMCID: PMC3683168 DOI: 10.4103/2152-7806.112614] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2013] [Accepted: 04/26/2013] [Indexed: 12/12/2022] Open
Abstract
Background: Inflammatory myofibroblastic tumors (IMTs) of the central nervous system (CNS) are rare entities with diverse histopathological features and varying propensities to recur. Case Description: A 26 year-old male with an IMT of the CNS of the left tentorium had tumor progression 2 months after partial surgical resection. Histopathological studies confirmed expression of ALK. Macroscopic total resection was performed followed by radiotherapy. A recurrence occurred 20 months after the second surgery that necessitate reoperation. Including the present case, we identified 30 cases of IMT of the CNS corresponding to our search criteria in the literature. The extent of resection was reported in 26 of these cases. Gross total resection was done in 75% of ALK-positive and in 61% of ALK-negative cases. Recurrence rate after gross total resection for ALK-positive and ALK-negative cases was 33% and 9%, respectively. Every recurrence in ALK-positive patients occurred within 2 years after surgery. Conclusion: IMT of the CNS are a heterogeneous group of tumors and the treatment of choice is complete surgical resection. Because of the high recurrence rate reported for IMT of the CNS expressing ALK, a closed follow-up is recommended. When faced with an early recurrence, a surgical resection followed by radiotherapy may be advised.
Collapse
Affiliation(s)
- Daniel J Denis
- Department of Surgery, Division of Neurosurgery, Centre Hospitalier de l'Université de Montréal, Hôpital Notre-Dame, Montreal, QC, Canada
| | | | | | | | | |
Collapse
|
|
9
|
Fan F, Lei C, Dong-Liang L, Peng S. Inflammatory myofibroblastic tumor mimicking malignant meningioma in the middle cranial fossa: a case report. ACTA ACUST UNITED AC 2012; 27:185-7. [PMID: 23062643 DOI: 10.1016/s1001-9294(14)60054-7] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022]
Abstract
INFLAMMATORY myofibroblastic tumor (IMT), also known as inflammatory pseudotumor or plasma cell granulomas, is an uncommon fibro-inflammatory lesion which is composed of inflammatory cells and myofibroblastic spindle cells. Its pathogenesis is still unknown. The tumor commonly occurs in the lung, upper respiratory tract, live, orbit, abdominal membrane, retroperitoneum, and genitourinary tract.It rarely involves the central nerve system, especially the middle cranial fossa. As far as we know, there are no more than 5 reports of IMT that developed in the middle cranial fossa without involving temporal bone. IMT can occur at any age but primarily affects children and young adults. It is difficult to diagnose IMT by clinical and imaging characteristics only. Here we present an 82-year-old female with intracranial IMT arising from the meninges and without involving temporal bone, which was diagnosed as malignant meningioma before operation.
Collapse
Affiliation(s)
- Fan Fan
- Department of Neurosurgical Intensive Care Unit,the Affiliated Hospital of Medical College, Qingdao University, Shandong 266003, China
| | | | | | | |
Collapse
|