|
1
|
Øines MN, Smith HG, Preisler L, Penninga L. Leiomyosarcoma of the abdomen and retroperitoneum; a systematic review. Front Surg 2024; 11:1375483. [PMID: 39086921 PMCID: PMC11288885 DOI: 10.3389/fsurg.2024.1375483] [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: 01/23/2024] [Accepted: 06/24/2024] [Indexed: 08/02/2024] Open
Abstract
Background Intraabdominal and retroperitoneal leiomyosarcomas are rare cancers, which cause significant morbidity and mortality. Symptoms, treatment and follow up differs from other cancers, and proper diagnosis and treatment of intraabdominal and retroperitoneal leiomyosarcomas is of utmost importance. We performed a systematic review to collect and summarize available evidence for diagnosis and treatment for these tumours. Methods We performed a systematic literature search of Pubmed from the earliest entry possible, until January 2021. Our search phrase was (((((colon) OR (rectum)) OR (intestine)) OR (abdomen)) OR (retroperitoneum)) AND (leiomyosarcoma). All hits were evaluated by two of the authors. Results Our predefined search identified 1983 hits, we selected 218 hits and retrieved full-text copies of these. 144 studies were included in the review. Discussion This review summarizes the current knowledge and evidence on non-uterine abdominal and retroperitoneal leiomyosarcomas. The review has revealed a lack of high-quality evidence, and randomized clinical trials. There is a great need for more substantial and high-quality research in the area of leiomyosarcomas of the abdomen and retroperitoneum. Systematic Review Registration PROSPERO, identifier, CRD42023480527.
Collapse
Affiliation(s)
- Mari Nanna Øines
- Department of Surgery and Transplantation, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark
| | | | - Louise Preisler
- Department of Surgery and Transplantation, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark
- Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark
| | - Luit Penninga
- Department of Surgery and Transplantation, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark
- Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark
| |
Collapse
|
|
2
|
Annicchiarico A, Montali F, Baldinu M, Casali L, Virgilio E, Costi R. Leiomyosarcoma of the rectum: A systematic review of recent literature. J Surg Oncol 2024; 129:365-380. [PMID: 37814590 DOI: 10.1002/jso.27481] [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: 07/11/2023] [Revised: 09/19/2023] [Accepted: 09/28/2023] [Indexed: 10/11/2023]
Abstract
Leiomyosarcomas (LMSs) are rare tumors originating from the muscular layer. We performed a literature review of cases of confirmed rectal leiomyosarcomas (rLMSs) to clarify the history of such an infrequent tumor arising at such an uncommon location. In this research local recurrence was related to poorly differentiated rLMS and no other association between recurrence and any criteria was found. Concerning overall survival (OS), rLMS patients developing recurrence presented shorter longevity compared with the group without.
Collapse
Affiliation(s)
- Alfredo Annicchiarico
- Department of Medicine and Surgery, University of Parma, Parma, Italy
- Department of General Surgery, Vaio Hospital, Fidenza, Italy
| | - Filippo Montali
- Department of General Surgery, Vaio Hospital, Fidenza, Italy
| | - Manuel Baldinu
- Department of Medicine and Surgery, University of Parma, Parma, Italy
| | - Lorenzo Casali
- Department of General Surgery, Vaio Hospital, Fidenza, Italy
| | - Edoardo Virgilio
- Department of Medicine and Surgery, University of Parma, Parma, Italy
- Department of General Surgery, Vaio Hospital, Fidenza, Italy
| | - Renato Costi
- Department of Medicine and Surgery, University of Parma, Parma, Italy
- Department of General Surgery, Vaio Hospital, Fidenza, Italy
| |
Collapse
|
|
3
|
González-Díaz E, Cengotitabengoa BG, Carbajo ABD, Fernández CF, Corona AF. Imaging Diagnosis of Perianal Leiomyoma: A Case Report. Ann Coloproctol 2021; 37:S58-S62. [PMID: 34107598 PMCID: PMC8359697 DOI: 10.3393/ac.2020.10.13.1] [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: 07/14/2020] [Accepted: 10/13/2020] [Indexed: 11/24/2022] Open
Abstract
A leiomyoma is a benign mesenchymal neoplasm that usually develops where smooth muscle is present. In this report, we present the case of a 75-year-old woman who sought medical attention due to pelvic organ prolapse and urinary incontinence, and without defecatory symptoms. Both pelvic floor ultrasound and magnetic resonance imaging (MRI) examination showed a well-defined mass in the ischioanal fossa, eventually diagnosed as a perianal leiomyoma. It is considered a rare entity because of its location involving the anal sphincter and clinical features. We describe the ultrasonographic and MRI findings of this perianal leiomyoma, together with differential diagnoses and immunohistochemical characterization.
Collapse
Affiliation(s)
- Enrique González-Díaz
- Pelvic Floor Unit, Department of Obstetrics and Gynaecology, Complejo Asistencial Universitario de León (CAULE), León, Spain
| | | | | | - Camino Fernández Fernández
- Department of Obstetrics and Gynaecology, Complejo Asistencial Universitario de León (CAULE), León, Spain
| | - A Fernández Corona
- Department of Obstetrics and Gynaecology, Complejo Asistencial Universitario de León (CAULE), León, Spain
| |
Collapse
|
|
4
|
Perry L, Chew L, Biebel C, Senatore P. Fibroepithelial Spindle Cell Tumor of the Rectum. Am Surg 2020; 88:2400-2401. [PMID: 32909444 DOI: 10.1177/0003134820951446] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Affiliation(s)
- Luke Perry
- Department of Colorectal Surgery, Inspira Health Network, Vineland, NJ, USA
| | - Lauren Chew
- Department of Colorectal Surgery, Inspira Health Network, Vineland, NJ, USA
| | - Craig Biebel
- Department of Colorectal Surgery, Inspira Health Network, Vineland, NJ, USA
| | - Peter Senatore
- Department of Colorectal Surgery, Inspira Health Network, Vineland, NJ, USA
| |
Collapse
|
|
5
|
Nassif MO, Habib RA, Almarzouki LZ, Trabulsi NH. Systematic review of anorectal leiomyosarcoma: Current challenges and recent advances. World J Gastrointest Surg 2019; 11:334-341. [PMID: 31523383 PMCID: PMC6715585 DOI: 10.4240/wjgs.v11.i8.334] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/28/2019] [Revised: 08/10/2019] [Accepted: 08/13/2019] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND The anorectal leiomyosarcoma (LMS) is an aggressive malignant neoplasm. Owing to the rarity of LMSs, an optimal treatment modality has yet to be determined.
AIM To collect all published data on anorectal LMS characteristics, explore current treatment options, and review recent cases of postradiation LMS.
METHODS A literature search of the PubMed electronic database was conducted using the MeSH terms “rectal neoplasms”, “anus neoplasms” and “gastrointestinal neoplasms” combined with “leiomyosarcoma”. The search was limited to English language and human studies. All available case reports and case series of anal or rectal LMSs that were published from the beginning of January 1996 to May 2017 were included if the diagnosis of LMS had been confirmed by histopathologic examination. Data were analyzed using simple statistics (mean, median, and standard deviation). Independent sample t-test was used to compare means for continuous variables.
RESULTS A total of 27 articles reporting on 51 cases of anorectal LMS were identified. Among these cases, 11.7% had undergone previous pelvic radiotherapy (developing LMS at 13-35 years afterwards). Anorectal LMS affected the rectum in 92.2% of the cases, and no sex-based predominance was observed. Surgical resection with negative margins remains the mainstay of treatment, which can be accomplished with wide local excision or radical resection. The local recurrence rate was higher among cases who received wide local excision (30%), as compared to radical resection (20%); however, the overall rate of metastasis was 51.61% regardless of the treatment approach. The use of neoadjuvant radiation lowers the risk of local recurrence compared to adjuvant radiotherapy, and facilitates R0 resection of the tumor. Cases treated with adjuvant chemotherapy showed better rates of distant recurrence and overall survival. Nonetheless, multidisciplinary team discussion is necessary to determine the optimal management plan whilst considering patient- and disease-related factors.
CONCLUSION A multidisciplinary team approach, considering the underlying patient- and disease- related factors, is necessary for optimal management of these complex tumors.
Collapse
Affiliation(s)
- Mohammed O Nassif
- Department of Surgery, King Abdulaziz University, Jeddah 21589, Saudi Arabia
| | - Razan A Habib
- Department of Surgery, King Abdulaziz University, Jeddah 21589, Saudi Arabia
| | - Luai Z Almarzouki
- Department of Surgery, King Abdulaziz University, Jeddah 21589, Saudi Arabia
| | - Nora H Trabulsi
- Department of Surgery, King Abdulaziz University, Jeddah 21589, Saudi Arabia
| |
Collapse
|
|
6
|
Sahli N, Khmou M, Khalil J, Elmajjaoui S, El Khannoussi B, Kebdani T, Elkacemi H, Benjaafar N. Unusual evolution of leiomyosarcoma of the rectum: a case report and review of the literature. J Med Case Rep 2016; 10:249. [PMID: 27633779 PMCID: PMC5025574 DOI: 10.1186/s13256-016-1047-8] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/29/2016] [Accepted: 08/29/2016] [Indexed: 01/01/2023] Open
Abstract
Background Leiomyosarcoma of the rectum is a rare entity that comprises less than 0.1 % of all rectal malignancies. Given the uncommon nature of this tumor and the controversy about its treatment we report one case and review the literature in an attempt to report a particular evolution and to discuss the most appropriate treatment. Case presentation This case report describes the presentation of leiomyosarcoma of the rectum. A 30-year-old man from the north of Morocco presented with rectorrhagia and constipation. On physical examination we found a mass in his rectum approximately 6 cm from his anal margin. Pelvic magnetic resonance imaging showed a rectal mass with a parietal attachment that invaded the fascia and his perirectal tissue. Before any treatment he defecated spontaneously the tumor. On histopathological examination a diagnosis of leiomyosarcoma was made. An anterior resection of his rectum was performed with adjuvant radiotherapy at a dose of 50 Gy. After 1 year of surveillance, he has not presented any clinical symptoms and pelvic magnetic resonance imaging was normal. Unfortunately, histological analysis of a superficial biopsy of a rectal leiomyosarcoma may not be reflective of the entire tumor mass, and a diagnosis is based essentially on postoperative pathological examination. The optimal treatment modality in patients with rectal leiomyosarcomas is controversial. Prognosis is also poor; tumor size, histological grade, mitotic index, and local staging are the most known prognosis factors. Conclusion The prognosis of rectal leiomyosarcoma is poor; more investigations are necessary to understand the progression of these tumors and to define an optimal treatment modality.
Collapse
Affiliation(s)
- N Sahli
- Department of Radiotherapy, National Institute of Oncology, University Mohammed V, Rabat, Morocco.
| | - M Khmou
- Department of Pathology, National Institute of Oncology, University Mohammed V, Rabat, Morocco
| | - J Khalil
- Department of Radiotherapy, National Institute of Oncology, University Mohammed V, Rabat, Morocco
| | - S Elmajjaoui
- Department of Radiotherapy, National Institute of Oncology, University Mohammed V, Rabat, Morocco
| | - B El Khannoussi
- Department of Pathology, National Institute of Oncology, University Mohammed V, Rabat, Morocco
| | - T Kebdani
- Department of Radiotherapy, National Institute of Oncology, University Mohammed V, Rabat, Morocco
| | - H Elkacemi
- Department of Radiotherapy, National Institute of Oncology, University Mohammed V, Rabat, Morocco
| | - N Benjaafar
- Department of Radiotherapy, National Institute of Oncology, University Mohammed V, Rabat, Morocco
| |
Collapse
|
|
7
|
Malignant Rectal Gastrointestinal Stromal Tumour: Case Report and Review of Literature. J Gastrointest Cancer 2016; 43 Suppl 1:S191-3. [PMID: 22399199 DOI: 10.1007/s12029-012-9377-2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/28/2022]
|
|
8
|
A rare case of leiomyoma of the internal anal sphincter. Int J Surg Case Rep 2016; 23:4-7. [PMID: 27078867 PMCID: PMC4855499 DOI: 10.1016/j.ijscr.2016.03.048] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/24/2016] [Accepted: 03/29/2016] [Indexed: 11/23/2022] Open
Abstract
INTRODUCTION Leiomyoma is a benign tumour which derives from the smooth muscle fibres and it may occurs in every site in which this type of muscle is present. Among all benign soft tissue tumours it represents almost 3.8% and its pathogenesis remains still unknown. PRESENTATION OF CASE The present case is about a 62year old woman referred to our centre complaining anal and perineal pain which increase after defecation in association with the appearance of a nodule in the perianal region fixed to the anal sphincter. A 360° tridimensional transanal ultrasound was performed and it showed an anterior nodular thickening of the internal anal sphincter. After an inconclusive preoperative biopsy and a counselling with the patient, the surgeons decided to proceed with the surgical excision. The immunohistochemical examination confirmed the preoperative suspicion of leiomyoma. At 1year follow-up the patient had not tumour-related symptoms or fecal incontinence and any signs of local recurrence at ultrasound imaging were demonstrated. DISCUSSION Leiomyomas are relatively insensitive to chemotherapy whereby surgery is the treatment of choice and it should be adequate to the site and dimension of the lesion achieving a complete resection with free margins. A further close follow-up is needed too. CONCLUSION Nowadays there is not a gold standard technique to treat such kind of lesions and the decision of the best surgical approach should depend on the dimension and site. In fact, surgery aims to the oncological outcome trying also to minimize the possible post-operative functional complications.
Collapse
|
|
9
|
Matsuhashi N, Takahashi T, Ichikawa K, Tanahashi T, Sasaki Y, Tanaka Y, Okumura N, Yamaguchi K, Osada S, Yoshida K. Transvaginal resection of a rectal leiomyoma: A case report. Oncol Lett 2015; 10:3785-3788. [PMID: 26788208 DOI: 10.3892/ol.2015.3816] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/13/2014] [Accepted: 07/30/2015] [Indexed: 11/05/2022] Open
Abstract
The present study reports the case of a patient with a rectal submucosal tumor (leiomyoma) that was resected transvaginally. A 51-year-old female presented with a rectal submucosal tumor on the anterior wall of the lower rectum, located within 3 cm of the anal verge. This location would normally require intersphincteric or abdominal perineal resection. However, in order to minimize the invasiveness of the treatment and reduce post-operative morbidity, transvaginal resection and laparoscopic diverting ileostomy were performed instead. With the patient under general anesthesia, the posterior vaginal mucosa was incised vertically. The tumor was then excised en bloc with the overlying rectovaginal septum and rectal submucosal tumor. A primary repair of the defect and a diverting stoma were performed. The procedure did not present any complications, and the patient was discharged on day 10 post-surgery. The diverting stoma was closed 3 months later, and the sphincter function of the patient following surgery was monitored by manometry. The results of the manometric tests indicated that the patient did not suffer from fecal incontinence. In addition, the patient did not experience anal dysfunction or discomfort following the surgical procedure.
Collapse
Affiliation(s)
- Nobuhisa Matsuhashi
- Department of Surgical Oncology, Gifu University School of Medicine, Gifu 501-1194, Japan
| | - Takao Takahashi
- Department of Surgical Oncology, Gifu University School of Medicine, Gifu 501-1194, Japan
| | - Kengo Ichikawa
- Department of Surgical Oncology, Gifu University School of Medicine, Gifu 501-1194, Japan
| | - Toshiyuki Tanahashi
- Department of Surgical Oncology, Gifu University School of Medicine, Gifu 501-1194, Japan
| | - Yoshiyuki Sasaki
- Department of Surgical Oncology, Gifu University School of Medicine, Gifu 501-1194, Japan
| | - Yoshihiro Tanaka
- Department of Surgical Oncology, Gifu University School of Medicine, Gifu 501-1194, Japan
| | - Naoki Okumura
- Department of Surgical Oncology, Gifu University School of Medicine, Gifu 501-1194, Japan
| | - Kazuya Yamaguchi
- Department of Surgical Oncology, Gifu University School of Medicine, Gifu 501-1194, Japan
| | - Shinji Osada
- Department of Surgical Oncology, Gifu University School of Medicine, Gifu 501-1194, Japan
| | - Kazuhiro Yoshida
- Department of Surgical Oncology, Gifu University School of Medicine, Gifu 501-1194, Japan
| |
Collapse
|
|
10
|
García-Santos EP, Ruescas-García FJ, Estaire-Gómez M, Martín-Fernández J, González-López L. [Anorectal leiomyoma: a case report and literature review]. REVISTA DE GASTROENTEROLOGÍA DE MÉXICO 2014; 79:58-60. [PMID: 24629571 DOI: 10.1016/j.rgmx.2013.05.002] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/17/2013] [Revised: 05/01/2013] [Accepted: 05/29/2013] [Indexed: 10/25/2022]
Affiliation(s)
- E P García-Santos
- Servicio de Cirugía General y de Aparato Digestivo, Hospital General Universitario de Ciudad Real, Ciudad Real, España.
| | - F J Ruescas-García
- Servicio de Cirugía General y de Aparato Digestivo, Hospital General Universitario de Ciudad Real, Ciudad Real, España
| | - M Estaire-Gómez
- Servicio de Cirugía General y de Aparato Digestivo, Hospital General Universitario de Ciudad Real, Ciudad Real, España
| | - J Martín-Fernández
- Servicio de Cirugía General y de Aparato Digestivo, Hospital General Universitario de Ciudad Real, Ciudad Real, España
| | - L González-López
- Servicio de Anatomía Patológica, Hospital General Universitario de Ciudad Real, Ciudad Real, España
| |
Collapse
|
|
11
|
Grahn SW, Kwaan MR, Madoff RD. Malignant Conditions Including Squamous Cell Carcinoma and Rare Cancers. ANUS 2014:71-90. [DOI: 10.1007/978-1-84882-091-3_7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
|
|
12
|
García-Santos E, Ruescas-García F, Estaire-Gómez M, Martín-Fernández J, González-López L. Anorectal leiomyoma: A case report and literature review. REVISTA DE GASTROENTEROLOGÍA DE MÉXICO (ENGLISH EDITION) 2014. [DOI: 10.1016/j.rgmxen.2014.05.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
|
|
13
|
Rammohan A, Sathyanesan J, Rajendran K, Pitchaimuthu A, Perumal SK, Srinivasan UP, Ramasamy R, Palaniappan R, Govindan M. A gist of gastrointestinal stromal tumors: A review. World J Gastrointest Oncol 2013; 5:102-112. [PMID: 23847717 PMCID: PMC3708046 DOI: 10.4251/wjgo.v5.i6.102] [Citation(s) in RCA: 119] [Impact Index Per Article: 9.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/31/2013] [Revised: 04/30/2013] [Accepted: 06/10/2013] [Indexed: 02/05/2023] Open
Abstract
Gastrointestinal stromal tumors (GISTs) have been recognized as a biologically distinctive tumor type, different from smooth muscle and neural tumors of the gastrointestinal tract (GIT). They constitute the majority of gastrointestinal mesenchymal tumors of the GIT and are known to be refractory to conventional chemotherapy or radiation. They are defined and diagnosed by the expression of a proto-oncogene protein detected by immunohistochemistry which serves as a crucial diagnostic and therapeutic target. The identification of these mutations has resulted in a better understanding of their oncogenic mechanisms. The remarkable antitumor effects of the molecular inhibitor imatinib have necessitated accurate diagnosis of GIST and their distinction from other gastrointestinal mesenchymal tumors. Both traditional and minimally invasive surgery are used to remove these tumors with minimal morbidity and excellent perioperative outcomes. The revolutionary use of specific, molecularly-targeted therapies, such as imatinib mesylate, reduces the frequency of disease recurrence when used as an adjuvant following complete resection. Neoadjuvant treatment with these agents appears to stabilize disease in the majority of patients and may reduce the extent of surgical resection required for subsequent complete tumor removal. The important interplay between the molecular genetics of GIST and responses to targeted therapeutics serves as a model for the study of targeted therapies in other solid tumors. This review summarizes our current knowledge and recent advances regarding the histogenesis, pathology, molecular biology, the basis for the novel targeted cancer therapy and current evidence based management of these unique tumors.
Collapse
|
|
14
|
Mastoraki A, Psarras D, Mastoraki S, Vassiliu P, Danias N, Smyrniotis V, Arkadopoulos N. Rectum sarcoma: challenging diagnostic and therapeutic modalities. J Gastrointest Cancer 2013; 44:260-3. [PMID: 23712252 DOI: 10.1007/s12029-013-9506-6] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Abstract
INTRODUCTION Sarcomas are malignant tumors that arise from mesenchymal tissue at any of the body sites. They incorporate the wide category of GISTs and are classified in various histological types. Histological grading is another indicator of the degree of malignancy, the probability of distant metastases, and survival but remains a poor definition of local recurrence. DISCUSSION The size and depth of invasion are the most important prognostic factors. Since they grow within the intestinal wall, the symptoms are usually few or late, leading to delays in diagnosis. Most common signs are rectal bleeding, abdominal or anal pain, diarrhea, tenesmus and weight loss. The diagnostic and staging protocol of stromal tumors of the rectum includes mainly endoscopic surveillance, computed tomography, and magnetic resonance imaging. Therefore, rectum sarcoma (RS) consists one of the most biologically virulent cancers and is difficult to cure by conventional procedures. The treatment is primarily surgical, where possible, and should guarantee complete clearance of the tumor, which often requires an aggressive approach. Unfortunately, the minority of patients is eligible to undergo surgical intervention. In addition, surgical removal of RS does not necessarily indicate a patient's long-term recovery. Alternative therapies, such as radio- and chemotherapy, proved insufficient. Elucidation of its molecular basis may prove useful in developing and identifying prognostic biomarkers.
Collapse
Affiliation(s)
- Aikaterini Mastoraki
- 4th Department of Surgery, Medical School, Athens University, Attikon University Hospital, 1 Rimini str., 12462, Chaidari, Athens, Greece.
| | | | | | | | | | | | | |
Collapse
|
|
15
|
Ouh YT, Hong JH, Min KJ, So KA, Lee JK. Leiomyosarcoma of the rectum mimicking primary ovarian carcinoma: a case report. J Ovarian Res 2013; 6:27. [PMID: 23587096 PMCID: PMC3637498 DOI: 10.1186/1757-2215-6-27] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/12/2013] [Accepted: 04/11/2013] [Indexed: 12/17/2022] Open
Abstract
Leiomyosarcoma of the rectum is a very rare mesenchymal tumor. Because of its rarity, its diagnosis, treatment, and pathology often present challenges to the clinician. The characteristics of this tumor, such as its anatomical location, heterogeneous solid features on imaging, and nonspecific lower gastrointestinal tract symptoms, can be confused with those of primary ovarian carcinoma. Here, we report the case of a 52-year-old-woman presenting with a low abdominal mass that was later pathologically confirmed to be a rectal leiomyosarcoma. The findings of preoperative ultrasonography, pelvic magnetic resonance imaging, and abdominopelvic computed tomography were suggestive of a malignant pelvic mass, most likely a primary ovarian carcinoma. The patient underwent explorative laparotomy, and intraoperative frozen examination revealed a sarcoma originating from the gastrointestinal tract. Low anterior resection and supracervical hysterectomy with bilateral salpingo-oophorectomy were performed. The patient’s postoperative course was uneventful, and adjuvant chemotherapy is currently being administered.
Collapse
Affiliation(s)
- Yung-Taek Ouh
- Department of Obstetrics and Gynecology, Guro Hospital, College of Medicine, Korea University, Seoul 152-703, Republic of Korea.
| | | | | | | | | |
Collapse
|
|
16
|
Clinicopathological features and prognostic factors of rectal gastrointestinal stromal tumors. J Gastrointest Surg 2013; 17:793-8. [PMID: 23288714 DOI: 10.1007/s11605-012-2086-0] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/05/2012] [Accepted: 11/12/2012] [Indexed: 01/31/2023]
Abstract
AIM Rectal gastrointestinal stromal tumor (GIST) was a rare disease. This study sought to summarize clinicopathological features and prognostic factors of rectal GISTs. METHODS Clinicopathological characteristics and prognostic factors of rectal GISTs were investigated by reviewing patients undergoing curative resection at the Fudan University Shanghai Cancer Center between 1986 and 2010. RESULTS Twenty-one patients, 15 male and 6 female, were included. The mean age of onset was 51 years. The most common initial presentation was hematochezia (7 cases). Eleven patients underwent radical resection, and the other 10 received local resection. No lymph node metastases were identified. All cases were positive for CD117. Seventeen patients were classified as high National Institutes of Health (NIH) risk category. The 5-year disease-free survival (DFS) and overall survival were 33 and 46 %, respectively. Fifteen cases had recurrence postoperatively. Both univariate and multivariate analyses demonstrated the NIH risk category (p = 0.028) and hematochezia symptom (p = 0.014) were independent prognostic factors of the DFS of patients. CONCLUSIONS Hematochezia was the most common initial symptom. Over 50 % of patients received radical surgery. More than 80 % of patients were at high NIH risk of recurrence. Hematochezia symptom and high NIH risk category indicated poor prognosis of rectal GISTs.
Collapse
|
|
17
|
Nassif MO, Trabulsi NH, Bullard Dunn KM, Nahal A, Meguerditchian AN. Soft tissue tumors of the anorectum: rare, complex and misunderstood. J Gastrointest Oncol 2013; 4:82-94. [PMID: 23450454 DOI: 10.3978/j.issn.2078-6891.2012.042] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/13/2012] [Accepted: 08/27/2012] [Indexed: 12/28/2022] Open
Abstract
Anorectal soft tissue tumors are uncommon and often present both diagnostic and therapeutic challenges. Although many of these tumors are identified with imaging performed for unrelated reasons, most present with nonspecific symptoms that can lead to a delay in diagnosis. Historically, radical surgery (abdominoperineal resection) has been the mainstay of treatment for both benign and malignant anorectal soft tissue tumors. However, a lack of proven benefit in benign disease along with changes in technology has called this practice into question. In addition, the role of radiation and/or chemotherapy remains controversial. In this manuscript, we review the history and current status of anorectal soft tissue tumor management, with a particular focus on challenges in optimizing survival.
Collapse
Affiliation(s)
- Mohammed O Nassif
- Division of Experimental Surgery, McGill University, 845 Sherbrooke Street West Montreal, Quebec, Canada H3A 2T5; ; Department of Surgery, King Abdulaziz University, P.O. Box 80205, Zip Code 21589, Jeddah, Saudi Arabia
| | | | | | | | | |
Collapse
|
|
18
|
Selcukbiricik F, Yalçın S, Tural D, Erdamar S, Demir G, Doğusoy G, Mandel NM. Gastrointestinal stromal tumors in Turkey: experiences from 3 centers. ACTA ACUST UNITED AC 2013; 36:18-24. [PMID: 23429327 DOI: 10.1159/000346673] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022]
Abstract
BACKGROUND Gastrointestinal stromal tumors (GIST) are rare tumors of the gastrointestinal system. The most common primary site of GIST is the stomach. The treatment is primarily surgery, and the standard medical therapy is imatinib. Long-term survival can be obtained with good follow-up and treatment. MATERIALS AND METHODS In this study, data entry was performed using a web-based patient registry system for patients who were referred to 3 centers and retrospectively were diagnosed with GIST. RESULTS The study cohort consisted of 249 patients, including 160 men (64.3%) and 89 women (35.7%). The mean age was 59 years (range 21-90 years). Initially, 69.9% of the patients had local disease, while 30.1% had metastatic disease. The tumor was located in the stomach in 45.6% of patients. According to the Fletcher risk classification, the very low risk group included 8 subjects (3.2%), the low risk group included 40 subjects (16.1%), the moderate risk group included 56 subjects (22.5%), and the high risk group included 117 subjects (47%); the unspecified group included 28 subjects (11.5%). CONCLUSION These data are important for revealing the clinicopathologic characteristics and survival data of patients with GIST, who are treated and followed up in Turkey.
Collapse
Affiliation(s)
- Fatih Selcukbiricik
- Cerrahpasa Faculty of Medicine, Internal Medicine, Medical Oncology, Istanbul University, İstanbul, Turkey.
| | | | | | | | | | | | | |
Collapse
|
|
19
|
Benign anal and perianal polypoid neoplasms and tumor-like lesions. Pathol Res Pract 2012; 208:719-25. [DOI: 10.1016/j.prp.2012.09.004] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/18/2012] [Revised: 08/28/2012] [Accepted: 09/13/2012] [Indexed: 11/18/2022]
|
|
20
|
AbdullGaffar B, Abdulrahim M, Ghazi E. Benign fibrous histiocytoma presenting as anal canal polyp: first case report. Ann Diagn Pathol 2012; 17:464-5. [PMID: 22999485 DOI: 10.1016/j.anndiagpath.2012.07.008] [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: 07/01/2012] [Revised: 07/17/2012] [Accepted: 07/24/2012] [Indexed: 11/25/2022]
Abstract
Nonepithelial and nonmelanocytic anal neoplasms are uncommon. The majority are mesenchymal tumors, most of which are malignant sarcomas, particularly leiomyosarcomas and gastrointestinal stromal tumors. Benign mesenchymal anal neoplasms are even rarer. The most common reported cases were anal leiomyomas, granular cell tumors, fibroadenomas, and xanthogranulomas. Benign fibrous histiocytomas (BFHs) or dermatofibromas are common mesenchymal cutaneous tumors that occur in different sites and at any age. Review of the literature did not show previous reports of BFH arising in the anal canal region. We report the first case of a solitary BFH, an unexpected occurrence of a common tumor type presenting as a polyp in an unusual site such as the anus. This rare occurrence can present diagnostic challenges for the surgeons and pathologists. Clinically, it can be confused with the usual anal tags, fibroepithelial polyps, or hemorrhoids. Benign fibrous histiocytoma is a neoplasm with a potential of local recurrence and, therefore, carries certain clinical implications for the patients' management and follow-up when compared with the common nonneoplastic causes of anal polyps such as the anal tags of anal fissures, hemorrhoids, or fibroepithelial papilla. Histologically, it should be differentiated from other histiocytic lesions. This can be resolved by the application of certain histologic features with the appropriate immunohistochemical markers taken within the correct clinical context.
Collapse
|
|
21
|
Basu I, Lemonas P. Leiomyosarcoma of the rectum following pelvic irradiation: a difficult histological diagnosis. Ann R Coll Surg Engl 2012; 94:e44-5. [PMID: 22524929 DOI: 10.1308/003588412x13171221499469] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Abstract
Rectal leiomyosarcomas are very rare mesenchymal tumours. This is a case report of a rectal leiomyosarcoma diagnosed initially as a leiomyoma in a patient who had undergone pelvic radiotherapy several years previously. In addition to its pathological rarity, this case is of particular interest because it reinforces the association between pelvic irradiation and rectal leiomyosarcomas and it highlights the importance of treating suspicious cases aggressively in spite of favourable preoperative radiological and histological assessment.
Collapse
Affiliation(s)
- I Basu
- Princess Alexandra Hospital, Harlow, UK.
| | | |
Collapse
|
|
22
|
Transanal endoscopic microsurgery: long-term experience, indication expansion, and technical improvements. Surg Endosc 2011; 26:312-22. [PMID: 21898025 DOI: 10.1007/s00464-011-1869-9] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/04/2010] [Accepted: 08/01/2011] [Indexed: 12/12/2022]
Abstract
BACKGROUND This study aimed to review the authors' 16-year experience with transanal endoscopic microsurgery (TEM). Mortality, morbidity, recurrence rate, and functional outcome were assessed. New indications and technical improvements are presented. METHODS From November 1991 to August 2008, 123 patients (72 men and 51 women; median age, 68 years; range, 21-91 years) underwent TEM for excision of 105 adenomas with low- or high-grade dysplasia, 9 invasive adenocarcinomas (5 curative and 4 palliative resections), 2 neuroendocrine tumors, and 2 extramucosal lesions. Five additional patients had excisional biopsies, allowing staging after previous endoscopic resection. Most of the resections were full-thickness rectal resections using electrocautery or, more recently, the Harmonic scalpel. The latest mucosectomies were performed using the endoscopic submucosal dissection (ESD) technique. In addition, nontumoral indications included pelvic abscess (7 patients) and rectal strictures, which were either anastomotic or chemical. Pelvic abscesses were drained transrectally, whereas rectal stenoses were treated by strictureplasty. Foreign object retrieval and collagen plug placement for anal fistulas were performed using TEM in three patients. RESULTS No mortality occurred. One intraoperative rectal perforation required conversion to laparotomy. The postoperative complications included one pneumoperitoneum, which was treated medically, and one rectal perforation requiring Hartmann's procedure. In the polyp subgroup, six patients (6/91, 7%) experienced local recurrence. Pelvic abscesses were successfully treated, and stenosis did not recur after strictureplasty. Anorectal manometry showed functional alterations without significant clinical impact. CONCLUSIONS The findings showed TEM to be a safe and effective procedure for local excision of rectal lesions with a low recurrence rate and minimal consequences in terms of anorectal function. In addition, TEM proved to be feasible and effective for pelvic abscess drainage and rectal stenosis treatment. New technologies such as the Harmonic scalpel and ESD increase the precision already offered by this approach.
Collapse
|
|
23
|
Mehta G, Mittal A, Verma S, Inamdar P. Anorectal Leiomyoma : Case Report of Rare Tumor with unusual Presentation. Indian J Surg Oncol 2010; 1:337-40. [PMID: 22693386 DOI: 10.1007/s13193-010-0037-3] [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: 08/04/2010] [Accepted: 11/26/2010] [Indexed: 11/26/2022] Open
Abstract
Gastrointestinal stromal tumour (GIST) is a rare tumour of gastrointestinal tract, comprising of 0.1-3.0% of all gastrointestinal malignancies. They are commonly found in stomach (60-70%), very rarely in rectum and anal canal (<5%). The present case report is of a large anorectal leiomyoma (142×80mm) arising from posterior rectal wall, with its unusual presentation of urinary retention. In our case, in view of its massive size, recurrence and malignant transformation, we planned abdominoperineal resection. Although size and histological grade play an important role, complete resection still is considered the most significant favorable prognostic factor.
Collapse
Affiliation(s)
- Garima Mehta
- Department of surgery, R.N.T medical college, Udaipur, Rajasthan PIN -313001 India
| | | | | | | |
Collapse
|
|
24
|
|
|
25
|
Rare anorectal neoplasms: gastrointestinal stromal tumor, carcinoid, and lymphoma. Clin Colon Rectal Surg 2010; 22:107-14. [PMID: 20436835 DOI: 10.1055/s-0029-1223842] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Several uncommon tumors occur in the anal canal such as gastrointestinal stromal tumors, carcinoids, and lymphoma. Increased clinical experience and advancements in molecular biology have improved the accuracy of pathologic diagnosis and guided treatment recommendations, which the author addresses in this article.
Collapse
|
|
26
|
|
|
27
|
Kim HJ, Lee KY, Lee GH, Kim YW. Case report: Imaging features of perianal leiomyoma. Br J Radiol 2009; 82:e168-70. [PMID: 19592401 DOI: 10.1259/bjr/22020156] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022] Open
Abstract
A leiomyoma is a benign mesenchymal neoplasm that usually develops where smooth muscle is present. In this report, we present a rare case of perianal leiomyoma. The external anal sphincter was stretched over the surface of the leiomyoma and the internal anal sphincter was intact on surgery. It was confirmed as a deep soft-tissue leiomyoma on pathology. We describe the ultrasonographic and MRI findings of a rare case of perianal leiomyoma, together with differential diagnoses.
Collapse
Affiliation(s)
- H J Kim
- Department of Radiology, Kyung Hee University Medical Center, Dongdaemun-Gu, Seoul, Korea.
| | | | | | | |
Collapse
|
|
28
|
Padberg BC, Emmermann A, Zornig C, Germer M, Schröder S. [Leiomyomatosis of the colon: case report and literature review]. DER PATHOLOGE 2008; 28:161-5. [PMID: 17279406 DOI: 10.1007/s00292-007-0898-6] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
Abstract
We present the findings of a 67 year old male patient with an intestinal leiomyomatosis localized in the rectum. To our knowledge, this is the fifth case of intestinal leiomyomatosis reported so far. The most characteristic findings of this rare disease include a cuff-like tumorous proliferation of smooth muscle within the bowel wall which may extend into extramural tissue and result in a stenosis of a longer bowel segment. Because of severe obstructive symptoms over 5 years, the patient had to undergo surgery with resection of the rectum. The histological examination revealed a morphology and immunophenotype comparable to usual leiomyomas with the exception of hyalinosis-like changes in the blood vessels, apparently a special feature of leiomyomatosis. A novel finding in our case was the occurrence of skeinoid fibers which have so far only been reported in gastrointestinal stromal tumors.
Collapse
Affiliation(s)
- B-C Padberg
- Institut für Pathologie und Rechtsmedizin, Kantonsspital Chur, Loestrasse 170, 7000 Chur, Switzerland.
| | | | | | | | | |
Collapse
|
|
29
|
Thalheimer L, Richmond B, Lohan J. Leiomyosarcoma of the Anal Canal: Case Report and Review of the Literature. Am Surg 2008. [DOI: 10.1177/000313480807400118] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
A rare case of a leiomyosarcoma of the anal canal is presented. A 68-year-old male presented with painful defecation and rectal bleeding. Endorectal ultrasound revealed a mass invading both sphincters (T4 lesion), and extending 6 cm into the anal canal. Colonoscopy revealed an ulcerated area in the anal canal, of which biopsies revealed high-grade leiomyosarcoma–only the eighteenth such case at the time of this submission. The details of the case and teaching images are presented in detail, as is a comprehensive review of the relevant literature.
Collapse
Affiliation(s)
- Liza Thalheimer
- West Virginia University School of Medicine, Morgantown, West Virginia and
| | - Bryan Richmond
- Department of Surgery, West Virginia University, Charleston Division, Charleston, West Virginia
| | - Jay Lohan
- Department of Surgery, West Virginia University, Charleston Division, Charleston, West Virginia
| |
Collapse
|
|
30
|
Abstract
This review is part three of three and will present an update on the therapeutic options and procedures concerning gastrointestinal (GI) submucosal tumors (SMTs). The aim of this paper is to investigate the treatments of GI SMTs and to present a case of a gastrointestinal stromal tumor (GIST). Literature searches were performed to find information on therapy for GI SMTs. Based on these searches, the optimal therapeutic procedures could be outlined. The choice of treatment of localized tumors is endoscopic resection if possible or, alternatively, laparoscopic resection or surgical resection by an open procedure. However, benign SMTs should only be excised if symptoms are present, and GISTs should be treated with particular precautions. Irresectable or recurrent GISTs may be successfully treated with the tyrosine kinase inhibitor, imatinib.
Collapse
Affiliation(s)
- Laura-Graves Ponsaing
- Department of Surgical Gastroenterology K, Bispebjerg University Hospital of Copenhagen, Bispebjerg Bakke 23, 2400 NV Copenhagen, Denmark.
| | | |
Collapse
|
|
31
|
Ponsaing LG, Kiss K, Loft A, Jensen LI, Hansen MB. Diagnostic procedures for submucosal tumors in the gastrointestinal tract. World J Gastroenterol 2007; 13:3301-10. [PMID: 17659668 PMCID: PMC4172709 DOI: 10.3748/wjg.v13.i24.3301] [Citation(s) in RCA: 54] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
This review is part one of three, which will present an update on diagnostic procedures for gastrointestinal (GI) submucosal tumors (SMTs). Part two identifies the classification and part three the therapeutic methods regarding GI SMTs. Submucosal tumors are typically asymptomatic and therefore encountered incidentally. Advances in diagnostic tools for gastrointestinal submucosal tumors have emerged over the past decade. The aim of this paper is to provide the readers with guidelines for the use of diagnostic procedures, when a submucosal tumor is suspected. Literature searches were performed to find information on diagnostics for gastrointestinal submucosal tumors. Based on the searches, the optimal diagnostic procedures and specific features of the submucosal tumors could be outlined. Standard endoscppy, capsule endoscopy and push-and-pull enteroscopy (PPE) together with barium contrast X-ray do not alone provide sufficient information, when examining submucosal tumors. Endoscopic ultrasound (EUS), computed tomography (CT), magnetic resonance imaging (MRI) and fluorodeoxyglucose-labeled positron emission tomography (FDG-PET) are recommended as supplementary tools.
Collapse
Affiliation(s)
- Laura-Graves Ponsaing
- Department of Surgical Gastroenterology K, Bispebjerg University Hospital of Copenhagen, Bispebjerg Bakke 23, 2400 NV Copenhagen, Denmark.
| | | | | | | | | |
Collapse
|
|
32
|
Sánchez-Romero A, Pérez-Vicente F, Arroyo-Sebastián A, Calpena-Rico R. Leiomiosarcoma anal asociado a síndrome de Lynch. Med Clin (Barc) 2007; 128:518-9. [PMID: 17419920 DOI: 10.1016/s0025-7753(07)72642-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
|
|
33
|
Stewart SL, Wike JM, Kato I, Lewis DR, Michaud F. A population-based study of colorectal cancer histology in the United States, 1998-2001. Cancer 2006; 107:1128-41. [PMID: 16802325 DOI: 10.1002/cncr.22010] [Citation(s) in RCA: 82] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
BACKGROUND Histology is an important factor in the etiology, treatment, and prognosis of cancer. The purpose of this study was to descriptively characterize colorectal cancer (CRC) histology in the United States population. METHODS Data from cancer registries in the National Program of Cancer Registries (NPCR) or Surveillance, Epidemiology and End Results (SEER) program, representing 88% of the U.S. population, were used in the study. The analysis included 522,630 microscopically confirmed CRC cases diagnosed from 1998-2001. RESULTS About 96% of CRCs were adenocarcinomas, approximately 2% were other specified carcinomas (including carcinoid tumors), about 0.4% were epidermoid carcinomas, and about 0.08% were sarcomas. The proportion of epidermoid carcinomas, mucin-producing carcinomas, and carcinoid tumors was greater among females. Several histologic patterns with regard to race and ethnicity existed, including a higher percentage of carcinoid tumors among most non-white populations. With respect to age, higher percentages of sarcomas, mucin-producing adenocarcinomas, signet ring cell tumors, and carcinoid tumors were found in individuals under age 40. Overall, adenocarcinomas were more likely to be diagnosed at regional stages with moderate differentiation. Compared with other adenocarcinomas, signet ring cell tumors were more often poorly differentiated and were at distant stage at diagnosis. Carcinoid tumors and sarcomas were mainly poorly differentiated and were at localized stage at diagnosis. Small cell carcinomas were more likely undifferentiated and were at distant stage at diagnosis. CONCLUSIONS To date, this is the largest population-based study to analyze CRC histology in the United States. Distinct demographic and clinical patterns associated with different histologies may be helpful for future epidemiologic, laboratory, and clinical studies.
Collapse
Affiliation(s)
- Sherri L Stewart
- Division of Cancer Prevention and Control, Centers for Disease Control and Prevention, Atlanta, Georgia, USA.
| | | | | | | | | |
Collapse
|
|
34
|
Madden JF, Burchette JL, Raj GV, Daly JT, Tannenbaum M. Anterior rectal wall gastrointestinal stromal tumor presenting clinically as prostatic mass. Urol Oncol 2005; 23:268-72. [PMID: 16018942 DOI: 10.1016/j.urolonc.2005.02.001] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/28/2004] [Revised: 01/18/2005] [Accepted: 01/19/2005] [Indexed: 12/12/2022]
Abstract
Gastrointestinal stromal tumors (GIST) of the anterior rectal wall can present as a prostatic mass, with concomitant obstructive symptoms. Transrectal biopsies of GIST may be misdiagnosed as primary prostatic sarcomas. We report 3 cases of GIST that were initially characterized as prostatic leiomyosarcomas and treated definitively with pelvic exenteration. The correct diagnosis was possible only after immunohistochemical staining for CD117 and was made retrospectively in 2 of 3 cases. Additional therapy with imatinib (Gleevec, Novartis Pharmaceuticals Corporation, East Hanover, NJ), an inhibitor of CD117 tyrosine kinase activity, treated recurrence in one patient and effected complete remission.
Collapse
Affiliation(s)
- John F Madden
- Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA.
| | | | | | | | | |
Collapse
|
|
35
|
Affiliation(s)
- David T Efron
- The Johns Hopkins Medical Institution, Baltimore, Maryland, USA
| | | |
Collapse
|
|
36
|
Campos FG, Leite AF, Araújo SEA, Atuí FC, Seid V, Habr-Gama A, Kiss DR, Gama-Rodrigues J. Anorectal leiomyomas: report of two cases with different anatomical patterns and literature review. ACTA ACUST UNITED AC 2004; 59:296-301. [PMID: 15543403 DOI: 10.1590/s0041-87812004000500013] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
Gastrointestinal mesenchymal tumors comprise a rare group of gastrointestinal tract wall tumors that have long been a source of confusion and controversy, especially in terms of pathological classification, preoperative diagnosis, management strategies, and prognosis. This report describes the clinical manifestations and management of 2 rectal leiomyomas and reviews the pertinent literature. Case 1: A 44-year-old woman was admitted reporting a nodule in the right para-anal region for the previous 2 years. At proctological examination, a 4-cm diameter fibrous mass situated in the para-anal region that produced an arch under the smooth muscle on the right rectal wall just above the anorectal ring was noted. Computed tomography and magnetic resonance imaging of the abdomen and pelvis showed the lesion and detected no other abnormalities. Surgical treatment consisted of wide local resection of the tumor through a para-anal incision, with no attempts to perform lymphadenectomy. Case 2: A 40-year-old male patient was admitted reporting constant anal pain for 4 months. He presented a 3-cm submucosal nodule at the anterior rectal wall just above the dentate line. After 2 inconclusive preoperative biopsies, transanal resection of the tumor was performed. Histological analysis of the specimen showed a benign leiomyoma. A review of the literature is presented, emphasizing some clinical and therapeutic aspects of this unusual rectal tumor.
Collapse
Affiliation(s)
- Fábio Guilherme Campos
- Department of Gastroenterology, Coloproctology Unit, Hospital das Clínicas, Faculty of Medicine, University of São Paulo - São Paulo/SP, Brazil.
| | | | | | | | | | | | | | | |
Collapse
|
|
37
|
Abstract
Leiomyosarcoma is a rare, aggressively malignant connective tissue tumor of mature adults, which arises from smooth muscle. It occurs most frequently in the uterus, bowel, vascular tissues, and less commonly in somatic soft tissue or bone. The tumor when it arises in soft tissue has distinctive histologic features which somewhat resemble malignant fibrous histiocytoma (otherwise known as myxofibrosarcoma). The Orthopaedic Oncology Service at our institution has treated 66 patients with these lesions and thus far, 1/2 of the patients have died of disease at a mean of 3 years after discovery. Factors that increase the death rate include size of the tumor, Musculoskeletal Tumor Society Stage of disease, and to a lesser extent particularly in the lower extremities, anatomic site. Radiation and chemotherapy had little direct effect on the outcome but patients treated with surgery and adjunctive agents seemed to live longer than their cohorts treated with surgery alone. The purpose of this study is a general review of the clinical and prognostic features of this cancer.
Collapse
Affiliation(s)
- Henry J Mankin
- Department of Orthopaedic Oncology Service, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
| | | | | | | | | | | |
Collapse
|
|
38
|
Caporale A, Giuliani A, Cosenza UM, Cannaviello C, Benvenuto E, Angelico F. Leiomyosarcoma of the rectum after pelvic radiation therapy for endometrial carcinoma. Am J Gastroenterol 2002; 97:1270-1. [PMID: 12014748 DOI: 10.1111/j.1572-0241.2002.05724.x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
|
|
39
|
Miettinen M, Furlong M, Sarlomo-Rikala M, Burke A, Sobin LH, Lasota J. Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the rectum and anus: a clinicopathologic, immunohistochemical, and molecular genetic study of 144 cases. Am J Surg Pathol 2001; 25:1121-33. [PMID: 11688571 DOI: 10.1097/00000478-200109000-00002] [Citation(s) in RCA: 412] [Impact Index Per Article: 17.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
Gastrointestinal stromal tumors (GISTs), the specific KIT-positive mesenchymal tumors of the gastrointestinal tract, have been sporadically reported in the rectum, but there are few clinicopathologic series. In this study we analyzed the clinicopathologic features of 133 anorectal GISTs, 3 intramural leiomyomas (LMs), and 8 leiomyosarcomas (LMSs) from the files of the Armed Forces Institute of Pathology and the Haartman Institute of the University of Helsinki. Ninety-six GISTs were documented as KIT-positive and three additional ones as CD34-positive. Thirty-four tumors were included by their histologic similarity to KIT- or CD34-positive cases. GIST-specific c-kit gene mutations, mostly in exon 11, were documented in 18 of 29 cases (62%). The GISTs occurred in adults with the age range of 17-90 years (median 60 years) with a significant male predominance (71%). The tumors ranged from small asymptomatic intramural nodules to large masses that bulged into pelvis causing pain, rectal bleeding, or obstruction. They were mostly highly cellular spindle cell tumors; four tumors had an epithelioid morphology. The tumors coexpressed CD34 and KIT and were rarely positive for smooth muscle actin or desmin and never for S-100 protein. Seventy percent of patients with tumors >5 cm with more than 5 mitoses/50 high power fields (HPF) (n = 31) died of disease, whereas only one tumor <2 cm with <5 mitoses/50 HPF (n = 21) recurred and none caused death. Long latency was common between primary operation and recurrences and metastases; either one occurred in 60 of 111 patients with follow-up (54%). Distant metastases were in the liver, bones, and lungs. Three benign actin- and desmin-positive and KIT-negative intramural LMs, similar to those seen in the esophagus, were identified. There were eight LMSs, six of which formed a polypoid intraluminal mass and were actin-positive and KIT-negative. Despite high mitotic counts, only one LMS patient died of disease. A great majority of rectal smooth muscle and stromal tumors are GISTs, which have a spectrum from minimal indolent tumors to overt sarcomas. Intramural LMs are exceptional, and true LMSs are rare, and similar to colonic ones, often present as intraluminal polypoid masses that appear to have a better prognosis than GISTs with similar mitotic rates.
Collapse
Affiliation(s)
- M Miettinen
- Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
| | | | | | | | | | | |
Collapse
|