Malignant Extra-Gastrointestinal Stromal Tumor of the Mesentery (original) (raw)
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Extra-Intestinal Gastrointestinal Stromal Tumor (Gist) of Mesentry
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Gastrointestinal stromal tumors are rare tumors, constituting less than 3% of all gastrointestinal malignant neoplasms but are the most common mesenchymal tumors of the gastrointestinal tract. Approximately 10% of gastrointestinal stromal tumors are extraintestinal and mostly arise from the mesentery or omentum. Here we report a rare case of an extraintestinal gastrointestinal stromal tumor of mesentery. Morphological and immunohistochemical features led to a diagnosis of extra-gastrointestinal stromal tumor.
Anticancer Research, 2018
Background/Aim: The objective of the present study was to determine the clinicopathological factors and treatment outcomes of patients suffering from mesenteric or retroperitoneal extragastrointestinal stromal tumors (EGISTs). Materials and Methods: A detailed search in PubMed, using the key words "extragastrointestinal stromal tumors" and "EGIST", found eight studies fulfilling the criteria of this study. Results: Thirty-six patients with a mesenteric and 24 patients with a retroperitoneal EGIST were analyzed, with a follow-up period ranging from 2 to 192 months. Retroperitoneal tumors presented as larger tumors than mesenteric ones, with 95% and 93% immunohistochemical positivity for CD117 antigen, respectively. Surgical resection was performed in 91% of cases, with 57% of patients with mesenteric and 70% of patients with retroperitoneal EGISTs being alive at the last follow-up. Conclusion: EGISTs most commonly are of considerable size and usually with a high mitotic count, rendering them high-risk tumors. Tumor necrosis, nuclear atypia, tumor histology, and mutations in the tyrosine kinase KIT or platelet-derived growth factor receptor A (PDGFRA) gene, seem to influence tumor behavior. Gastrointestinal stromal tumors (GISTs) are the most frequent mesenchymal tumors of the gastrointestinal (GI) tract (1). They may arise from any part of the digestive tract, with the stomach and the small intestine being the most common sites (2). GISTs are thought to originate from, or to show differentiation toward, the interstitial cells of Cajal, which are the pacemaker cells present in the GI tract. Their diagnosis is based on histomorphological, as well as immunohistochemical features, and in the majority of cases, on histochemical immunopositivity for the tyrosine kinase receptor of KIT (CD117) (3-5). Recent studies have demonstrated that mesenchymal tumors with clinicopathological and molecular profiles resembling GISTs (6) may arise outside the GI tract, especially in soft tissues of the abdomen and the retroperitoneum (1, 7). These tumors are called extragastrointestinal stromal tumors (EGISTs) and account for <10% of all stromal tumors. Most EGISTs arise from the mesentery, the retroperitoneum, and the omentum as well as other abdominal sites. Although most EGISTs are described as malignant tumors, due to their rarity little is known about their incidence, pathogenesis, genetic profile and prognosis. Moreover, even nowadays, their malignant potential is determined by the same parameters used for their GI counterparts, but it still remains unclear whether this is a rational approach. In the present study, we aimed to review the clinicopathological, immunohistochemical and genetic alterations that are present in EGISTs of the mesentery and retroperitoneum, that are two of the most common sites of EGIST localization.
A gastrointestinal stromal tumor with mesenteric and retroperitoneal invasion
World Journal of Surgical Oncology, 2007
Background: Gastrointestinal stromal tumors are rare visceral sarcomas arising in the gastrointestinal tract wall. In this report we present a case of gastrointestinal stromal tumors with mesenteric and retroperitoneal invasion, describe and discuss its computed tomography findings. Case presentation: A 57-years-old male patient has been complaining of abdominal distention, weight lose, and hematuria. During physical examination, significant distention and multiple palpable tumor masses were identified on the abdomen. Abdominal computed tomography showed multiple, well-defined, soft tissue masses with homogenous and heterogeneous pattern, in the mesenteric and retroperitoneal areas. Unlike specific features of gastrointestinal stromal tumor, renal obstruction and atypical central calcification without chemotherapy that has not been yet described were seen in this case. Computed tomography did not reveal liver metastases and/or the lymph nodes with pathological size. Ultrasonography-guided true-cut ® biopsy was made, histopathologic and immunohistochemical analyses demonstrated stromal tumor which, C-KIT (+). The patient underwent left ureterectomy, left nephrectomy and total colectomy. Postoperative histopathological analyses revealed lower grade malignant GISTs. As of 17 months after the surgery, he is alive and free of recurrence. Conclusion: When intraabdominal, multiple, large (>5 cm), well-circumscribed, homogenous or heterogeneous mass lesions without ascites, omental caking and lymph nodes metasteses were seen, gastrointestinal stromal tumors should be considered in the differential diagnosis.
Gastrointestinal stromal tumors: a clinicopathological and immunohistochemical study of 121 cases
Indian Journal of Gastroenterology, 2010
Background Primary mesenchymal tumors of the gastrointestinal tract are a heterogeneous group of tumors with a wide clinical spectrum, of which gastrointestinal stromal tumors (GIST) typically occur in middle-aged to older individuals. This study evaluated the clinicopathological and morphological features of GIST of the intestinal tract. Methods The study included 108 gastrointestinal and 13 extra-gastrointestinal stromal tumors involving the mesentry and retroperitoneum between January 1989 and July 2007. Immunohistochemical expression of CD117, CD34, SMA, Desmin, S100, and Ki-67 were studied. Results GIST comprised 108 of 120 (90%) of the mesenchymal tumors. The tumor was located in the stomach in the majority (55%) of patients followed by small intestine (30%), retroperitoneum (7%) and the colorectum (4%). There was a significant correlation between tumor size and mitotic index, with larger tumors having higher mitotic index (p<0.001). Mitotic index per 5 mm 2 correlated with high cellularity (p<0.001), presence of necrosis (p<0.001) and presence of mucosal invasion (p=0.01). Expression of CD117 was seen in 94%, CD34 in 59%, SMA in 41%, S-100 in 33%, and desmin in 4% of tumors. Conclusion We found GIST to be the most common mesenchymal tumor of the gastrointestinal tract.
Indian Journal of Cancer, 2013
BACKGROUND: Extra gastrointestinal stromal tumors (EGIST) are uncommon compared to their gastrointestinal counterparts. EGISTs involve omentum, mesentery, retroperitoneum, pancreas, and pelvis. MATERIALS AND METHODS: Ten EGISTs were analyzed in this study from January 1995 to November 2011. They were analyzed with respect to clinical features, imageological, histopathological, and immunohistochemical findings. The immunohistochemical stains used were Smooth muscle actin (SMA), Desmin, S-100 protein, CD34 and CD-117. RESULTS: There was slight female preponderance with wide age range. Four of the tumors were in retroperitoneum, three in mesentery, and two in omentum and one in pelvis. Histopathologically majority were spindle cell tumors. Immunohistochemically CD117 was consistently positive followed by CD34. Smooth muscle actin was positive in eight cases, S-100 protein and desmin were positive in two cases each. CONCLUSION: EGISTs are rare and should be considered in the differential diagnosis of the mesenchymal tumors and immunohistochemistry helps to confirm the diagnosis. Further study with better follow-up is desired to characterize these uncommon tumors.
Non-GIST Mesenchymal Tumors of the Gastrointestinal Tract
2020
Aim: Mesenchymal tumors of the gastrointestinal tract other than gastrointestinal stromal tumors are rare and most of them are benign. The aim of this study to reveal the clinical and pathological features of non-gastrointestinal stromal tumor mesenchymal tumors of the gastrointestinal tract. Materials and Methods: All gastrointestinal endoscopic and surgical resection materials, between 2008-2018, were retrospectively reviewed. Cases diagnosed with mesenchymal tumor other than gastrointestinal stromal tumors were included. Results: Twenty four lipomas, 14 inflammatory fibroid polyps, six leiomyomas, four lymphangiomas, four hemangiomas, four schwannomas, two neuromas, two malignant melanomas, one leiomyosarcoma, one granular cell tumor and one Kaposi sarcoma were detected. The median ages of the cases were 61 years (29-87), the median tumor size was 1.5 cm (0.2-14). Thirty seven (58.7%) cases were female and 26 (41.3%) were male. They caused nonspecific symptoms. No recurrence or m...
Multiple and Recurrent Extra intestinal gastrointestinal stromal tumor (EGIST) in omentum and peritoneum -a case report Introduction: Gastrointestinal tumors are the intraabdominal tumors arising from muscular propia of gastrointestinal tract. Similar morphology, histopathological tumors rarely may arise from peritoneum, mesentry, or omentum and these tumors are called extra intestinal gastrointestinal stromal tumors. Case Presentation: 45-year male presented with lump over left flank and right hypochondrium region without bowel, bladder and biliary symptoms. Abdominal sonogram and computerized tomography of abdomen suggested two large tumors located separately at right sub-diaphragmatic and left lumbar regions. The preoperative needle aspiration cytology was inconclusive. DISCUSSION: Diagnostic laparoscopy showed two tumors situated at different locations. Tumor at the right sub-diaphragmatic region was arising from peritoneum and another large tumor at the lumbar region from omentum. Excision biopsy and further immunohistochemistry revealed extra intestinal GIST Conclusion: Extra intestinal gastrointestinal stromal tumors arising from peritoneum and omentum in the same patient is rare occurrence.