Extragastrointestinal Stromal Tumor Localized in the Retroperitoneum (original) (raw)
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 Rare Case of a Giant Retroperitoneal Gastrointestinal Stromal Tumor of Malignant Potential
International Journal of Innovative Research in Medical Science
Gastrointestinal stromal tumors (GIST) are mesenchymal tumors that originate in the gastrointestinal tract. We present the case of a 30-year-old man who presented with progressive abdominal distension of one-years’ duration. A physical examination revealed a distended, tense, non-tender abdomen, and initial imaging investigations revealed a huge cystic, septate abdominal mass. A provisional diagnosis was made of a huge retroperitoneal, malignant, cystic mass. However, aspiration cytology showed signs suggestive of a benign cyst. Cystic tumor excision performed with control of drain site to prevent leakeage throughout the procedure. The tumor extending into the pelvis, measuring approximately 17 x 31 x 42 cm in diameter and weighing 3.78 kg. The tumor was resected, and a histopathological examination showed findings consistent with a GIST of mixed spindle and epithelioid type. Further evaluations revealed that the tumor was positive for CD 117 and CD 34, prompting the oncology team t...
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.
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.
Modern Pathology, 2000
The clinicopathologic features of 48 tumors that were histologically similar to gastrointestinal stromal tumors but occurred in the soft tissues of the abdomen were analyzed to determine their overall similarity to their gastrointestinal counterpart, their biologic behavior, and the parameters that predict risk for adverse outcome. Classic leiomyomas and leiomyosarcomas were specifically excluded. The tumors occurred in 32 women and 16 men, who ranged in age from 31 to 82 years (mean, 58 years). Forty tumors arose from the soft tissue of the abdominal cavity, and the remainder arose from the retroperitoneum. They ranged in size from 2.1 to 32.0 cm and varied from tumors composed purely of rounded epithelioid cells to those composed of short fusiform cells set in a fine fibrillary collagenous background with some cases showing a mixed pattern. Tumors displayed variable amounts of stromal hyalinization, myxoid change, and cyst formation. The tumors expressed CD117 (c-kit receptor) (100%), CD34 (50%), neuron-specific enolase (44%), smooth muscle actin (26%), desmin (4%), and S-100 protein (4%). Tumors were evaluated with respect to several parameters: size (<10 cm or >10 cm), cellularity (low or high), mitoses (0 to 2 per 50 high-power fields, >2 per 50 high-power fields), nuclear atypia (1 to 3؉), cell type (epithelioid, spindled, or mixed), and necrosis (absent or present). These parameters were then evaluated in univariate and multivariate analysis with respect to adverse or nonadverse outcome, the former defined as metastasis or death from tumor. Follow-up information was obtained for 31 patients (range, 4 to 84 months; median, 24 months). One patient presented with an adverse event and, therefore, was excluded from subsequent analysis. Twelve patients (39%) developed metastases or died of tumor. In univariate analyses, cellularity, mitotic activity (>2 per 50 high-power fields), and necrosis were associated with statistically significant increases in the risk for adverse outcome. Despite the relatively small sample size, in a multivariable analysis mitotic activity (relative risk, 7.46; P ؍ .09) and necrosis (relative risk, 3.75; P ؍ .07) displayed trends toward independent predictive value. No association was noted between histologic pattern and outcome. Although only 39% of tumors behaved in a malignant fashion, this figure probably represents a conservative estimate because long-term follow-up (>5 years) was available for only a limited number of patients. Stratification of patients who have extragastrointestinal stromal tumor into those with 0 to 1 adverse histologic factors versus those with 2 to 3 offers the advantage of separating patients into two groups that have a markedly different risk for adverse outcome in the short term (0.02 events versus 0.54 events per person-year; P < .001, respectively). Extragastrointestinal (soft tissue) stromal tumors are histologically and immunophenotypically similar to their gastrointestinal counterpart but have an aggressive course more akin to small intestinal than gastric stromal tumors. KEY WORDS: Gastrointestinal stromal tumor, Leiomyosarcoma, Sarcoma. Mod Pathol 2000;13(5):577-585
Impact of retroperitoneal tumors on the digestive tract (Review)
Experimental and Therapeutic Medicine
Primary retroperitoneal tumors are a rare group of neoplasms that often prove to be a real therapeutic challenge. The clinical picture is in most cases nonspecific, being the result of nearby tissue and organ compression, displacement and/or invasion, and it usually includes a variety of deceiving signs and symptoms that may lead to diagnostic errors. During the course of the disease, up to half of the patients diagnosed with retroperitoneal tumors can develop gastrointestinal symptoms, the incidence of such symptoms significantly increasing with tumor size. Therefore, symptoms such as abdominal distension, diffuse abdominal pain or epigastric pain mimicking acute pancreatitis, diarrhea, constipation, jaundice, hematemesis, melena and hematochezia can be found. The mainstream treatment for primary retroperitoneal tumors is surgical excision, chemotherapy and radiotherapy but associated with poor results. Due to their rarity, as well as to the complexity of these tumors, patients should be managed in high volume centers that allow the possibility of a multidisciplinary approach.
ARS Medica Tomitana, 2015
Gastrointestinal stromal tumors are an uncommon sarcomas with mesenchymal origin that arises in the gastrointestinal tract. Recently, most of the published reports describe such tumors located outside of the gastrointestinal tract, with similar pathological characteristics and are named extra-gastrointestinal stromal tumors. We report the case of a patient suffering for a giant retroperitoneal extra-gastrointestinal stromal tumor, complicated with intra-tumoral hemorrhage. The particularity of this case is the presence of the intra-tumoral hemorrhage in a retroperitoneal extragastrointestinal stromal tumor that grew rapidly in volume (in 1 week).
Case Report on Rare Outcome of a Retroperitoneal Mass: Management Challenges and Lessons Learnt
International Journal of Medicine and Medical Research
Background. Gastrointestinal stromal tumours (GIST) are non-epithelial mesenchymal solid neoplasm with varied presentation. The study reports the case of a retroperitoneal GIST in a 21‑year‑old male presented with an abdominal lump for six months. The lesion was initially thought to be a retroperitoneal sarcoma. Exploratory laparotomy revealed an abdominopelvic mass covering the entire right side of abdomen and pelvis. The tumour was adherent to the terminal ileum and ascending colon. There were dense adhesions between the retroperitoneum with involvement of the middle third of the right ureter. The tumour was resected with right hemicolectomy with ileotransverse anastomosis. Post-operative histopathology revealed it as high-grade spindle cell type GIST. The patient is presently on post-operative chemotherapy with Imatinib mesylate. Objective. Atypical presentations of GIST are seldom discussed but frequently encountered in clinical practice. This article depicts different challenge...