Extragastrointestinal Stromal Tumor Localized in the Retroperitoneum (original) (raw)
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Malignant Retroperitoneal Extra-Gastrointestinal Stromal Tumor: A Unique Entity
World Journal of Oncology, 2016
Extra-gastrointestinal stromal tumors (EGISTs) are a recently described group of tumors. A handful of less than 70 cases have been reported in English literature, so far, to the best of our knowledge. Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the alimentary canal. EGISTs are a unique entity, which require distinction from GISTs because, even though, they exhibit similar histology and immunohistochemistry to GISTs, they occur outside the gastrointestinal tract, i.e. in omentum, mesentery, retroperitoneum, etc. and have different behavior patterns as far as their prognosis and management are concerned. Retroperitoneal subgroup of EGISTs is extremely rare and we report such a case of primary malignant EGIST of the retroperitoneum which presented as a soft tissue mass on radiological evaluation. The tumor turned out to be a histopathological surprise, and could be distinctively labeled as EGIST only after morphological and immunohistochemical studies. It is imperative for radiologists, pathologists and oncologists, among other clinicians, to be able to recognize and understand the presentation of this group of tumors due to their rapid progression and poor prognosis, so that an early diagnosis and management may be able to improve the final disease outcome.
Malignant extragastrointestinal stromal tumor of retroperitoneum
Acta Oncologica, 2005
Context Gastrointestinal stromal tumors are CD117 (C-Kit) positive mesenchymal neoplasms considered to originate from the interstitial cells of Cajal. Gastrointestinal stromal tumors have been described outside the gastrointestinal tract in sites, such as the mesentery, omentum and retroperitoneum; however, pancreatic extra-gastrointestinal stromal tumors are extremely rare and there have only been seven previous reports in the literature. Case report We describe a 38-year-old man with a malignant pancreatic gastrointestinal stromal tumor. The tumor was located in the head of pancreas, measured 6.5x5.0 cm and was well circumscribed. On histology, it showed a mixed spindle and epithelioid cell morphology with the presence of sheets and short intersecting fascicles of tumor cells. The mitotic count was 12-15 mitoses per 50 high-power fields. The differential diagnosis included a pancreatic smooth muscle tumor and a neuroendocrine tumor. Immunohistochemistry revealed diffuse cytoplasmic positivity for CD117 and vimentin. Tumor cells were negative for CD34, S100, desmin, smooth muscle actin (SMA), cytokeratin, neuron specific enolase, chromogranin and synaptophysin. The patient developed isolated liver metastasis two years after the resection of the primary tumor. The resected metastasis showed a similar tumor. The patient was treated with imatinib mesylate and the post-operative course two years after resection of the liver metastasis has been uneventful. Conclusion We report a rare case of pancreatic gastrointestinal stromal tumor presenting as a solid neoplasm and review the cases previously described in the literature.
Retroperitoneal extra gastrointestinal stromal tumor: A case report
2023
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors. Researchers do not know precisely what leads to GISTs, but genetic mutations play an important role. These mutations have no apparent cause. GISTs are usually asymptomatic tumors, although GI bleeding and weight loss can occur. CT is preferred for investigating potential GISTs. Case presentation: A 36-year-old unmarried Syrian female came to the hospital complaining of recurrent abdominal pain. CT revealed a large mass occupying a significant portion of the left hypochondrium and the lower part of the epigastrium. The tumor exceeded the median line to the right, pressing on the mesenteric vessels and the intestinal loops below. Immunohistochemistry results showed moderate positivity to CD117 and CD34, which were compatible with the diagnosis of GIST. The entire mass was excised. Physicians performed CT follow-ups every three months for 18 months, and no evidence of recurrence was observed. Discussion: Extragastrointestinal GISTs are a rare subtype of GISTs that occur outside the GI tract. GISTs previously used to be misdiagnosed as leiomyoma, leiomyosarcoma, leiomyoblastoma, and schwannoma. Treatment depends on surgery with adjuvant therapy tyrosine kinase inhibitors. Follow-up is recommended as the risk of recurrence is high. Conclusion: We recommend that GIST, as an extremely rare tumor, should be considered in the differential diagnoses of masses that occur in the extra-intestinal region. Usually, patients need surgery with lymph node resection. However, this was not needed in our case.
Retroperitoneal gastrointestinal stromal tumor: A case report and literature review
Radiology Case Reports, 2020
Retroperitoneal gastrointestinal tumor is the rarest subtype among 3 subtypes of extragastrointestinal tumors, which are uncommon stromal tumors. We herein report a case of a 55year-old man with retroperitoneal gastrointestinal tumor detected by magnetic resonance imaging and confirmed by postoperative histology and immunohistochemistry.
An unusual presentation of a retroperitoneal gastrointestinal stromal tumors
International Surgery Journal, 2021
We report a case of 52 years old female patient who presented in our surgery clinic with pain, and a gradually increasing lump in the lower abdomen associated with menorrhagia. Physical examination revealed a huge pelvic mass measuring 16x20 cm occupying almost whole of the lower abdomen and extending to right hypochondrium. The lump was bimanually palpable along with fibroid uterus and broad ligament fibroid. Ultrasound radiography revealed a huge mass of mixed echogenicity in the pelvis and another large multilobulated mass arising from right iliac fossa to right lumbar region reaching up to right hypochondrium. ABSTRACT
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.