Background Extragastrointestinal stromal tumors (EGISTs) are extremely rare mesenchymal tumors histologically and immunophenotypically similar to GI stromal tumors (GISTs). The aim of this study was to analyze the clinicopathological factors and treatment outcome in 13 patients with EGISTs treated at a tertiary care center. Methods Of 109 patients with GISTs treated at our center between April 2002 and December 2012, 13 patients with EGISTs were analyzed for clinicopathological factors and treatment outcome. Results Mean age was 45.8 (range 30-61) years, and females constituted 62% with a male:female ratio of 0.6:1. The most common tumor sites were mesentery in 10 patients and retroperitoneum in 3 patients. Mean tumor size was 11.7 (range 5-18) cm. Four (31%) patients were metastatic at presentation, the most common site of metastases being the liver in 3 (75%) patients. Lymph node enlargement was seen in 2 patients. Surgery was performed in 8 (62%) patients, 7 with localized disease, and 1 with metastatic disease. R0 resection was achieved in 3 (38%) patients. Five (71%) patients were considered as high-risk. Recurrences were seen in 3 patients (patient 3, 5 and 13) with localized disease after surgical resection, at 18, 7 and 137 months, respectively. At the last follow up, 7 patients were alive and 6 died of disease progression. The median overall survival was 34 (7-148) months. Conclusions EGISTs present at a younger age in the developing than in the developed countries. Females are more commonly affected than males. Lymph node metastases may be commonly present. Keywords Extrgastrointestinal stromal tumors, mesentery, retroperitoneum Ann Gastroenterol 2015; 28 (1): 105-108

Clinicopathological and treatment analysis of 13 extragastrointestinal stromal tumors of mesentery and retroperitoneum.

Objective This study was performed to discuss the characteristics, diagnosis, and treatment of primary prostatic extragastrointestinal stromal tumor (EGIST). Methods The case history data of a patient with an EGIST were analyzed and discussed with a literature review. Results The patient was diagnosed with a pelvic tumor, possibly malignant. We ascertained the diagnosis by exploratory surgery and pathological biopsy. The tumor was present in the prostate and infiltrated and pressed against the anterior rectal wall. Pathological biopsy showed that the tumor comprised spindle cells, which were also present at the junction of the tumor and prostate tissue. Immunohistochemically, the tumor cells were positive for CD117, DOG-1, CD34, and smooth muscle actin and negative for S100 and desmin; Ki-67LI was about 10%. These results support the diagnosis of primary prostatic EGIST. Conclusion The rarity and nonspecific clinical manifestation of prostatic EGIST facilitate misdiagnosis. Diagnosis mainly depends on imaging examination and characteristic histopathological and immunohistochemical features, and GIST must be excluded. Surgery is the main treatment method, and imatinib is suggested for unresectable and malignant EGISTs.

https://journals.sagepub.com/doi/pdf/10.1177/0300060518791677

Primary prostatic extragastrointestinal stromal tumor: a case report and literature review:

Gastrointestinal stromal tumors (GISTs), the commonest mesenchymal tumors of gastrointestinal tract are often described to take origin from the interstitial cells of Cajal (ICC) or its precursor cells. Rarely these tumors do arise in structures other than the alimentary tract like omentum, mesentery, retroperitoneum, etc., of varying malignant potential and are known as extra-gastrointestinal stromal tumors (eGISTs). This is a case report of a 70-year-old female with multicentric malignant eGISTs arising in the mesentery of ileum. On laparotomy, a large mass of 20 × 15 cm was found in the small bowel mesentery without involvement of the adjacent ileum, with multiple other small nodules resembling lymph nodes, present adjacent to it. Histopathological study of the excised lump, confirmed the mass to be malignant eGIST without involvement of the adjacent ileum, with cluster differentiation (CD)117 positive and of high-risk stratification. The mesenteric nodule was confirmed on histopathology to be malignant eGIST, similar to that of that of the primary, without any lymphoid tissue. Adjuvant imatinib mesylate treatment was started immediately postoperation with the patient doing well at 1 year of follow-up. We report this case, due to the rare occurrence of multifocal malignant eGISTS of small bowel mesentery, which is yet to be reported. The existing literature is unclear regarding the clinicopathology and management of multifocal malignant stromal tumors of the mesentery.

/pdf/malignant-extra-gastrointestinal-stromal-tumor-of-the-35iip59se5.pdf

Malignant Extra-Gastrointestinal Stromal Tumor of the Mesentery

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors located in the alimentary tract. A small portion of GISTs are observed in extra-gastrointestinal regions, primarily in the omentum, mesentery and retroperioneum, and these types of GISTs are referred to as extra-gastrointestinal stromal tumors. The present study reports of a patient with unique primary liver GIST. The patient underwent en bloc resection and post-operative administration of imatinib, and subsequently experienced a good prognosis. The present case is followed by a brief review of reported cases of liver GISTs identified in the literature. The literature revealed that primary liver GISTs are usually large in size and possess a high mitotic index, which contributes to malignant characterization, thus classifying these tumors as high-risk. En bloc resection remains the mainstay of treatment for resectable primary liver GISTs. However, the prognosis of these patients is not favorable. Perioperative administration of imatinib may be useful to a certain extent, and interventional therapy, including radiofrequency ablation, should be considered.

/pdf/primary-gastrointestinal-stromal-tumor-of-the-liver-a-case-3qyn0is3mr.pdf

Primary gastrointestinal stromal tumor of the liver: A case report and review of the literature.

Extra-gastrointestinal stromal tumors (EGISTs) predominantly occur outside of the gastrointestinal tract, and their biological and histological characteristics are similar to those of GISTs. Primary EGIST occurrence in the liver is extremely rare. The present study reports a case of primary EGIST in the caudate lobe of the liver in a 61-year-old Chinese man. Contrast-enhanced computed tomography revealed a 7.3×5.1-cm heterogeneously enhanced neoplasm with solid and cystic components located in the caudate lobe of the liver. The patient underwent caudate lobe (specifically, Spiegel lobe) resection. Immunohistochemical analysis of the resected tumor revealed a strong positivity for cluster of differentiation (CD)117, discovered on GIST-1 and CD34. Thus, based on the histological and immunohistochemical findings, the final diagnosis was primary hepatic EGIST. Follow up was conducted at 3-month intervals for the first year and 6-months thereafter. The patient was asymptomatic without any sign of recurrence during the follow-up period. Lab tests were in normal range, and no mass was found in CT scan.

/pdf/malignant-extra-gastrointestinal-stromal-tumor-of-the-liver-kq7dezynxs.pdf

Malignant extra-gastrointestinal stromal tumor of the liver: A case report

The gastrointestinal stromal tumor is the most common mesenchymal neoplasm of the gastrointestinal tract. The gastrointestinal stromal tumor universally expresses KIT and DOG-1 and frequently harbors oncogenic mutations in the KIT gene. While the gastrointestinal stromal tumor usually arises in the alimentary tract, it is rarely found in the extragastrointestinal area. When it is, it is called an extragastrointestinal stromal tumor. Although the pathogenesis, prognostic factors and outcomes of gastrointestinal stromal tumors are well known, those of extragastrointestinal stromal tumors have not been fully studied. We report, herein, a unique primary extragastrointestinal stromal tumor from the pleura in a 73-year-old woman who presented with pleural mass. The extragastrointestinal stromal tumor was surgically resected and confirmed by means of an immunohistochemical study and a molecular analysis.

/pdf/the-primary-extra-gastrointestinal-stromal-tumor-of-pleura-a-2gtkpekjie.pdf

The Primary Extra-gastrointestinal Stromal Tumor of Pleura: A Case Report and a Literature Review

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The Primary Extra-gastrointestinal Stromal Tumor of Pleura: A Case Report and a Literature Review