Atypical Presentation of Gastrointestinal Stromal Tumor as Multiple Intra-Abdominal Cysts: A Case Report

Abstract

Gastrointestinal stromal tumors (GISTs) are the most common nonepithelial solid neoplasms involving the alimentary tract. We report a case of cystic GIST with multiple cystic metastases. A 61-year-old man presented with upper abdominal pain for two months. Further evaluation revealed a large intra-abdominal cyst in the lesser sac and another cyst over the segment VII of the liver on imaging. Multiple intra-abdominal hydatidoses were suspected based on the imaging and its endemic nature in the geographical area. However, the hydatid serology was normal. In view of hemorrhagic cyst fluid, an intraoperative frozen biopsy of the cyst wall was done, which revealed features suspicious of a mesenchymal tumor. Sleeve gastrectomy with enbloc excision of the gastric cyst, excision of the hepatic cyst, and complete excision of multiple other intra-abdominal cysts were performed considering GIST as a possibility. Histology revealed a clear cell variant of GIST. Gastric GISTs primarily presenting as multiple intraabdominal cysts and of clear cell histological variants had never been reported in the literature. The patient was started on imatinib, and he has shown no evidence of recurrence after 12 months of follow-up. A high index of suspicion, intraoperative frozen section, meticulous surgery, and immunohistochemistry are all crucial for the effective management of atypical cases. GIST may be considered as a part of differential diagnosis in clinical scenarios with multiple intraabdominal cysts, especially in the equivocal setting.

Multiple, large intra-abdominal cystic lesions and iron deficiency anaemia as the presenting symptoms of SDHD gastrointestinal stromal tumour (GIST) in a young sub-Saharan woman

“…In our patient, the considerable number of unusually large cysts was our initial unique diagnostic challenge, and we have identified only one prior similar case that was very recently reported in the literature 17. Having said that, GISTS with one or a few cysts, and sometimes quite large, are more common, although still rare.…”