Spindled signet ring cell-like gastrointestinal stromal tumor

Abstract

Amanda B. Moyer, Jae Y. Ro, Luan D. Truong, Diana Liang,Wade Rosenberg, Mojgan Amrikachi

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the GI tract. It is usually well-demarcated and comprised of sheets or fascicles of uniform spindled or epithelioid cells. Although focal cytoplasmic vacuolization has been described in epithelioid GISTs, generally, it is a minor component of the tumor. GIST is usually positive for c-kit, CD34, and DOG1, negative for desmin, and variably stains for smooth muscle actin (SMA), S-100 protein, and cytokeratin. Completely resected GISTs usually have a good prognosis. We report a rare case of a GIST composed exclusively of spindled signet ring cells (SRC), an entity previously reported only once in the stomach. The 74-year-old female patient presented with an incidental 6.8 cm gastric mass. The submucosal lesion, completely excised with a rim of gastric mucosal tissue, was round, tan-white, hemorrhagic, and focally cystic. Histologically, the tumor consisted of spindle cells with prominent SRC morphology, mitotic count <2 mitoses/50 high-power fields, and no pleomorphism or necrosis. The tumor was positive for c-kit and CD34, and negative for cytokeratin, S-100 protein, and SMA. The immunohistochemistry helped distinguish the GIST from other SRC neoplasms that can be found in the same location, such as adenocarcinomas, metastatic melanomas, and schwannomas. Extensive signet ring changes can occur in GIST but are rare. Signet ring changes can lead to misdiagnosis of GIST as a more aggressive tumor, particularly in biopsies with limited tissue sampling. Therefore, it is important to be aware of and recognize the SRC morphology in GIST.

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