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
Gastrointestinal stromal tumor, signet ring cell, electron microscopy
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