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Giant gastric myxoid epithelioid gastrointestinal stromal tumor

Published:November 12, 2022DOI:https://doi.org/10.1016/j.dld.2022.10.020
      A 45-year-old woman presented to the gastrointestinal surgical department with a 2-month history of enlarging abdominal mass. Physical examination revealed an abdominal tumor of approximately 30.0 cm in diameter. Endoscopic ultrasonography showed an extraluminal growing tumor arising from the muscular layer of the gastric wall. Abdominal contrast-enhanced CT revealed a giant cystic-solid tumor originated from the big curved side of gastric corpus, which was considered as a plexiform fibromyxoma of the stomach (Fig. 1A and B). An exploratory laparotomy was performed and the tumor was confirmed originated from the stomach and it was rich in mucus (Fig. 2A). The tumor was completely removed with partial gastrectomy and postoperative pathological examination revealed gastric myxoid epithelioid tumor (Fig. 2B and C). Immunohistochemistry showed that CD117, CD34 and Dog-1 were positive, but CK and Melan-A were negative, which was consistent with the diagnosis of gastrointestinal stromal tumor. Gene detection revealed the C-kit gene was wild-type but the exon 18 of PDGFR-α gene was mutant-type (D842V). The patient recovered well after surgery and is undergoing the treatment of avapritinib.
      Fig 1
      Fig. 1Abdominal contrast-enhanced CT revealed a giant cystic-solid tumor originated from the big curved side of gastric corpus.
      Fig 2
      Fig. 2Postoperative specimen revealed the tumor was rich in mucus (A); pathological examination revealed gastric myxoid epithelioid tumor (B, HE stain × 200; C, HE stain × 400).
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