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Journal of Radiological Review 2020 September-October;7(5):404-9

DOI: 10.23736/S2723-9284.20.00034-6

Copyright © 2020 EDIZIONI MINERVA MEDICA

language: English, Italian

Computed tomography and virtual gastroscopy: diagnosis in a rare case of gastric PEComa

Roberto PRIOTTO 1, Ilaria BARALIS 1 , Paolo DEMARIA 1, Alberto NEGRI 1, Andrea CERUTTI 1, Alessandra MARANO 2, Paolo VIOLINO 1, Maurizio GROSSO 1

1 Department of Diagnostic and Interventional Radiology, S. Croce e Carle Hospital, Cuneo, Italy; 2 Unit of General and Oncologic Surgery, Department of Surgery, S. Croce e Carle Hospital, Cuneo, Italy


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PEComas (perivascular epithelioid cell tumors) are rare neoplasm, described for the first time in 1992, with tumor cells mostly with epithelioid appearance, associated with blood enlarged vessels and spindle cells arranged in sheets and nest pattern, with abundant clear eosinophilic cytoplasm, surrounded by capillary vasculature. There is not a typical anatomic site of origin, but arise most commonly at uterus; gastrointestinal tract represent the second most involved location counting 20% to 25% of all reported cases. Biological behavior and peculiar features of PEComas are still unclear because of few data available due to rareness of this kind of neoplasm. Furthermore, the few cases described in literature do not allow to define a clear pattern or typical features at radiological exam, particularly computed tomography (CT) scan. Surgical treatment is the mainstay of management, eventually followed by adjuvant thearapies, even if these therapies have not enough literature evidence that could make survival benefit or improving the progression free survival. This article reports a rare case of PEComa gastric localization highlighting the role of CT with gas stomach distension, myorelaxation and following endoscopic virtual navigation, which confirmed the lesion yet diagnosed with upper endoscopy. CT and virtual gastroscopy allowed a better definition of the origin site and more information about presurgical staging. Nevertheless it is a rare entity, PEComas should be considered as a differential diagnosis with the more widespread GIST when a infiltrative gastrointestinal lesion is detected.


KEY WORDS: Perivascular epithelioid cell neoplasms; Gastroscopy; Stomach neoplasms

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