Perivascular epithelioid cell tumor (PEComa) of the pterygopalatine fossa

Autor: Michael I. Dougherty, Spencer C. Payne, Akriti Gupta, Jose L. Mattos
Jazyk: angličtina
Rok vydání: 2020
Předmět:
Zdroj: Clinical Case Reports, Vol 8, Iss 3, Pp 553-558 (2020)
Druh dokumentu: article
ISSN: 2050-0904
DOI: 10.1002/ccr3.2676
Popis: Abstract Perivascular epithelioid cell tumors (PEComas) are a rare group of mesenchymal tumors associated with tuberous sclerosis. These tumors are typically treated with resection and rarely recur or exhibit malignant behavior. A 78‐year‐old woman presented with an incidentally discovered pterygopalatine fossa/retroantral mass. Excisional biopsy was performed and revealed pathology consistent with PEComa. Upon review of the literature, there have been 43 reported cases of PEComa of the head and neck. There is only one previously reported case of PEComa in the skull base, and none reported in the pterygopalatine fossa. Of note, the previously reported case of skull base PEComa involved an aggressive tumor with widespread metastasis. Here, we report the first case of a PEComa of the pterygopalatine fossa/retroantral region, which was treated conservatively. This rare pathology should be considered in the differential diagnosis for atypical skull base tumors.
Databáze: Directory of Open Access Journals
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