Perivascular epithelioid cell tumor (PEComa) of the pterygopalatine fossa. Issue 3 (5th February 2020)

• Record Type: Journal Article
• Authors: Dougherty, Michael I.; Payne, Spencer C.; Gupta, Akriti; Mattos, Jose L.
• Is Part Of: Clinical case reports. Volume 8:Issue 3(2020)
• Page Start: 553
• Page End: 558
• Publication Date: 2020-02-05
• Subjects: head and neck -- PEComa -- pterygopalatine fossa -- retroantral -- skull base tumor; Medicine -- Periodicals; 616.09
• DOI: 10.1002/ccr3.2676
• Languages: English
• ISSNs: 2050-0904

Abstract

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. Abstract : While perivascular epithelioid cell tumor (PEComa) is a rare pathology in the head and neck, it should be considered on the differential for atypical skull base tumors.