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A 78\u2010year\u2010old 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.<\/jats:p>","DOI":"10.1002\/ccr3.2676","type":"journal-article","created":{"date-parts":[[2020,2,6]],"date-time":"2020-02-06T05:09:51Z","timestamp":1580965791000},"page":"553-558","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Perivascular epithelioid cell tumor (PEComa) of the pterygopalatine fossa"],"prefix":"10.1002","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3682-336X","authenticated-orcid":false,"given":"Michael I.","family":"Dougherty","sequence":"first","affiliation":[{"name":"Department of Otolaryngology\u2010 Head and Neck Surgery University of Virginia School of Medicine Charlottesville Virginia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Spencer C.","family":"Payne","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2010 Head and Neck Surgery University of Virginia School of Medicine Charlottesville Virginia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akriti","family":"Gupta","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Virginia School of Medicine Charlottesville Virginia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jose L.","family":"Mattos","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2010 Head and Neck Surgery University of Virginia School of Medicine Charlottesville Virginia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,2,5]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.anndiagpath.2015.06.003"},{"issue":"3","key":"e_1_2_6_3_1","first-page":"198","article-title":"Early onset of a nasal perivascular epithelioid cell neoplasm not related to tuberous sclerosis complex","volume":"32","author":"Gana S","year":"2012","journal-title":"Acta Otorhinolaryngol Ital"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1590\/1516-3180.2017.0120050717"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/cup.13061"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.pas.0000173232.22117.37"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12105-011-0268-9"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.anl.2015.11.003"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/hed.23972"},{"issue":"4","key":"e_1_2_6_10_1","first-page":"E20","article-title":"Cutaneous metastasis of a perivascular epithelioid cell tumor","volume":"93","author":"Wu J","year":"2014","journal-title":"Cutis"},{"key":"e_1_2_6_11_1","first-page":"5749421","article-title":"An inguinal perivascular epithelioid cell tumor metastatic to the orbit","volume":"2018","author":"Tynski Z","year":"2018","journal-title":"Case Rep Pathol"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.pas.0000128668.34934.81"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2017.11.016"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1177\/014556131509400713"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.2500\/ajra.2012.26.3760"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1177\/106689690100900410"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1827.2009.02443.x"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12105-013-0470-z"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1177\/000348941312201110"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.otohns.2009.03.009"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1080\/00016480510029419"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaoto.2013.4397"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1590\/S1808-86942011000200021"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2559.2002.01424_4.x"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/hed.2880160312"},{"issue":"9","key":"e_1_2_6_26_1","first-page":"789","article-title":"Mucocutaneous angiomyolipoma. 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