Cutaneous PEComa

Primary cutaneous perivascular epithelioid cell tumor: A clinicopathological and molecular reappraisal

“…CD68 immunopositivity was present among neoplastic cells in 88% of our cases, in contrast to 45% in previous reports. 2,14,15,20 Histologic criteria for establishing malignancy have been proposed for visceral PEComas and suggested to evaluate pcPEComas. 16,28 None of our cases fulfilled such criteria because they were small, had a virtually null mitotic count, and lacked necrosis or vascular invasion.…”

A rare case of primary cutaneous malignant perivascular epithelioid cell tumor and review of the literature

“…The histopathologic differential of PEComas is broad and includes a balloon cell melanoma, balloon cell nevus, and clear cell sarcoma, but these lesions will show strong and diffuse S100 and SOX‐10 positivity unlike PEComas 10 . Clear cell sarcomas can also be differentiated by identification of a t(12;22) (q13; q12) translocation 12 . Additionally, the cellular proliferation of melanocytic lesions with clear‐cell change often contains a dermal‐epidermal junctional component, which is not seen in PEComas 10 .…”

“…PEComas are rare and more commonly present in organs such as the uterus, retroperitoneum, gastrointestinal tract, urinary bladder, prostate, bone, and soft tissues 1,4 . Cutaneous PEComas are even more unusual and represent only around 8% of PEComas 12 . PEComas generally behave in a benign fashion; however, malignant PEComas have been rarely described.…”

Angiomyolipoma of the Glabellar Region

“…Although TSC is an autosomal dominant hereditary disease that involves chromosome 9 or chromosome 16 [ 15 ] and mutations in tumor suppressor gene TSC1 (encodes protein hamartin) or TSC2 (encodes protein tuberin) genes [ 17 18 ], sporadic form that might arise from spontaneous mutations, have also been reported [ 15 ]. TSC is a multisystem disease with autosomal dominant inheritance, variable expressivity, and high penetrance that can be characterized with seven different hamartomas (cortical tubers, subependymal nodule, retinal phakoma, facial angiofibromas, ungual firbromas, fibrous forehead plaque, and multiple renal angiomyopliomas) [ 12 ].…”

“…The extrarenal AML has several synonyms that include mucocutaneous AML (MCAML) (proposed by Watanabe and Suzuki, 1999) [ 18 ], Cutaneous AML, and Cutaneous angiolipoleiomyoma (CALLM) [ 7 14 ]. Cutaneous AMLs are usually painless solitary lesions located in the acral area [ 4 ].…”