title: Perivascular epithelioid cell tumor located retroperitoneally with pulmonary lymphangioleiomyomatosis: report of a case.
abstract: Perivascular epithelioid cell neoplasms, also known as "PEComas", are unusual mesenchymal tumors, exhibiting perivascular epithelioid cell differentiation and characterized by a mixed myogenic and melanocytic phenotype. "PEComas not otherwise specified" (PEComas-NOS) are especially rare; consequently, there are no published large series, but only case reports. These tumors are rarely located retroperitoneally, with only about 15 such cases reported. We report a case of pulmonary diffuse lymphangioleiomyomatosis with large retroperitoneal PEComa-NOS in a 66-year-old woman. Treatment consisted only of tumor resection, without additional adjuvant therapy. We emphasize the importance of correct immunohistochemistry diagnosis, initiation of recommended treatment, and surveillance of this unique family of tumors.
authors.affiliation: 2nd Division of General Surgery, Department of Medical and Surgical Sciences, Brescia Civic Hospital, P.le Spedali Civili 1, 25124, Brescia, Italy, giacomopata@alice.it.
authors.forename: Giacomo
authors.initials: G
authors.lastname: Pata
authors.forename: Andrea
authors.initials: A
authors.lastname: Tironi
authors.forename: Leonardo
authors.initials: L
authors.lastname: Solaini
authors.forename: Travaglia
authors.initials: T
authors.lastname: Tiziano
authors.forename: Fulvio
authors.initials: F
authors.lastname: Ragni
mesh_terms: D000368:Aged; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D009378:Neoplasms, Multiple Primary; D054973:Perivascular Epithelioid Cell Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D012186:Retroperitoneal Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome