title: Perivascular epithelioid cell neoplasm (PEComa) of the uterus: A systematic review.
abstract: BACKGROUND
  Perivascular epithelioid cell neoplasm (PEComa) is a rare mesenchymal tumor. Gynecological PEComas account for just over one-fourth of the overall PEComa cases reported in the literature. Surgery is the most recommended primary treatment while adjuvant therapy is generally reserved for high-risk cases. However, the best management of this neoplasia has not been well established, primarily because of the paucity of cases described to date.
  
  
  OBJECTIVE
  The aim of this systematic review is to summarize what is known thus far regarding the etiopathogenesis, clinical and pathologic features of PEComas, focusing also on the most valid treatment options for uterine cases.
  
  
  METHODS
  Pubmed articles on PEComas published in various journals over the past 70 years were analyzed.
  Although the optimal treatment of gynecological PEComas is controversial, surgical resection remains the cornerstone. The use of adjuvant treatment is warranted in high risk patients to increase disease control. A multidisciplinary approach should be key in treatment decision-making regarding gynecological PEComas.
authors.affiliation: Department of Gynecology, Obstetrics and Urological Sciences, "Sapienza" University of Rome, Viale Regina Elena 326, 00161 Rome, Italy.
authors.forename: Angela
authors.initials: A
authors.lastname: Musella
authors.affiliation: Department of Radiotherapy, Policlinico Umberto I "Sapienza" University of Rome, Viale Regina Elena 326, 00161 Rome, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: De Felice
authors.affiliation: "Sapienza" University of Rome, Viale Regina Elena 326, 00161 Rome, Italy.
authors.forename: A Kyriacos
authors.initials: AK
authors.lastname: Kyriacou
authors.affiliation: San Giovanni-Addolorata Company Hospital, via Amba Aradam 9, 00184 Rome, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Barletta
authors.affiliation: Department of Surgical Sciences, "Sapienza" University of Rome, Viale Regina Elena 324, 00161 Rome, Italy.
authors.forename: Filippo Maria
authors.initials: FM
authors.lastname: Di Matteo
authors.affiliation: Department of Gynecology, Obstetrics and Urological Sciences, "Sapienza" University of Rome, Viale Regina Elena 326, 00161 Rome, Italy.
authors.forename: Claudia
authors.initials: C
authors.lastname: Marchetti
authors.affiliation: Department of Surgery "Pietro Valdoni", Policlinico Umberto I "Sapienza" University of Rome, Viale Regina Elena 326, 00161 Rome, Italy.
authors.forename: Luciano
authors.initials: L
authors.lastname: Izzo
authors.affiliation: Department of Gynecology, Obstetrics and Urological Sciences, "Sapienza" University of Rome, Viale Regina Elena 326, 00161 Rome, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Monti
authors.affiliation: Department of Gynecology, Obstetrics and Urological Sciences, "Sapienza" University of Rome, Viale Regina Elena 326, 00161 Rome, Italy.
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Benedetti Panici
authors.affiliation: Department of Surgical Sciences, "Sapienza" University of Rome, Viale Regina Elena 324, 00161 Rome, Italy.
authors.forename: Adriano
authors.initials: A
authors.lastname: Redler
authors.affiliation: Department of Surgical Sciences, "Sapienza" University of Rome, Viale Regina Elena 324, 00161 Rome, Italy. Electronic address: vito.dandrea@uniroma1.it.
authors.forename: Vito
authors.initials: V
authors.lastname: D'Andrea
mesh_terms: D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D054973:Perivascular Epithelioid Cell Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D014594:Uterine Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy
keywords: Gynecological; Neoplasm; PEComa; Perivascular epithelioid cell; Uterus