Format

Send to

Choose Destination
Int J Surg. 2015 Jul;19:1-5. doi: 10.1016/j.ijsu.2015.05.002. Epub 2015 May 14.

Perivascular epithelioid cell neoplasm (PEComa) of the uterus: A systematic review.

Author information

1
Department of Gynecology, Obstetrics and Urological Sciences, "Sapienza" University of Rome, Viale Regina Elena 326, 00161 Rome, Italy.
2
Department of Radiotherapy, Policlinico Umberto I "Sapienza" University of Rome, Viale Regina Elena 326, 00161 Rome, Italy.
3
"Sapienza" University of Rome, Viale Regina Elena 326, 00161 Rome, Italy.
4
San Giovanni-Addolorata Company Hospital, via Amba Aradam 9, 00184 Rome, Italy.
5
Department of Surgical Sciences, "Sapienza" University of Rome, Viale Regina Elena 324, 00161 Rome, Italy.
6
Department of Surgery "Pietro Valdoni", Policlinico Umberto I "Sapienza" University of Rome, Viale Regina Elena 326, 00161 Rome, Italy.
7
Department of Surgical Sciences, "Sapienza" University of Rome, Viale Regina Elena 324, 00161 Rome, Italy. Electronic address: vito.dandrea@uniroma1.it.

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.

OBJECTIVES:

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.

DATA SOURCES:

Pubmed articles on PEComas published in various journals over the past 70 years were analyzed.

CONCLUSIONS AND KEY FINDINGS:

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.

KEYWORDS:

Gynecological; Neoplasm; PEComa; Perivascular epithelioid cell; Uterus

PMID:
25981307
DOI:
10.1016/j.ijsu.2015.05.002
[Indexed for MEDLINE]
Free full text

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center