Perivascular epithelioid cell tumors (PEComa) of the female genital tract: A challenging question for gynaecologic oncologist and pathologist
Perivascular epithelioid cell tumors (PEComa)s are mesenchymal neoplasms composed of perivascular epithelioid cells, which express both melanocytic and myogenic markers. These neoplastic cells are thought to arise from undifferentiated cells of the neural crest, or from myoblastic cells harbouring a...
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| Format: | Article |
| Language: | English |
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Elsevier
2020-08-01
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| Series: | Gynecologic Oncology Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2352578920300692 |
| _version_ | 1828308454416056320 |
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| author | Angiolo Gadducci Gian Franco Zannoni |
| author_facet | Angiolo Gadducci Gian Franco Zannoni |
| author_sort | Angiolo Gadducci |
| collection | DOAJ |
| description | Perivascular epithelioid cell tumors (PEComa)s are mesenchymal neoplasms composed of perivascular epithelioid cells, which express both melanocytic and myogenic markers. These neoplastic cells are thought to arise from undifferentiated cells of the neural crest, or from myoblastic cells harbouring a molecular alteration leading to expression of melanocytic markers, or from pericytic cells. The majority of cases are benign, but a small subset behaves in a malignant fashion. Unfortunately, given their rarity, reliable criteria for predicting malignancy have yet to be established. PEComas of the female genital tract account for nearly 25% of PEComas of all body sites, and the most common site of occurrence is the uterine corpus; less common sites include the cervix, adnexa, vagina/vulva or broad or round ligament exceptionally. The present review aims to elucidate the clinical, pathological and molecular features of gynecological PEComas. Moreover, prognostic and therapeutic implications are also discussed. |
| first_indexed | 2024-04-13T15:13:39Z |
| format | Article |
| id | doaj.art-a3a15ecdd332439ca80563bdf66cb180 |
| institution | Directory Open Access Journal |
| issn | 2352-5789 |
| language | English |
| last_indexed | 2024-04-13T15:13:39Z |
| publishDate | 2020-08-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Gynecologic Oncology Reports |
| spelling | doaj.art-a3a15ecdd332439ca80563bdf66cb1802022-12-22T02:41:57ZengElsevierGynecologic Oncology Reports2352-57892020-08-0133100603Perivascular epithelioid cell tumors (PEComa) of the female genital tract: A challenging question for gynaecologic oncologist and pathologistAngiolo Gadducci0Gian Franco Zannoni1Department of Clinical and Experimental Medicine, Division of Gynecology and Obstetrics, University of Pisa, Italy; Corresponding author at: Department of Procreative Medicine, Division of Gynecology and Obstetrics, University of Pisa, Via Roma 56, Pisa 56127, Italy.Division of Anatomic Pathology and Histology – Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore School of Medicine, Rome, ItalyPerivascular epithelioid cell tumors (PEComa)s are mesenchymal neoplasms composed of perivascular epithelioid cells, which express both melanocytic and myogenic markers. These neoplastic cells are thought to arise from undifferentiated cells of the neural crest, or from myoblastic cells harbouring a molecular alteration leading to expression of melanocytic markers, or from pericytic cells. The majority of cases are benign, but a small subset behaves in a malignant fashion. Unfortunately, given their rarity, reliable criteria for predicting malignancy have yet to be established. PEComas of the female genital tract account for nearly 25% of PEComas of all body sites, and the most common site of occurrence is the uterine corpus; less common sites include the cervix, adnexa, vagina/vulva or broad or round ligament exceptionally. The present review aims to elucidate the clinical, pathological and molecular features of gynecological PEComas. Moreover, prognostic and therapeutic implications are also discussed.http://www.sciencedirect.com/science/article/pii/S2352578920300692Uterine perivascular epithelioid cell tumors (PEComas)Cervical PEComasOvarian PEComasMelanocytic markersMyogenic markers |
| spellingShingle | Angiolo Gadducci Gian Franco Zannoni Perivascular epithelioid cell tumors (PEComa) of the female genital tract: A challenging question for gynaecologic oncologist and pathologist Gynecologic Oncology Reports Uterine perivascular epithelioid cell tumors (PEComas) Cervical PEComas Ovarian PEComas Melanocytic markers Myogenic markers |
| title | Perivascular epithelioid cell tumors (PEComa) of the female genital tract: A challenging question for gynaecologic oncologist and pathologist |
| title_full | Perivascular epithelioid cell tumors (PEComa) of the female genital tract: A challenging question for gynaecologic oncologist and pathologist |
| title_fullStr | Perivascular epithelioid cell tumors (PEComa) of the female genital tract: A challenging question for gynaecologic oncologist and pathologist |
| title_full_unstemmed | Perivascular epithelioid cell tumors (PEComa) of the female genital tract: A challenging question for gynaecologic oncologist and pathologist |
| title_short | Perivascular epithelioid cell tumors (PEComa) of the female genital tract: A challenging question for gynaecologic oncologist and pathologist |
| title_sort | perivascular epithelioid cell tumors pecoma of the female genital tract a challenging question for gynaecologic oncologist and pathologist |
| topic | Uterine perivascular epithelioid cell tumors (PEComas) Cervical PEComas Ovarian PEComas Melanocytic markers Myogenic markers |
| url | http://www.sciencedirect.com/science/article/pii/S2352578920300692 |
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