An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the Orbit

An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the Orbit

Malignant PEComas are rare mesenchymal neoplasms. These tumors harbor distinct myomelanocytic phenotype. The PEComa family of tumors includes lymphangioleiomyomatosis, angiomyolipoma, clear cell sugar tumor of the lung, and myomelanocytic tumor of the falciparum ligament/ligamentum teres. PEComas ha...

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Main Authors: Zofia Tynski, Way Chiang, Albert Barrett
Format: Article
Language:English
Published: Hindawi Limited 2018-01-01
Series:Case Reports in Pathology
Online Access:http://dx.doi.org/10.1155/2018/5749421
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spelling doaj-de7a16425eba467ebf70cce330b2d0f92020-11-25T00:17:40ZengHindawi LimitedCase Reports in Pathology2090-67812090-679X2018-01-01201810.1155/2018/57494215749421An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the OrbitZofia Tynski0Way Chiang1Albert Barrett2Department of Pathology, Clarion Hospital, Clarion, PA 16214, USADepartment of Family Medicine, Clarion Hospital, Clarion, PA 16214, USADepartment of Radiology, Clarion Hospital, Clarion, PA 16214, USAMalignant PEComas are rare mesenchymal neoplasms. These tumors harbor distinct myomelanocytic phenotype. The PEComa family of tumors includes lymphangioleiomyomatosis, angiomyolipoma, clear cell sugar tumor of the lung, and myomelanocytic tumor of the falciparum ligament/ligamentum teres. PEComas have no known normal cell counterpart. Majority of PEComas are benign and occur predominantly in the middle-age women. These tumors are commonly encountered in the uterus. Herein, we report a 20-year-old woman with a left inguinal mass metastatic to orbit, brain, lumbar spine, and skin at presentation. To our knowledge, this is the first case of metastatic PEComa to the orbit. This is the third case of primary PEComa of the inguinal area.http://dx.doi.org/10.1155/2018/5749421
collection DOAJ
language English
format Article
sources DOAJ
author Zofia Tynski
Way Chiang
Albert Barrett
spellingShingle Zofia Tynski
Way Chiang
Albert Barrett
An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the Orbit
Case Reports in Pathology
author_facet Zofia Tynski
Way Chiang
Albert Barrett
author_sort Zofia Tynski
title An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the Orbit
title_short An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the Orbit
title_full An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the Orbit
title_fullStr An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the Orbit
title_full_unstemmed An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the Orbit
title_sort inguinal perivascular epithelioid cell tumor metastatic to the orbit
publisher Hindawi Limited
series Case Reports in Pathology
issn 2090-6781
2090-679X
publishDate 2018-01-01
description Malignant PEComas are rare mesenchymal neoplasms. These tumors harbor distinct myomelanocytic phenotype. The PEComa family of tumors includes lymphangioleiomyomatosis, angiomyolipoma, clear cell sugar tumor of the lung, and myomelanocytic tumor of the falciparum ligament/ligamentum teres. PEComas have no known normal cell counterpart. Majority of PEComas are benign and occur predominantly in the middle-age women. These tumors are commonly encountered in the uterus. Herein, we report a 20-year-old woman with a left inguinal mass metastatic to orbit, brain, lumbar spine, and skin at presentation. To our knowledge, this is the first case of metastatic PEComa to the orbit. This is the third case of primary PEComa of the inguinal area.
url http://dx.doi.org/10.1155/2018/5749421
work_keys_str_mv AT zofiatynski aninguinalperivascularepithelioidcelltumormetastatictotheorbit
AT waychiang aninguinalperivascularepithelioidcelltumormetastatictotheorbit
AT albertbarrett aninguinalperivascularepithelioidcelltumormetastatictotheorbit
AT zofiatynski inguinalperivascularepithelioidcelltumormetastatictotheorbit
AT waychiang inguinalperivascularepithelioidcelltumormetastatictotheorbit
AT albertbarrett inguinalperivascularepithelioidcelltumormetastatictotheorbit
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