Malignant perivascular epithelioid cell tumor of the oropharynx with strong TFE3 expression mimicking alveolar soft part sarcoma: a case report and review of the literature

Karan Saluja; Jaiyeola Thomas; Songlin Zhang; Erich M Sturgis; Kunal S. Jain; Victor G. Prieto; Adel K. El-Naggar; Diana Bell

doi:10.1016/j.humpath.2017.11.016

Malignant perivascular epithelioid cell tumor of the oropharynx with strong TFE3 expression mimicking alveolar soft part sarcoma: a case report and review of the literature

Karan Saluja, Jaiyeola Thomas, Songlin Zhang, Erich M Sturgis, Kunal S. Jain, Victor G. Prieto, Adel K. El-Naggar, Diana Bell

Research output: Contribution to journal › Article › peer-review

12 Scopus citations

Abstract

Perivascular epithelioid cell tumors (PEComas) in the head and neck region are rare, with 26 cases described in literature. These distinct mesenchymal tumors normally express both myoid and melanocytic markers. We here report an interesting and challenging case of malignant PEComa that showed transcription factor E3 (TFE3) protein expression and rearrangement, paucity of muscle and melanocytic marker expression, and morphologically mimicked alveolar soft part sarcoma. Awareness of this morphologic pitfall and recognition of TFE3 gene–rearranged PEComa, as a distinct subtype of PEComa, is essential to avoid misdiagnosis.

Original language	English (US)
Pages (from-to)	149-155
Number of pages	7
Journal	Human Pathology
Volume	76
DOIs	https://doi.org/10.1016/j.humpath.2017.11.016
State	Published - Jun 2018

Keywords

Head and neck
Malignant
Oropharynx
PEComa
Perivascular epithelioid cell tumor
TFE3

ASJC Scopus subject areas

Pathology and Forensic Medicine

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10.1016/j.humpath.2017.11.016

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title = "Malignant perivascular epithelioid cell tumor of the oropharynx with strong TFE3 expression mimicking alveolar soft part sarcoma: a case report and review of the literature",

abstract = "Perivascular epithelioid cell tumors (PEComas) in the head and neck region are rare, with 26 cases described in literature. These distinct mesenchymal tumors normally express both myoid and melanocytic markers. We here report an interesting and challenging case of malignant PEComa that showed transcription factor E3 (TFE3) protein expression and rearrangement, paucity of muscle and melanocytic marker expression, and morphologically mimicked alveolar soft part sarcoma. Awareness of this morphologic pitfall and recognition of TFE3 gene–rearranged PEComa, as a distinct subtype of PEComa, is essential to avoid misdiagnosis.",

keywords = "Head and neck, Malignant, Oropharynx, PEComa, Perivascular epithelioid cell tumor, TFE3",

author = "Karan Saluja and Jaiyeola Thomas and Songlin Zhang and Sturgis, {Erich M} and Jain, {Kunal S.} and Prieto, {Victor G.} and El-Naggar, {Adel K.} and Diana Bell",

note = "Publisher Copyright: {\textcopyright} 2017 Elsevier Inc.",

year = "2018",

month = jun,

doi = "10.1016/j.humpath.2017.11.016",

language = "English (US)",

volume = "76",

pages = "149--155",

journal = "Human Pathology",

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T2 - a case report and review of the literature

AU - Saluja, Karan

AU - Thomas, Jaiyeola

AU - Zhang, Songlin

AU - Sturgis, Erich M

AU - Jain, Kunal S.

AU - Prieto, Victor G.

AU - El-Naggar, Adel K.

AU - Bell, Diana

PY - 2018/6

Y1 - 2018/6

N2 - Perivascular epithelioid cell tumors (PEComas) in the head and neck region are rare, with 26 cases described in literature. These distinct mesenchymal tumors normally express both myoid and melanocytic markers. We here report an interesting and challenging case of malignant PEComa that showed transcription factor E3 (TFE3) protein expression and rearrangement, paucity of muscle and melanocytic marker expression, and morphologically mimicked alveolar soft part sarcoma. Awareness of this morphologic pitfall and recognition of TFE3 gene–rearranged PEComa, as a distinct subtype of PEComa, is essential to avoid misdiagnosis.

AB - Perivascular epithelioid cell tumors (PEComas) in the head and neck region are rare, with 26 cases described in literature. These distinct mesenchymal tumors normally express both myoid and melanocytic markers. We here report an interesting and challenging case of malignant PEComa that showed transcription factor E3 (TFE3) protein expression and rearrangement, paucity of muscle and melanocytic marker expression, and morphologically mimicked alveolar soft part sarcoma. Awareness of this morphologic pitfall and recognition of TFE3 gene–rearranged PEComa, as a distinct subtype of PEComa, is essential to avoid misdiagnosis.

KW - Head and neck

KW - Malignant

KW - Oropharynx

KW - PEComa

KW - Perivascular epithelioid cell tumor

KW - TFE3

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SN - 0046-8177

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ER -

Malignant perivascular epithelioid cell tumor of the oropharynx with strong TFE3 expression mimicking alveolar soft part sarcoma: a case report and review of the literature

Abstract

Keywords

ASJC Scopus subject areas

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