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Eur J Med Res. 2017 Mar 7;22(1):7. doi: 10.1186/s40001-017-0248-y.

Two cases of perivascular epithelioid cell tumor of the uterus: clinical, radiological and pathological diagnostic challenge.

Author information

1
Department of Obstetrics and Gynecology, Pusan National University School of Medicine, 179, Gudeok-Ro, Seo-Gu, Busan, 49241, South Korea.
2
Biomedical Research Institute and Pusan Cancer Center, Pusan National University Hospital, 179, Gudeok-Ro, Seo-Gu, Busan, 49241, South Korea.
3
Department of Radiology, Pusan National University School of Medicine, Busan, 49241, South Korea.
4
Department of Pathology, Pusan National University School of Medicine, Busan, 49241, South Korea.
5
Department of Obstetrics and Gynecology, Pusan National University School of Medicine, 179, Gudeok-Ro, Seo-Gu, Busan, 49241, South Korea. ghkim@pusan.ac.kr.
6
Biomedical Research Institute and Pusan Cancer Center, Pusan National University Hospital, 179, Gudeok-Ro, Seo-Gu, Busan, 49241, South Korea. ghkim@pusan.ac.kr.

Abstract

BACKGROUND:

Perivascular epithelioid cell tumor (PEComa) is a rare subtype of mesenchymal origin tumor composed of epithelioid cells which exhibits immunohistochemical co-expressions of melanocytic markers and smooth muscle markers.

CASE PRESENTATION:

In the first case, malignant uterine PEComa with vaginal and multiple lung metastasis was misdiagnosed preoperatively as uterine leiomyosarcoma despite a preoperative punch biopsy and immunohistochemical analysis of the metastatic vaginal mass. In the second case, synchronous uterine PEComa showing benign histology with lymph node involvement was incidentally detected after a staging operation for ovarian cancer. Definitive diagnosis of uterine PEComa was achieved only after hysterectomy despite preoperative assessment with pelvic magnetic resonance imaging and punch biopsy of metastatic lesion.

CONCLUSION:

The authors report two rare cases of uterine PEComa diagnosed postoperatively based on the morphologic and immunohistochemical features.

KEYWORDS:

Immunohistochemistry; Morphology; Perivascular epithelioid cell tumor (PEComa)

PMID:
28270196
PMCID:
PMC5341375
DOI:
10.1186/s40001-017-0248-y
[Indexed for MEDLINE]
Free PMC Article

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