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

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.

• Authors: Byung Su Kwon, Dong Soo Suh, Nam Kyung Lee, Yong Jung Song, Kyung Un Choi, Ki Hyung Kim
• Journal: European journal of medical research (Eur J Med Res) Vol. 22 Issue 1 Pg. 7 (Mar 07 2017) ISSN: 2047-783X [Electronic] England
• PMID: 28270196 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Diagnosis, Differential
• Female
• Humans
• Middle Aged
• Perivascular Epithelioid Cell Neoplasms (diagnostic imaging, pathology)
• Uterine Neoplasms (diagnostic imaging, pathology)