Obstet Gynecol Sci.  2014 Sep;57(5):409-411. 10.5468/ogs.2014.57.5.409.

A 6-year-old girl with vaginal spotting who was diagnosed with perivascular epithelioid cell neoplasm after vaginoscopic resection

• Cho HJ
• Lee MK
• Kang BM
• Kim SH
• Chae HD
• Kim CH

• KMID: 2051725
• DOI: http://doi.org/10.5468/ogs.2014.57.5.409

Abstract

Perivascular epithelioid cell neoplasm (PEComa) is a rare tumor with unknown malignant potential. We report a case of a 6-year-old child with history of brain tumor (pineoblastoma), who presented with intermittent vaginal spotting for 6 months. A vaginoscopy revealed a 1.5x1.0-cm mass on the vaginal wall. Pathological examination demonstrated that the tumor was composed of clear cells with organoid patterns, which were immunohistochemically positive for HMB-45 and TFE3, and negative for CK, HNF1-B, SOX10, Melan A, and S-100 protein. These findings were consistent with PEComa arising from the vagina. Regular follow-up with magnetic resonance imaging has shown no signs of recurrence. This case shows that early detection of PEComa and subsequent regular follow-ups are important because of the neoplasm's unknown malignant potential.

Keyword

Perivascular epithelioid cell neoplasms; Resection; Vagina; Vaginoscopy

MeSH Terms

Brain Neoplasms
Child*
Epithelioid Cells*
Female
Follow-Up Studies
Humans
Magnetic Resonance Imaging
MART-1 Antigen
Metrorrhagia*
Organoids
Perivascular Epithelioid Cell Neoplasms
Recurrence
S100 Proteins
Vagina
MART-1 Antigen
S100 Proteins

Figure

• 

  Fig. 1 (A) A 1.5×1.0-cm benign looking mass (M) on vaginoscopy arising from the left lateral vaginal wall. (B) Vaginal wall (arrow) after complete resection.

• 

  Fig. 2 (A) Microscopic examination showed a clear cells with organoid patterns (H&E, ×200). (B) Immunohistochemistry of the tumor cells was positive for HMB-45 (HMB-45, ×200).

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