J Pathol Transl Med.  2019 Jan;53(1):62-65. 10.4132/jptm.2018.08.30.

TFE3-Expressing Perivascular Epithelioid Cell Tumor of the Breast

• Kim H
• Kim J
• Lee SK
• Cho EY
• Cho SY

• KMID: 2437580
• DOI: http://doi.org/10.4132/jptm.2018.08.30

Abstract

Perivascular epithelioid cell tumor (PEComa) is a very rare mesenchymal tumor with a distinctive morphology and immunophenotype. PEComas usually harbor TSC2 alterations, although TFE3 translocations, which occur in MiT family translocation renal cell carcinoma and alveolar soft part sarcoma, are also possible. We recently experienced a case of PEComa with TFE3 expression arising in the breast. An 18-year-old female patient presented with a right breast mass. Histologically, the tumor consisted of epithelioid cells with alveolar structure and showed a diffuse strong expression of HMB45 and TFE3. TSC2 was preserved. Melan A and smooth muscle actin were negative. To our knowledge, this is the first Korean case of PEComa of the breast that intriguingly presented with TFE3 expression.

Keyword

Perivascular epithelioid cell neoplasms; TFE3; Breast

MeSH Terms

Actins
Adolescent
Breast*
Carcinoma, Renal Cell
Epithelioid Cells*
Female
Humans
MART-1 Antigen
Muscle, Smooth
Perivascular Epithelioid Cell Neoplasms
Sarcoma, Alveolar Soft Part
Actins
MART-1 Antigen

Figure

• 

  Fig. 1. Microscopic findings of the TFE3-expressing breast perivascular epithelioid cell tumor. (A) Epithelioid cells were arranged in a nested architecture with surrounding branching thin-walled vessels. (B) The individual epithelioid cells show clear to granular cytoplasm. (C) Occasional melanin pigments are noted.

• 

  Fig. 2. The immunohistochemical findings of the TFE3-expressing breast perivascular epithelioid cell tumor. The tumor is diffusely positive for human melanoma black 45 (A) and shows nuclear positivity for TFE3 (B) and progesterone receptor (C).

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