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Perivascular epithelioid cell tumor (PEComa) of the cystic duct

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Abstract

Perivascular epithelioid cell tumors, also known as PEComas, are rare mesenchymal tumors composed mainly of epithelioid cells found in perivascular tissue. PEComas occur most frequently in the kidney, uterus, the gastrointestinal tract, liver, and retroperitoneum; those originating in the biliary tree are extremely rare. We report a case of benign PEComa of the cystic duct with positive TFE3 staining on immunohistochemistry.

A 66-year-old woman was referred for a 20 mm mass adjacent to the common bile duct discovered incidentally on abdominal ultrasound. Laboratory data including tumor markers were unremarkable. The tumor appeared to arise from the cystic duct, showed early enhancement, and compressed the common bile duct on imaging studies. Endoscopic ultrasound-guided fine-needle aspiration revealed round- and spindle-shaped atypical cells with eosinophilic cytoplasm and brown deposits suggestive of melanin granules. Histological examination of the resected specimen revealed a tumor consisting of epithelioid cells forming an alveolar structure, with melanin pigmentation. Immunohistochemistry was positive for HMB-45 and TFE3, consistent with benign pigmented PEComa of the cystic duct. Melanotic, myogenic, and TFE3 staining are helpful when diagnosing PEComas arising in unusual locations.

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Data availability

The data that support the findings of this study are available from the corresponding author upon reasonable request.

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Funding

The authors have received no funding for the publication of this article.

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Authors and Affiliations

  1. Department of Hepato-Biliary-Pancreatic Medicine, Cancer Institute Hospital of Japanese Foundation for Cancer Research, 3-8-31 Ariake, Koto-Ku, Tokyo, 135-8550, Japan

    Takeshi Okamoto, Takashi Sasaki, Masato Matsuyama, Masato Ozaka & Naoki Sasahira

  2. Department of Hepato-Biliary-Pancreatic Surgery, Cancer Institute Hospital of Japanese Foundation for Cancer Research, Koto-Ku, AriakeTokyo, 135-8550, Japan

    Yu Takahashi

  3. Department of Pathology, Cancer Institute Hospital of Japanese Foundation for Cancer Research, 3-8-31 Ariake, Koto-Ku, Tokyo, 135-8550, Japan

    Manabu Takamatsu & Hiroaki Kanda

  4. Department of Diagnostic Imaging, Diagnostic Imaging Center, Cancer Institute Hospital of Japanese Foundation for Cancer Research, 3-8-31 Ariake, Koto-Ku, Tokyo, 135-8550, Japan

    Makiko Hiratsuka

Authors
  1. Takeshi Okamoto
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  2. Takashi Sasaki
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  3. Yu Takahashi
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TO wrote the manuscript. TS, YT, MT, HK, MH, MM, MO, and NS critically reviewed the manuscript. All authors approved the final manuscript.

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Correspondence to Takashi Sasaki.

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Okamoto, T., Sasaki, T., Takahashi, Y. et al. Perivascular epithelioid cell tumor (PEComa) of the cystic duct. Clin J Gastroenterol 16, 87–95 (2023). https://doi.org/10.1007/s12328-022-01730-5

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  • DOI: https://doi.org/10.1007/s12328-022-01730-5

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