Perirenal Malignant Perivascular Epithelioid Cell Tumor Originating from Right Retroperitoneum : A Case Report

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  • Nakanishi, Shotaro
    The Department of Urology, Graduate School of Medicine, University of the Ryukyus
  • Miyazato, Minoru
    The Department of Urology, Graduate School of Medicine, University of the Ryukyus
  • Yonemori, Kan
    National Cancer Center Hospital, Mammary Gland and Oncology
  • Tamashiro, Tomoko
    The Department of Pathology and Oncology, Graduate School of Medicine, University of the Ryukyus
  • Yoshimi, Naoki
    The Department of Pathology and Oncology, Graduate School of Medicine, University of the Ryukyus
  • Saito, Seiichi
    The Department of Urology, Graduate School of Medicine, University of the Ryukyus

Bibliographic Information

Other Title
  • 右腎周囲から発生したMalignant PEComaの1例
  • ウジンシュウイ カラ ハッセイ シタ Malignant PEComa ノ 1レイ

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Abstract

A 51-year-old woman was admitted to our hospital for detailed examinations and medical treatment against the right retroperitoneum tumor and liver lesions. Computed tomography showed a 20 cm diameter enhanced mass in the right perirenal space and multiple liver metastases. Pathological examination by needle biopsy revealed a perivascular epithelioid cell tumor (PEComa). With weekly administration of a mTOR inhibitor, Temsirolimus, the tumor was reduced by about 20% one month later. The maximal response was stable disease due to RECIST v 1. 1. However, shortly after the minimal response, the disease showed progression. She died about five months after the start of molecular targeted therapy. In regard to the origin of PEComa, our report is the fifth in Japan as a case of retroperitoneal origin to the best of our knowledge.

Journal

  • Hinyokika Kiyo

    Hinyokika Kiyo 60 (12), 627-630, 2014-12

    泌尿器科紀要刊行会

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