Renal Epithelioid Angiomyolipoma with Malignant Behavior

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  • Ishikawa, Tetsuo
    The Department of Urology, Tokyo Women's Medical University Medical Center East
  • Ito, Fumio
    The Department of Urology, Tokyo Women's Medical University Medical Center East
  • Kanemitsu, Izumi
    The Department of Urology, Tokyo Women's Medical University Medical Center East
  • Suzuki, Koji
    The Department of Urology, Tokyo Women's Medical University Medical Center East
  • Nakazawa, Hayakazu
    The Department of Urology, Tokyo Women's Medical University Medical Center East
  • Fujibayashi, Mariko
    The Department of Surgical Pathology, Tokyo Women's Medical University Medical Center East

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Other Title
  • 悪性の経過をたどった類上皮型腎血管筋脂肪腫の1例
  • アクセイ ノ ケイカ オ タドッタ ルイ ジョウヒガタジンケッカンキン シボウ シュ ノ 1レイ

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Abstract

The patient was a 22-year-old woman with a history of tuberous sclerosis who complained of abdominal distention. Computed tomography showed bilateral renal masses with minimal fat tissue that were unlike classical angiomyolipoma (AML), as well as enlargement of the paraaortic lymph nodes, pleural effusion, and ascites, similar to other advanced malignant neoplasms such as renal cell carcinoma and renal sarcoma. We performed a left nephrectomy to relieve the symptoms. A pathological examination revealed that the tumor was composed of polygonal epithelioid cells positive for both αSMA and HMB-45, as well as spindle-shaped cells, while immunoreactivity proved the tumor to be an eAML, a subtype of AML with malignant potential. Although the patient was scheduled for postoperative adjuvant chemotherapy, her condition worsened and she died on postoperative day 24 before receiving chemotherapy. We discuss this case with reference to previous reports.

Journal

  • Hinyokika Kiyo

    Hinyokika Kiyo 58 (1), 21-24, 2012-01

    泌尿器科紀要刊行会

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