Journal of the Formosan Medical Association
Volume 106, Issue 2, Supplement , Pages S51-S54, 2007

Malignant Epithelioid Angiomyolipoma of the Kidney

  • Kuo-How Huang

      Affiliations

    • Department of Urology, National Taiwan University Hospital, Taipei, Taiwan
    • ,
  • Chao-Yuan Huang

      Affiliations

    • Department of Urology, National Taiwan University Hospital, Taipei, Taiwan
    • ,
  • Shiu-Dong Chung

      Affiliations

    • Department of Urology, National Taiwan University Hospital, Taipei, Taiwan
    • ,
  • Yeong-Shiau Pu

      Affiliations

    • Department of Urology, National Taiwan University Hospital, Taipei, Taiwan
    • ,
  • Chia-Tung Shun

      Affiliations

    • Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan
    • Corresponding Author InformationCorrespondence to: Dr Chia-Tung Shun, Department of Pathology, National Taiwan University Hospital, 7 Chung-Shan South Road, Taipei 100, Taiwan
    • ,
  • Jun Chen

      Affiliations

    • Department of Urology, National Taiwan University Hospital, Taipei, Taiwan

Received 26 October 2005; received in revised form 25 November 2005; accepted 7 March 2006.

Article Outline

Angiomyolipoma (AML) is a common benign renal tumor composed of thick-walled blood vessels, smooth muscle, and adipose tissue, but the malignant epithelioid variant is extremely rare. A 78-year-old woman presented with fever and left flank pain for 3 days. Computed tomography showed a heterogeneously enhanced mass without fat density in the left kidney. Radical nephrectomy was performed and pathology showed malignant epithelioid AML with regional lymph node metastases. The tumor cells were positive for human melanosome-associated protein (HMB-45) on immunohistochemical staining. The patient died of disseminated metastases (lungs and bones) 5 months postoperatively. Epithelioid AML is a potentially aggressive tumor. The prognosis is poor in metastatic disease. HMB-45 immunoreactivity is a useful marker to make diagnosis. [J Formos Med Assoc 2007;106(2 Suppl):S51-S54]

Key Words:  human melanosome-associated protein , malignant epithelioid angiomyolipoma

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PII: S0929-6646(09)60353-3

doi:10.1016/S0929-6646(09)60353-3

Journal of the Formosan Medical Association
Volume 106, Issue 2, Supplement , Pages S51-S54, 2007

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