Renal angiomyolipoma with malignant transformation, simultaneous occurrence with malignity and other complex clinical situations |
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Authors: | Osman Inci Mustafa Kaplan Ömer Yalcin Irfan Huseyin Atakan Huseyin Kubat |
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Institution: | (1) Department of Urology, Faculty of Medicine, Trakya University, Avrupa Kent Yapi Kooperatifi, Iskenderkoy Mevkii, B7-4, 22030 Edirne, Turkey;(2) Department of Pathology Faculty of Medicine, Trakya University, Edirne, Turkey |
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Abstract: | Renal angiomyolipoma is a benign kidney tumor, which is characterized by the presence of mature or immature fat tissue, thick-walled
blood vessels, and smooth muscles. However, there is a rare possibility of transformation to a malignancy. This transformation
could be toward sarcoma. And also angiomyolipoma could be associated with renal adenocarcinoma in the same kidney. The aim
of this study is to show the association of angiomyolipoma with complex clinical situations such as malign transformation,
simultaneous occurrence with adenocarcinoma, bilateral tumors with tuberous sclerosis or Wunderlich Syndrome. Findings of
clinical presentation, pathological evaluation, urography, ultrasonography, and computerized tomography of 19 patients who
were diagnosed angiomyolipoma in our clinic during 1994–2003 were examined retrospectively. Our records indicated that radical
nephrectomy was performed in three cases of angiomyolipoma in which the differential diagnosis from adenocarcinoma could not
be made and in another case of angiomyolipoma, which was associated with adenocarcinoma. Simple nephrectomy was performed
in four symptomatic angiomyolipoma cases with tumor size larger than 4 cm and partial nephrectomy in another case. In one
case, renal angiomyolipoma was associated with adenocarcinoma in the same kidney. In one case, post-operative recurrence of
angiomyolipoma developed 7 months after nephrectomy. This patient was diagnosed low-grade leiomyosarcoma. Angiomyolipoma is
regarded as a benign tumor of the kidney; however, it may also show aggressive behaviors and rarely transform to sarcoma.
It may also exist in the same kidney along with adenocarcinoma or sarcoma. |
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Keywords: | Malignancy Renal angiomyolipoma Sarcoma Tuberous sclerosis Tumor |
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