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Rapidly Progressive Malignant Epithelioid Angiomyolipoma of the Kidney
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- Takumi Yamamoto
- From the Department of Urology and First Department of Pathology, Gunma University School of Medicine, Maebashi and Department of Urology, National Takasaki Hospital, Takasaki, Japan
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- Kazuto Ito
- From the Department of Urology and First Department of Pathology, Gunma University School of Medicine, Maebashi and Department of Urology, National Takasaki Hospital, Takasaki, Japan
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- Kazuhiro Suzuki
- From the Department of Urology and First Department of Pathology, Gunma University School of Medicine, Maebashi and Department of Urology, National Takasaki Hospital, Takasaki, Japan
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- Hidetosi Yamanaka
- From the Department of Urology and First Department of Pathology, Gunma University School of Medicine, Maebashi and Department of Urology, National Takasaki Hospital, Takasaki, Japan
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- Kazunori Ebihara
- From the Department of Urology and First Department of Pathology, Gunma University School of Medicine, Maebashi and Department of Urology, National Takasaki Hospital, Takasaki, Japan
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- Atsushi Sasaki
- From the Department of Urology and First Department of Pathology, Gunma University School of Medicine, Maebashi and Department of Urology, National Takasaki Hospital, Takasaki, Japan
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Description
In rare cases renal angiomyolipoma exhibits infiltrative growth, extensive destruction, local recurrence and metastasis. We report on a patient who had the worst prognosis among the known cases of angiomyolipoma. CASE REPORT A 47-year-old man without the tuberous sclerosis complex presented with acute upper abdominal pain. Computerized tomography of the abdomen demonstrated a 10 cm. tumor that enhanced unequally arising from the upper pole of the left kidney and multiple liver tumors with perihepatic hematoma (fig. 1). We clinically diagnosed this case as renal cell carcinoma of the left kidney with multiple liver metastases. We did not consider this patient a surgical candidate and, thus, used conservative treatment. Approximately 1 month later the patient presented with lumbago, and bone scintigraphy confirmed metastasis to the lumbar spine. Approximately 2 months after initial presentation lung metastasis was noted on thoracic x-ray. The patient’s general condition subsequently worsened, and he died approximately 3 months after initial presentation. At autopsy the tumor of the left kidney measured 20 10 10 cm. Dissection revealed most of the neoplasm to be expansive growth, while a few parts exhibited infiltrative growth into the renal parenchyma and adrenal gland. The tumor was gray with necrotic and hemorrhagic areas, and was firm in consistency. The weight of the tumor plus left kidney was 1,565 gm. Many grayish masses were found in the liver, and metastatic lesions were present in the lungs and at the lumbar spine, and involved lymph nodes at the hilum of the left kidney, the para-aorta and the hila of the lungs. Histopathological appearance of the left kidney tumor was epithelioid angiomyolipoma consisting of blood vessels, smooth muscle and adipose tissue, which are all characteristic of angiomyolipoma. However, the predominant cell type was epithelioid, and all metastatic lesions consisted of epithelioid cells (fig. 2, A and B). Immunohistochemical findings were positive for HMB-45, -smooth muscle actin and epithelial membrane antigen (fig. 2, C). Based on these findings, we diagnosed this case as malignant epithelioid angiomyolipoma of the left kidney.
Journal
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- Journal of Urology
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Journal of Urology 168 (1), 190-191, 2002-07
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