Primary angiosarcoma arising in an angiomyolipoma of the kidney: case report and literature review
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CASE REPORT
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Primary angiosarcoma arising in an angiomyolipoma of the kidney: case report and literature review Hongwei Guan, Lizhi Zhang, Qiuping Zhang, Wenjing Qi, Suling Xie, Jinping Hou and Huali Wang*
Abstract Background: Primary angiosarcoma of the kidney is a rare and aggressive malignant tumor presenting with a recognizable vascular differentiation. Its prognosis is fatal and the pathogenesis remains unclear. Renal angiomyolipoma is a relatively infrequent renal cortical neoplasm and is composed of variable proportions of adipose tissue, spindle cells, epithelioid smooth muscle cells and abnormal thick-walled blood vessels. Case presentation: Here, we reported a case in which a 64-year-old woman presenting with the chief complaint of a progressively enlarged mass in the left abdomen. Abdominal computed tomography confirmed presence of a tumor measuring 18 cm × 11 cm in the left posterior renal fascia. Microscopic examination and immunohistochemical staining confirmed co-existence of angiomyolipoma and angiosarcoma. Furthermore, the two components interspersed with each other and there were transitional zones between them. Conclusions: In this case, we described for the first time a primary renal angiosarcoma possibly arising in a pre-existing angiomyolipoma of the kidney. Keywords: Primary angiosarcoma, Angiomyolipoma, Kidney, Pathology
Background Angiosarcoma (AS) is a rare and aggressive malignant tumor presenting with a recognizable vascular differentiation. It occurs mainly in the adulthood and elderly, with occasional cases reported in children [1]. Angiosarcoma is usually located in the skin, superficial soft tissue or other organs, such as bone, breast or liver [2]. Primary renal angiosarcoma is extremely rare and its prognosis is poor due to the rapid local recurrence and bloodstream dissemination [3]. Renal angiomyolipoma (AML) is the most common mesenchymal tumor of the kidney and it was first reported by Morgan in 1951 [4]. The World Health Organization (WHO) defines renal angiomyolipoma as a benign mesenchymal tumor composed of variable proportions of adipose tissue, spindle cells, epithelioid smooth muscle cells and abnormal thick-walled blood vessels [5]. It belongs to a family of lesions called perivascular * Correspondence: [email protected] Department of Pathology, Dalian Medical University First Hospital, 222 Zhongshan Road, Xigang District, Dalian 116011, Liaoning, China
epithelioid cell tumors (PEComas), which are characterized by proliferation of perivascular epithelioid cells [5]. They are not always located in the kidney and also can be found in uterus, liver, fallopian tubes and spleen [6]. Here we described a case of primary renal angiosarcoma concomitant with an AML in a 64-year-old woman. To our best knowledge, this is the first report of a renal angiosarcoma arising in an AML background.
Case presentation A 64-year-old woman presented to our hospital with the chief complaint of a progressively enlarged mass in the left abdomen accompanied with flank pain, fatigue
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