Primary angiosarcoma arising in an angiomyolipoma of the kidney: case report and literature review

Hongwei Guan,Hongwei Guan,Huali Wang,Jinping Hou,Qiuping Zhang,Qiuping Zhang,Suling Xie,Suling Xie,Huali Wang,Lizhi Zhang,Lizhi Zhang,Wenjing Qi,Wenjing Qi

doi:10.1186/s13000-018-0730-z

Abstract

BackgroundPrimary 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 presentationHere, 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.ConclusionsIn this case, we described for the first time a primary renal angiosarcoma possibly arising in a pre-existing angiomyolipoma of the kidney.

Highlights

BackgroundAngiosarcoma (AS) is a rare and aggressive malignant tumor presenting with a recognizable vascular differentiation
Primary angiosarcoma of the kidney is a rare and aggressive malignant tumor presenting with a recognizable vascular differentiation
In this case, we described for the first time a primary renal angiosarcoma possibly arising in a pre-existing angiomyolipoma of the kidney

Summary

Background

Angiosarcoma (AS) is a rare and aggressive malignant tumor presenting with a recognizable vascular differentiation. 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. The other component (including the areas adjacent to the renal cortex) was primarily composed of adipose tissue, smooth muscle cells and abnormal thick-walled blood vessels (Fig. 2f and g), indicating the feature of AML.

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Discussion

Conclusions