IJCEP Copyright © 2007-All rights reserved.
Int J Clin Exp Pathol 2013;6(9):1935-1941

Case Report
Composite hemangioendothelioma arising from the kidney: case report with review of
the literature

Jin Zhang, Bo Wu, Gui-Qian Zhou, Ru-Song Zhang, Xue Wei, Bo Yu, Zhen-Feng Lu, Heng-Hui Ma, Qun-Li Shi, Xiao-Jun Zhou

Department of Pathology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China; Department of Pathology, the second people’
s hospital of Chizhou, Chizhou, China. Equal contributors.

Received June 26, 2013; Accepted July 27, 2013; Epub August 15, 2013; Published September 1, 2013

Abstract: Reported herein is a medical curiosities vascular tumor primary arising from the kidney and exhibiting unique histopathological
features. A 32-year-old woman underwent a total nephrectomy of right kidney because of a mass localized in the inferior pole. Distinct from
other vascular lesions, on histology the tumor had a peculiar composite pattern, consisting of benign and malignant vascular components,
which were haphazardly intermixed without any definite margins. The malignant component was composed of epithelioid
hemangioendothelioma (45%) and angiosarcoma (50%) with moderate differentiation. Immunohistochemically, the oval to cuboidal to spindle
tumor cells expressed only endothelial markers (CD31, CD34 and factor VIII-related antigen). And the angiosarcomatous component was
characterized by the presence of a greater proliferation index Ki-67. Unlike other epithelial tumors, smooth muscle actin (SMA), cytokeratin, EMA
and S-100 were all negative in the epithelioid tumor cells. These findings led to the diagnosis of a low-grade vascular neoplasm with
morphological features consistent with so-called composite hemangioendothelioma (CHE). At 11 month follow up the patient was alive,
without evidence of tumor recurrence. CHE is an extremely rare vascular neoplasm, with borderline malignant potential, which mostly occurs in
distal extremity of the limbs at the cutaneous level and, only 30 cases have been previously described until now. To our knowledge, this is the
first report of CHE arising from the kidney and widens the spectrum of primary vascular tumors arising in the kidney. (IJCEP1306030).

Keywords: Composite hemangioendothelioma, hemangioendothelioma, rare tumors, kidney

Address correspondence to: Dr. Jin Zhang or Dr. Xiao-Jun Zhou, Department of Pathology, Nanjing Jinling Hospital, Nanjing University School
of Medicine, Nanjing 210002, P.R. China. E-mail: zhangjin20040311@163.com (JZ); zhouxj1@126.com (XJZ)