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Open Access Case Report

Clinical and pathological characteristics of giant cell angioblastoma: a case report

Rong-Jun Mao1, Zhi-Ming Jiang2, Hui-Zhen Zhang2, Xiong-Zeng Zhu3* and Qing-Ling Zhang45*

Author Affiliations

1 Department of Pathology, Foshan Hospital of Traditional Chinese Medicine, Guangzhou University of Chinese Medicine, Foshan, People’s Republic of China

2 Department of Pathology, Shanghai Sixth People’s Hospital Affiliated to Shanghai Jiaotong University, Shanghai, People’s Republic of China

3 Department of Pathology, Cancer Hospital, Fudan University, Shanghai, People’s Republic of China

4 Department of Pathology, Nanfang Hospital, Southern Medical University, Guangzhou, People’s Republic of China

5 Department of Pathology, College of Basic Medicine, Southern Medical University, Guangzhou, People’s Republic of China

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Diagnostic Pathology 2012, 7:113  doi:10.1186/1746-1596-7-113

Published: 29 August 2012

Abstract

Giant cell angioblastoma (GCAB) is an extremely rare soft tissue tumor of early childhood and only five cases have been described to date. As such the clinical, pathological, and prognostic features are poorly defined. We prensent here a new case of GCAB in bone of a child aged 4-years old. The lesion was composed of dense and loose cell regions. The dense regions were characterized by nodular, linear, and plexiform aggregates of oval- to spindle-shaped tumor cells around small vascular channels and interspersed with large mononuclear cells and multinucleate giant cells. The loose cell areas were characterized by distributed fibroblasts and abundant myxoid matrix, which diminished with patient age. Infiltrative growth was observed in some areas. Oval-to-spindle cells showed positivity for Vimentin, CD31 and CD34 staining, and partial positivity for smooth muscle actin. Mononuclear cells and multinucleate giant cells showed Vimentin and CD68 positivity. Seventeen months after thorough curettage of the lesion, a local recurrence was found. Based upon the clinical, histological and immunohistochemical findings, infiltrate condition, and prognosis, we classified GCAB into two subtypes. Type I does not infiltrate surrounding tissues and has good prognosis. Type II infiltrates the surrounding tissues, relapses earlier, and has worse prognosis. This report augments the limited GCAB literature to promote our understanding and guide therapy of this rare disease.

Virtual slides

The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/6699811297488137 webcite

Keywords:
Giant cell angioblastoma; Bone tumor; Rare tumor; Pediatric malignancies; Interferon-α