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Clinicopathologic features of pleomorphic hyalinizing angiectatic tumor of soft parts

Chinese Medical Journal 2007年第10期120卷 876-881页

作者： KE Qi Erbolat ZHANG Hong-ying BU Hong LI Sheng SHI Dan-ni YANG Guang-hua CHEN Hui-jiao WEI Bing Department of Pathology West China Hospital Sichuan University Chengdu 610041 China Department of Pathology Kuitun Hospital Yili 833200 Xinjiang China Department of Pathology Langzhong Hospital Langzhong 637400 China Department of Pathology Luzhou Hospital Luzhou 646000 China

Background pleomorphic hyalinizing angiectatic tumor （PHAT） of soft parts is a rare soft tissue tumor, which is generally considered low-grade. To distinguish the tumor from other soft tissue lesions, we analyzed the clinicopathologic and ultrastructural features, immunophenotypes, and flow cytometric DNA ploidy of PHAT in 9 cases. Methods PHAT specimens were collected from 9 patients with PHAT from 1990 to 2004. Each specimen was cut into pieces and stained with hematoxylin-eosin, phosphotungstic acid-hematoxylin, Prussian blue, and Masson trichrome, respectively. Immunohistochemical stains for vimentin, S-100 protein, CD34, CD31, CD99, VEGF, desmin, CDl17, α-SMA, and MIB-1 were performed with the Envision system. Flow cytometry was used in four specimens, two of which were observed by electron microscopy. Results In the 9 cases, the PHAT occurred at the lower extremity in 2 patients, inguinal in 2, waist in 1, forearm in 1, buttock in 1, foot in 1, and the chest wall in 1. All the lesions presented in the superficial subcutaneous tissues. Follow-up data were available in 7 of the patients, among whom 2 （28.6%） had recurrence after primary therapy. Microscopically, typical PHAT was characterized by sheet-like proliferation of spindle or pleomorphic cells and clusters of thin-walled hyalinized cstatic vessels. In some areas of the tumor, hemosiderin-laden spindle cells, numerous small single vessels, and myxoid extracellular matrix could be identified, indicating an ＂atypical PHAT＂. Mitotic figures were rare in all the cases In 5 of the 9 patients （55.6%）, the tumor was typical PHAT; and in the other 4 （44.4%）, typical and atypical PHAT coexisted. Immunohistochemically, the neoplastic cells were positive for vimentin, CD34, CD99, and VEGF, but negative for S-100 protein, desmin, SMA, and CD31. In all the cases, the MIB-1 proliferative activity of the neoplastic cells was lower than 2%. Ultrastructural analysis did not reveal any evidence of specific differentiat

关键词： pleomorphic hyalinizing angiectatic tumor soft tissue neoplasms

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