Kuo Fang-Ying, Lin Hsin-Ching, Eng Hock-Liew, Huang Chao-Cheng
Department of Pathology, Chang Gung Memorial Hospital, Kaohsiung Medical Center, No. 123, Ta-Pei Road, Niao-sung Hsiang, Kaohsiung Hsien, 833, Taiwan.
Head Neck. 2005 Feb;27(2):124-9. doi: 10.1002/hed.20122.
Sinonasal hemangiopericytoma-like tumor (SHPCL) is an uncommon vascular tumor that is sometimes difficult to diagnose.
We report five cases arising from the nasal cavity. The tumors were subdivided into two types, prototype and soft tissue type, by the difference in histologic features.
The prototype, made up of three tumors, is characterized by uniform, spindle-shaped myoid cells that have eosinophilic cytoplasm and uniform nuclei and lay down in an orderly fashion. The soft tissue type, made up of two tumors, is similar to the hemangiopericytoma of soft tissue. Immunohistochemically, the tumors were positive for vimentin (100%), smooth muscle actin (80%), muscle-specific actin (100%), fibroblast growth factor-2 (100%), and vascular endothelial growth factor (100%). All were negative for cytokeratin, desmin, CD34, and factor VIII-related antigen. These histopathologic features suggest myoid differentiation and angiogenic activity of the tumors. All the tumors were treated with wide surgical excision. Only one case of soft tissue-type tumor with active mitoses received additional postoperative radiotherapy. No recurrence was found during 13 to 120 months of follow-up.
Our data suggest that SHPCL with convincing myoid differentiation may have a more favorable clinical course than its soft tissue counterpart.
鼻窦血管外皮细胞瘤样肿瘤（SHPCL）是一种罕见的血管肿瘤，有时难以诊断。
我们报告了5例起源于鼻腔的病例。根据组织学特征的差异，将肿瘤分为原型和软组织型两种类型。
原型由3个肿瘤组成，其特征是由均匀的梭形肌样细胞构成，这些细胞具有嗜酸性细胞质和均匀的细胞核，并呈有序排列。软组织型由2个肿瘤组成，类似于软组织血管外皮细胞瘤。免疫组织化学检查显示，肿瘤波形蛋白（100%）、平滑肌肌动蛋白（80%）、肌肉特异性肌动蛋白（100%）、成纤维细胞生长因子-2（100%）及血管内皮生长因子（100%）均呈阳性。细胞角蛋白、结蛋白、CD34及因子VIII相关抗原均为阴性。这些组织病理学特征提示肿瘤具有肌样分化和血管生成活性。所有肿瘤均采用广泛手术切除治疗。仅1例有活跃核分裂的软组织型肿瘤术后接受了辅助放疗。随访13至120个月，均未发现复发。
我们的数据表明，具有令人信服的肌样分化的SHPCL可能比其软组织对应物具有更良好的临床病程。
