鼻腔鼻窦型血管外皮瘤临床病理分析

Sinonasal-type hemangiopericytoma: a clinicopathologic analysis of 6 cases

目的探讨鼻腔鼻窦型血管外皮瘤的临床病理学特征及诊断和鉴别诊断及其组织发生。方法结合其临床和影像学特点，应用HE、嗜银网状纤维染色、免疫组织化学和电镜对6例鼻腔鼻窦型血管外皮瘤的病理改变进行了分析，并对其中5例进行了随访。结果男性4例，女性2例，年龄56—71岁，平均60．5岁；肿瘤均位于右鼻腔，分别为鼻中隔1例、中鼻道1例、右总道1例、鼻腔＋筛窦2例、右眼球＋筛窦＋左右额窦1例，临床上表现为鼻塞、鼻出血、鼻腔分泌物增多及眼球胀痛、视力减退、流泪、头痛等症状。巨检：病变呈息肉样2例、呈棕色光滑肿块3例、呈灰白碎组织1例。镜下观察呈弥漫分布的短梭形细胞，规则，核分裂象少见，胞质透明或嗜酸性，血管丰富，薄壁，并可见管壁玻璃样变，鹿角状血管少见，瘤细胞多以单相排列，也可呈束状、席纹状、环状及编织状。免疫组织化学检测显示瘤细胞波形蛋白6／6阳性、平滑肌肌动蛋白5／6阳性和CD34 3／6阳性，超微结构显示瘤细胞胞质内有肌动蛋白丝，细胞间有未发育完全的连接结构。随访5例，2例复发。结论鼻腔鼻窦型血管外皮瘤是一种真正起源自血管外皮细胞，具有特殊临床病理特征、少见的中间型或低度恶性肿瘤，需结合肿瘤的组织形态和免疫组织化学特点做出诊断和鉴别诊断。

Objective To study the clinicopathologic features, histologic diagnosis and differential diagnosis of sinonasal-type of hemangiopericytoma （SNTHPC）. Methods The clinical, radiographic and pathologic findings of 6 cases of SNTHPC were analyzed. Immunohistochemistry and electron microscopy were performed on selected examples. Results Amongst the 6 patients studied, 4 were males and 2 were females. The age of patients ranged from 56 to 71 years （mean =60.5 years old）. The commonest clinical presentation was nasal obstruction and/or epistaxis. Other symptoms could include increased nasal secretion, eyeball pain, decreased visual acuity, increased tear secretion and headache. The tumor involved nasal cavity and/or paranasal sinuses. Gross examination showed polypoid tumor masses, brownish fleshy tissue or whitish tumor tissue fragments, Histologically, the tumor showed a mixture of diffuse, fascicular, storiform, reticulated and whorled growth patterns. The tumor cells were spindle-shaped and possessed clear to eosinophilic cytoplasm. Mitotic figures were rarely seen. The intervening vasculature was characteristically thin-walled, with focal hyalinization changes and rarely the staghorn pattern. Immunohistochemical study showed that the tumor cells expressed vimentin （6/6）, smooth muscle actin （5/6） and CD34 （3/6）. Electron microscopy demonstrated the presence of intracytoplasmic myofilaments. The tumor cells were linked together by primitive cell junctions. In general, the histologic diagnosis of SNTHPC was difficult, and only 1 case had the correct initial pathologic diagnosis made. Follow-up data were available in 5 patients and 2 of them had local recurrences. Conclusions SNTHPC is a low to intermediate grade soft tissue tumor with pericytos differentiation. Correct diagnosis relies on detailed pathologic assessment and application of ancillary investigations.