Sinonasal-type hemangiopericytoma - A clinicopathologic and immunophenotypic analysis of 104 cases showing perivascular myoid differentiation
Sinonasal-type hemangiopericytoma - A clinicopathologic and immunophenotypic analysis of 104 cases showing perivascular myoid differentiation
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DOI：
10.1097/00000478-200306000-00004
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发表时间：
2003-06-01
影响因子：
5.6
通讯作者：
Wenig, BM
中科院分区：
文献类型：
--
作者：
Thompson, LDR;Miettinen, M;Wenig, BM
鼻窦型血管外皮细胞瘤是一种不常见的上呼吸消化道肿瘤，其细胞分化不定。我们报告104例鼻窦型血管外皮细胞瘤诊断之间的1970年和1995年的档案武装部队病理研究所。年龄5 ~ 86岁（平均62.6岁）。最常见的临床表现是气道阻塞（n = 57）和/或鼻出血（n = 54），症状平均持续10个月。肿瘤仅累及鼻腔（n = 47）或累及副鼻窦（n = 26），均为息肉样。平均3.1厘米组织学上，肿瘤呈粘膜下和无包膜，呈弥漫性生长，呈束状（n = 37）至实性（n = 50）至局灶性螺旋状（n = 7）模式。肿瘤细胞外观均匀，多形性最少，细胞核呈梭形（n = 82）至圆形/卵圆形（n = 18），染色质呈囊泡状至深染，细胞质嗜酸性至嗜两性至透明，细胞边界不清。在少数病例中发现了多核（肿瘤）巨细胞（5）。核分裂像不明显，无坏死。肿瘤血管丰富，包括鹿角状血管，特征性地具有显著的血管周围玻璃样变（n = 92）。在大多数病例（n = 87）中观察到相关炎性细胞浸润，包括肥大细胞和嗜酸性粒细胞。免疫组化谱包括与波形蛋白（98%）、平滑肌肌动蛋白（92%）、肌肉特异性肌动蛋白（77%）、因子XIIIa（78%）和层粘连蛋白（52%）的反应性。手术是所有患者的治疗选择：4名患者接受了连续放疗。18例患者在诊断后1-12年内复发。97例患者存活（n = 51，平均16.5年）或死亡（n = 46，平均9.6年），但无疾病。4名患者在末次随访时患病：3名患者因疾病死亡（平均3.6年），1名患者因疾病存活（28.3年）。复发性肿瘤（17.8%）可通过额外手术治疗。大多数鼻窦型血管外皮细胞瘤表现为良性，单纯手术后的长期预后良好（原始5年生存率为88%）。鼻腔鼻窦型血管外皮细胞瘤在光镜下具有特征性的外观，其免疫表型特征与血管球瘤相似。
Sinonasal-type hemangiopericytoma is an uncommon upper aerodigestive tract tumor of uncertain cellular differentiation. We report 104 cases of sinonasal-type hemangiopericytoma diagnosed between 1970 and 1995 from the files of the Armed Forces Institute of Pathology. There were 57 females and 47 males ranging in age from 5 to 86 years (mean 62.6 years). The most common clinical presentation was airway obstruction (n = 57) and/or epistaxis (n = 54), with symptoms averaging 10 months in duration. The tumors involved the nasal cavity alone (n = 47) or also a paranasal sinus (n = 26), were polypoid. and measured an average of 3.1 cm. Histologically, the tumors were submucosal and unencapsulated and showed a diffuse growth with fascicular (n = 37) to solid (n = 50) to focally Whorled (n = 7) patterns. The tumor cells were uniform in appearance With minimal pleomorphism and had spindle-shaped (n = 82) to round/oval (n = 18) nuclei with vesicular to hyperchromatic chromatin and eosinophilic to amphophilic to clear-appearing cytoplasm with indistinct cell borders. Multinucleated (tumor) giant cells were identified in a minority of cases to 5). Mitotic figures were inconspicuous and necrosis was absent. The tumors were richly vascularized, including staghorn-appearing vessels that characteristically had prominent perivascular hyalinization (n = 92). An associated inflammatory cell infiltrate that included mast cells and eosinophils was noted in the majority of cases (n = 87). The immunohistochemical profile included reactivity with vimentin (98%), smooth muscle actin (92%), muscle specific actin (77%), factor XIIIa (78%), and laminin (52%). Surgery was the treatment of choice for all of the patients: adjunctive radiotherapy was given to four patients. Recurrences developed in 18 patients within 1-12 years from diagnosis. Ninety-seven patients were either alive (n = 51, mean 16.5 years) or dead (n = 46, mean 9.6 years) but free of disease. Four patients had disease at the last follow-up: three died with disease (mean 3.6 years) and one patient is alive with disease (28.3 years). Recurrent tumor ( 17.8%) can be managed by additional surgery. The majority of sinonasal-type hemangiopericytomas behave in a benign manner with excellent long-term prognosis (88% raw 5-year survival) following surgery alone. Sinonasal-type hemangiopericytomas have a characteristic light microscopic appearance with an immunophenotypic profile resembling that of glomus tumors.