骶骨神经源性肿瘤临床病理分析

目的:探讨骶骨神经源性肿瘤的临床病理学特征、诊断及鉴别诊断.方法:收集21例发生在骶骨的神经源性肿瘤,通过光镜观察及免疫组织化学分析其临床、影像学、病理学特征、免疫表型、鉴别诊断及手术预后.结果:21例中女15例,男6例,平均年龄44.9岁.临床上以骶尾部疼痛为主,影像学上表现为骶骨或骶骨及骶前肿块.神经鞘瘤17例,其中经典型神经鞘瘤8例,富于细胞神经鞘瘤9例.神经纤维瘤3例,节细胞神经瘤1例.累及骶骨的肿块,多数有不同程度的骨质破坏.免疫表型:神经鞘瘤均弥漫强阳性表达S-100蛋白,不表达NF.神经纤维瘤和节细胞神经瘤表达NF.富于细胞神经鞘瘤有4例为复发病例,平均复发时间6.5年.经典型神经鞘瘤和神经纤维瘤各有1例为复发病例.结论:骶骨神经源性肿瘤是少见肿瘤,以良性多见.各病理类型及亚型在形态学和生长方式上有一定的差异,故诊断时应明确病理类型及亚型,以供临床随访、治疗.

Clinicopathologic analysis of sacral neurogenic tumors

Objective: To investigate clinicopathologic features, diagnosis, differential diagnosis of sacral neurogenic tumors. Methods: Twenty one cases of sacral neurogenic tumors were collected. Microscopic examination and immunohistochemistry were performed for studying the clinical feature, radiologic appearance, pathologic characteristic, immunophenotyping, differential diagnosis and postoperative prognosis. Results: There were 15 females and 6 males, whose mean age was 44.9 years old. The majority of patients complained of pain in sacrococcygeal region. Radiographically, there was an endosacral or endosacral and presacral mass. Seventeen cases were schwannoma, including 8 cases of conventional schwannoma and 9 cases of cellular schwannoma. Three cases were neurofibroma. One case was ganglioneuroma. The destruction of bone were found in the cases of endosacral mass. Immunohistochemically, tumor cells of schwannoma were diffuse and strong positive for S-100 protein, negative for NF. Neurofibroma and ganglioneuroma cells were positive for NF, tumor recurrence of 4 cases occurred several years after initial surgical resection. The mean interval to recurrence was 6.5 years. Conventional schwannoma and neurofibroma each have 1 case for recurrent. Conclusion: Sacral neurogenic tumor is a rare tumor. Most of them are benign. There are many different pathologic features and growth pattern in pathological types and subtypes. So pathological diagnosis of the tumor should be noted for clinic follow-up and treatment.