肾上腺节细胞神经瘤的临床病理学分析

The clinical and pathologic characteristics of adrenal ganglioneuroma

目的探讨肾上腺节细胞神经瘤的临床及病理学特点。方法收集经手术病理证实的13例肾上腺节细胞神经瘤患者临床资料进行分析。患者男5例,女8例。平均年龄38岁。肿瘤位于左侧7例,右侧5例,双侧1例。体检时B超偶然发现6例,上腹部不适或间歇性腹痛6例,高血压表现1例。血液生化检查未见特殊异常。影像学检查提示肾上腺实性占位。肿瘤最大直径平均6．7 cm。结果行开放手术治疗12例,腹腔镜手术治疗1例。13例手术顺利,无并发症发生。病理表现:肿瘤主要由成熟的神经轴突、神经束和纤维组织构成,其间散在成熟的节细胞、Schwann细胞。其中3例肿瘤部分区域细胞分化不成熟,有异形性。肿瘤伴囊性变2例,伴钙化1例。随访时间4～116个月,平均43个月,未见肿瘤局部复发和远处转移。结论肾上腺节细胞神经瘤是少见的良性肿瘤,具有独特的病理特点,手术治疗效果良好。

Objective To elucidate the clinical and pathologic characteristics of adrenal ganglioneuroma. Methods The data of 13 patients （5 men and 8 women） with adrenal ganglioneuroma diagnosed pathologically were reported and discussed with review of the relevant literatures. The patient mean age was 38 years. Seven patients had left adrenal ganglioneuroma;5 had the tumor on the right;and 1 ,on both sides. Six patients with asymptomatic mass were detected on routine B-ultrasound ,6 had upper abdominal discomfort or intermittent pain,and 1 had hypertension. The blood testing showed no abnormality. The adrenal imaging demonstrated solid space-occupying lesions. The mean greatest diameter of the tumors was 6.7 era. Twelve patients underwent open surgical excision of the adrenal tumors;and 1 underwent laparoscopic resection. Results The operations were completed successfully, All patients recovered soon without complications. Pathology showed that adrenal ganglioneuroma contained mature ganglion cells, Schwann cells, neurites and sustentacular fibers. The adrenal ganglioneuroma was partially composed of immature and atypical cells in 3 cases,had partially cystic degeneration in 2 cases,and had partially calcification in 1 case, The mean followup period was 43 months （range,4 - 116 months）. Until now no recurrence and metastasis of tumor were detected. Conclusions Adrenal ganglioneuroma is a rare benign tumor and has special pathologic features. The surgical resection has an excellent effect.