混合性嗜铬细胞瘤病例报道并文献复习

Composite pheochromocytoma:a case report and review of the literature

目的探讨肾上腺混合性嗜铬细胞瘤的临床和病理学特征,了解病理特点、鉴别诊断及预后情况,以提高认识。方法分析1例肾上腺混合性嗜铬细胞瘤的临床资料,电话随诊患者;HE染色、光镜下观察其组织学表现,并进行免疫组化染色。结果光镜观察,肿瘤中可见两种不同类型的细胞区域:一种为瘤细胞排列成不规则巢团状,以小巢团为主,核圆形或卵圆形,胞浆细颗粒状、嗜碱性或者双嗜性,分裂象罕见;一种为束状及编织状排列的施万细胞背景下,散在及聚集分布怪异、多核性神经节细胞,免疫组化显示前一区域瘤细胞CgA、Syn强阳性,后一区域S-100弥漫强阳性,其间神经节细胞NF强阳性。结论肾上腺混合性嗜铬细胞瘤临床罕见,临床表现不一,病理是目前唯一准确诊断手段,手术切除是治疗的最佳选择,术后随访是必要的。

Objective By investigating the clinical and pathological features of composite pheochromocytoma of the adrenal gland（ pheo-chromocytoma-ganglioneuroma）,to understand the pathological characteristics,differential diagnosis and prognosis,and to improve the under-standing to this disease. Methods one case of composite pheochromocytoma of adrenal gland was analyzed on clinical materials and follow-up data was obtained by telephone. Adopting HE staining and immunohistochemical staining,the histological features and immunohistochemical ex-pression of the tumor were analyzed. Results Microscopically,two different types of cells could be seen：one type of tumor arranged in irregular nests,maily in small nests with round or oval nuclei,fine granular and basophilic or amphophilic cytoplasm,mitoses were rare;the other type area was scattered and aggregated distributed and strange nuclear ganglion cells,in the background of Schwann cells which aligned bundles and interwo-ven. The tumor cells of the former areas were strongly positive for CgA and Syn,and scattered spindle cells were positive for S-100. As to the lat-ter,tumor cells were strongly positive for NF. Conclusion Composite pheochromocytoma of adrenal gland is rare. It has diversified clinical mani-festations. Biopsy is the only accurate diagnostic method. Surgical resection is the best choice for treatment,postoperative follow-up is necessary.