肾上腺皮质癌皮肤转移1例国内首报

Cutaneous Metastasis from Adrenocortical Carcinoma: the First Case Reported in China

目的探讨肾上腺皮质癌(ACC)皮肤转移的临床表现、病理学特点及诊断,提高对本病的认识。方法分析1例ACC皮肤转移的临床表现、组织病理及免疫组织化学,复习相关文献并进行讨论。结果患者右臀部肿物进行性增大伴轻度疼痛1月余。ACC行腹腔镜下左肾上腺区肿瘤切除术2年。皮肤科情况:右臀部为一直径约5cm近圆形暗红色肿块,局部皮温略高,质地硬,境界较清楚,压痛明显。肿块组织病理示:真皮内可见境界清楚的肿瘤细胞团块,细胞核大,圆形或椭圆形,呈不典型性,有核丝分裂相,胞浆丰富呈嗜酸性或淡染;免疫组织化学染色示:肿瘤细胞Synaptophysin,Vimentin强阳性;Calretinin,Inhibin阳性;Chromogranin A,SMA,CD10,CK,S100,HMB45,Melan A,Desmin,CEA均阴性。结合免疫组织化学和病史,符合ACC皮肤转移。结论 ACC皮肤转移国内尚未见报道,国外罕有报道。组织病理及免疫组织化学对本病的诊断及鉴别诊断有重要作用。

Objective To investigate the clinical manifestations, pathological features and diagnosis of cutaneous me- tastasis from adrenocortical carcinoma （ACC） , and to raise awareness of the disease. Methods A case of cutaneous meta ＇asis from ACC was reported. The clinical data, histopathological and immunohistoehemical features of this case were analyzed. The relevant literatures were reviewed while the discussions were con- ducted. Results The patient was introduced to our department because of the more than 1-month history of tumor with progressive enlargement on right buttock accompanied with light pain. The patient received tumor resection of left adrenal gland assisted by laparoscopy due to ACC 2 years ago. Physical examination showed a near round ,5cm in diameter,dark red tumor which was well demarcated and hard accompanied with slightly high local skin temperature and obvious tenderness. The histopathology of the tumor showed that there were well- demarcated tumor cell masses in the dermis, in which the nuclei were large, round or oval, atypical with nuclear mitotic phase while the cytoplasm were rich presented with acidophilia or light color. The immunohistoehemistry staining showed that the expressions of Synaptophysin and Vimentin were strong positive, those of Calretinin and Inhibin were positive, and those of Chromogranin A, SMA, CD10, CK, S100, HMB45, Melan A, Desmin and CEA were negative in the tumor cells. According to the immuno- histochemistry staining and medical history,the patient was diagnosed as cutaneous metastasis from ACC. Conclusion This is the first case of cutaneous metastasis from ACC reported in China while the disease is rarely reported abroad. Histopathological and immunohistochemical features play important roles in the diagnosis and differential diagnosis of the disease.