Castleman病的外科诊治

目的探讨Castleman病外科相关肿瘤的临床特点及外科治疗。方法分析北京协和医院1997-2004年20例Castleman病外科相关肿瘤患者的临床资料,其中男6例,女14例,年龄15~70岁,平均年龄39岁。肿瘤最小约2cm×3cm,最大约10cm×9cm。肿瘤分布:纵隔肿瘤6例,腹膜后2例,腹腔内2例,肾上腺区域1例,腋下、颈部、耳后等肿瘤6例,多中心肿瘤3例。结果20例患者中19例行手术切除肿瘤或行淋巴结活检。病理均提示为Castleman病,肿瘤类型多为巨大淋巴结增生,血管滤泡型和透明血管型。大多数肿瘤为良性病变。其中3例为副肿瘤性天疱疮(PNP)。结论Castleman病临床上少见,表现为不明原因的淋巴结肿大。肿块可发生在淋巴组织的任何部位,以纵隔、颈部、腋为好发部位,腹膜后肾上腺区域罕见。因此,在临床发现原因不明的肿块时,还要考虑其他少见的肿瘤。手术切除肿瘤为首选治疗。

Surgical diagnosis and treatment of Castleman's disease

OBJECTIVE: To investigate the clinical features and treatment of the surgical tumors associated with Castleman's disease. METHODS: The clinical data of 20 patients with Castleman's disease, 6 males and 14 females, aged 39 (15-70), with the tumor size ranging from 2 cm x 3 cm to 10 cm x 9 cm, and with the tumor located in the mediastinal cavity (n = 6), retroperitoneal area (n = 2), abdominal cavity (n = 1), adrenal area (n = 1), neck and armpit (n = 2), and multi-centers (n = 2), were analyzed. RESULTS: Nineteen of the 20 patients underwent surgical resection of the tumor or lymph node. All were diagnosed as Castleman's disease by pathological examination. Most of the tumor was benign. Three cases were diagnosed as with paraneoplastic pemphigus. CONCLUSION: Surgical resection of the tumor is the first choice for treatment of Castleman's disease.