摘要目的 探讨Castleman病的临床特点及外科诊疗.方法 回顾性分析19例Castleman病患者临床资料,其中男8例,女11例,发病年龄7～74岁,中位年龄40岁.肿瘤分布:颈部12例,颈部合并纵隔2例,腋下2例,腹膜后2例,腹腔1例.结果 19例患者中18例行手术切除,术后病理明确为Castleman病,按Frizzera分类标准分为局灶型16例(其中浆细胞型-霍奇金病1例,透明血管型11例,混合型4例),多中心型3例(其中浆细胞型2例,混合型1例).术后所有患者均长期生存,其中浆细胞型1例术后2年复发,行二次手术+CVP方案(环磷酰胺+长春新碱+泼尼松)化疗6周期,现存活5年;浆细胞型转化为霍奇金病1例,初次手术6个月后复发,行二次手术切除+ABVD方案(阿霉素+博来霉素+长春碱+达卡巴嗪)化疗6周期,现无病生存15个月.腹腔浆细胞型1例给予CHOP方案(环磷酰胺+长春新碱+阿霉素+泼尼松)化疗1周期后肠梗阻解除出院.结论 Castleman病临床多表现为无痛性肿物渐进性增大,局灶型Castleman病首选手术治疗,预后良好;而多中心型和浆细胞型Castleman病易于复发和转化为淋巴瘤,其治疗应选择以手术切除+术后辅助化疗为主的综合治疗,临床预后不良.

abstractsObjective To explore the clinical features and surgical treatment of tumors associated with Castleman's disease(CD).Methods The clinical profiles of 19 patients with neck giant lymph node hyperplasia were analyzed retrospectively.There were 8 males and 11 females with a median age of 40 years old(range:7-74).The tumor locations were neck(n=12),neck & mediastinal cavity(n =2),axillary fossa(n=2),retroperitoneal area(n=2)and abdominal cavity(n=1).Results Eighteen of them underwent surgical resection of tumor or lymph nodes. All were diagnosed as CD by pathological examinations.There were 16 localized CD(LCD)including hyaline vascular type(HV type,n=11),mixed type(mix type,n =4)and plasma cell type-Hodgkin's disease(n=1).Among 3 multicentric CD (MCD),there were 2 case of plasma cell type(PC type)and Ⅰ case of mixed type(mix type).Long-term survival was achieved in 19 cases among which 1 case of plasma cell type MCD survived for 5 years and underwent a second operation and postoperative chemotherapy of CVP(cyclophosphamide,vincristine & prednisone)regimen for 3 cycles due to recurrence in 2 years and 1 case of plasma cell type LCD-Hodgkin's disease survived for 15 months and underwent a second operation and postoperative chemotherapy of ABVD (adriamycin,bleomycin,vinblastine & dacarbazine)regimen for 6 cycles due to recurrence in 6 months.One case of plasma cell type MCD in abdominal cavity on chemotherapy of CHOP(cyclophosphamide,hydroxydaunorubicin,vincristine & prednisone)regimen for 6 cycles was discharged after a successful management of intestinal obstruction. ConclusionsThe major clinical symptom of CD is a gradually enlarging painless mass.Surgical resection of tumor remains the first-line treatment for localized CD and the prognosis is excellent.Multicentric and plasma cell type CDs are prone to recurrence and transformation to lymphoma.And their first-line therapeutic should encompass multi-modality regimens of surgery and adjuvant chemotherapy.However,the clinical prognosis is still poor.