基于网络患者自助群的肾上腺皮质癌现况调查

Status survey of adrenocortical carcinoma based on internet patients support group

目的对我国肾上腺皮质癌(ACC)患者进行现况调查,为该病的临床诊疗提供参考。方法以问卷调查的形式对来自肾上腺皮质肿瘤患者群、2010-2019年明确诊断为ACC的患者进行信息采集,收集患者的性别、年龄、临床表现、病程、手术方式、辅助治疗手段、疗效等信息进行回顾性分析。结果最终纳入ACC患者156例,男女比例为1:1.33,40~60岁患者占比最高。除1例为双侧肾上腺受累外,其余均为单侧肾上腺占位,左侧多于右侧。81例(51.9%)存在肾上腺皮质激素分泌过多,其中33例(21.2%)存在满月脸、水牛背、向心性肥胖、肌无力等皮质醇增多表现,12例出现包括皮质醇在内的两种或两种以上肾上腺皮质激素自主分泌过度。147例进行了手术治疗,其中行开腹手术者占比最高(86例,55.1%),其次为腹腔镜手术(51例,32.7%)。最常见远处转移部位为肝脏(50例,32.1%)、肺(46例,29.5%)及腹膜后淋巴结(34例,21.8%)。124例(79.5%)应用米托坦进行辅助治疗,其中83例(66.9%)因术后复发转移而开始用药。米托坦中位稳定剂量为2.0 g,58例(64.4%)用药后达到部分或完全应答,24例(26.7%)病情稳定,8例(8.9%)出现疾病进展。将其他辅助治疗联合应用情况纳入分析后显示,单用米托坦、米托坦联合化疗、米托坦联合其他治疗三组间应答情况差异无统计学意义(P>0.05)。结论ACC患者中超过50%为功能性占位,皮质醇分泌过度者比例最高。部分患者易出现腹膜后淋巴结转移,因此根治术中应常规行淋巴结清扫。米托坦作为重要的辅助治疗手段,应答率较高,可与化疗等辅助治疗方式联合应用。

Objective To investigate the current situation of patients with adrenocortical carcinoma(ACC)in China,for providing references to clinical diagnosis and treatment of ACC.Methods The information was collected of patients(from adrenocortical tumor patient group)definitely diagnosed as ACC from 2010 to 2019 by questionnaire,including gender,age,clinical manifestations,course of disease,surgical and adjuvant treatment methods and curative effects,and retrospective analysis was conducted.Results A total of 156 ACC patients were included with a male to female ratio of 1:1.33.Patients aged 40-60 years accounted for the highest proportion.Except one patient with bilateral adrenal tumors,the others were unilateral involved,and the proportion of left adrenal tumor was slightly higher than that of the right side.Eighty-one(51.9%)patients had adrenocortical hormone hypersecretion,33 patients(21.2%)of them had manifestations of hypercortisolism such as full moon face,buffalo back,centripetal obesity,and muscle weakness,and 12 patients(7.7%)had spontaneous hypersecretion of two or more adrenal hormones,including cortisol.One hundred and forty-seven patients underwent surgery,of which open surgery accounted for the highest proportion(n=86,55.1%),followed by laparoscopic surgery(n=51,32.7%).The most common distant metastasis sites were liver(n=50,32.1%),lung(n=46,29.5%)and retroperitoneal lymph nodes(n=34,21.8%).79.5%of the studied population(n=124)used mitotane for adjuvant treatment,and 66.9%(n=83)of the patients started medication due to postoperative recurrence and metastasis.The median stable dosage of mitotane was 2.0 g.64.4%(n=58)of the patients could achieve partial or complete response,26.7%(n=24)of the patients were in stable condition,and the remaining 8.9%(n=8)had disease progression.After the combined application of other adjuvant treatments was included in the analysis,no significant difference was observed in the response among the three groups of mitotane alone,mitotane combined with chemotherapy,and mitotane combined with other treatments(P>0.05).Conclusions Functional occupancy accounted for more than 50%in ACC patients,and the proportion of cortisol hypersecretion is the highest.Some patients are prone to retroperitoneal lymph node metastasis,therefore,lymph node dissection should be performed routinely in radical operations.As an essential adjuvant therapy,mitotane possesses high response rate when used alone or in conjunction with chemotherapy or other adjuvant medicines.