Hubertus J, Boxberger N, Redlich A, von Schweinitz D, Vorwerk P
Pediatric Surgery, Ludwig-Maximilians University, Munich, Germany.
Klin Padiatr. 2012 Apr;224(3):143-7. doi: 10.1055/s-0032-1304627. Epub 2012 Apr 13.
Adrenocortical carcinomas (ACCs) are a rare entity, with an incidence of 1.5 per million population per year. The prognosis of ACC is poor. Complete surgical resection is essential for a curative approach and significantly determines overall prognosis. Tumor resection is sophisticated and complicated by the vulnerability of the tumor and its invasive growth. Chemotherapy and Mitotane are additional therapeutic approaches that are combined with surgery in an interdisciplinary strategy. In this study, 59 patients between 2 months and 18 years of age with histologically verified ACC were analyzed retrospectively with respect to oncosurgical aspects. Patients were registered in the GPOH-MET 97 trial of the Society of Pediatric Oncology and Haematology. Preoperative management, factors influencing surgical severity, and operative complications were assessed.The gender ratio was 1:2 (m:f). A total of 58 patients showed increased hormonal activity and associated clinical signs of hormonal excess. Tumor volume was ≥ 300 mL in 25 patients. These patients showed an increased rate of operative complications and a poorer overall survival (OS) rate (p<0.01). A total of 14 patients showed metastatic spread, particularly to the lungs and lymph nodes. Biopsy of the tumor was performed in 12 patients. Tumor rupture occurred in 11 patients. Preoperative biopsy and/or experienced tumor rupture were associated with poorer OS rate. R2 resection only was achievable in 5 patients, and surgery was not feasible in 3 patients.In conclusion, since most of the pediatric ACC are hormone active and can be diagnosed clinically, the need of a tumor biopsy has to be discussed critically. Thorough pre- and perioperative management is essential for oncosurgical success.
肾上腺皮质癌（ACC）是一种罕见疾病，每年发病率为百万分之一。ACC的预后较差。完整的手术切除是治愈的关键，对总体预后有显著影响。肿瘤切除复杂，因其易损性和浸润性生长而变得棘手。化疗和米托坦是在多学科策略中与手术联合使用的其他治疗方法。本研究对59例年龄在2个月至18岁之间、经组织学证实为ACC的患者进行了肿瘤外科方面的回顾性分析。患者登记在小儿肿瘤学和血液学协会的GPOH-MET 97试验中。评估了术前管理、影响手术难度的因素和手术并发症。性别比为1:2（男:女）。共有58例患者表现出激素活性增加及相关的激素过多临床体征。25例患者肿瘤体积≥300 mL。这些患者手术并发症发生率增加，总体生存率（OS）较差（p<0.01）。共有14例患者出现转移扩散，尤其是肺和淋巴结转移。12例患者进行了肿瘤活检。11例患者发生肿瘤破裂。术前活检和/或肿瘤破裂与较差的OS率相关。仅5例患者实现了R2切除，3例患者无法进行手术。总之，由于大多数儿童ACC具有激素活性且可临床诊断，必须审慎讨论肿瘤活检的必要性。全面的术前和围手术期管理对肿瘤外科手术成功至关重要。
