Background: The management of rare tumors at reference centers with specialized multidisciplinary tumor boards (MTBs) improves patient survival. No international standardized diagnostic and treatment recommendations for pediatric patients with adrenocortical carcinoma (ACC) exist so far. The aim of this case-based study is to determine how congruent treatment recommendations are in different specialized institutions in different countries. Methods: In this cross-sectional, case-based survey study, five anonymized patient cases, including imaging data of pediatric adrenocortical carcinomas (pACCs), were distributed to seven international specialized centers located in Brazil, Germany, India, Italy, Poland, Turkey, and the USA. The centers were tasked with providing recommendations regarding the treatment sequence and modalities, as well as indicating the level of consensus on these decisions within their MTBs. For each case, the treatment approach recommended by the highest number of centers was recorded to calculate an agreement percentage relative to all treatment recommendations made. The consensus level for each case was determined on a scale of 1 to 10, with higher scores indicating greater agreement among MTB members. Results: A total of five patient cases were discussed across seven MTBs, yielding a total of 34 treatment recommendations. The agreement percentages for the primary therapeutic approach for each case were as follows: primary therapeutic approach: 88.6%; surgical approaches: 51.8%; and systemic chemotherapy recommendations: 53%. Conclusions: This study represents the first case-based analysis of international inter-center agreement on pediatric ACC management. Inter-center agreement regarding treatment allocation and sequencing was low, while the level of consensus within each MTB was high. These findings point to potentially significant and clinically relevant differences in treatment standards across centers, highlighting the need for international collaboration and standardized diagnostic and therapeutic recommendations, especially for rare tumors. Increased case-based exchanges between centers are essential to reduce the substantial discrepancies observed here and to further standardize the management of pediatric patients with ACC.
背景：在具备专业多学科肿瘤委员会（MTBs）的参考中心对罕见肿瘤进行管理可提高患者生存率。目前国际上尚无针对儿童肾上腺皮质癌（ACC）的统一标准化诊断与治疗建议。本病例研究旨在评估不同国家专业机构间治疗建议的一致性程度。方法：在这项横断面病例调查研究中，将五例包含儿童肾上腺皮质癌影像学资料的匿名病例分发给位于巴西、德国、印度、意大利、波兰、土耳其和美国的七家国际专业中心。各中心需提供治疗顺序与方案建议，并说明其MTB内部对这些决策的共识程度。针对每个病例，记录最多中心推荐的治疗方案，并计算其占所有治疗建议的同意百分比。各病例的共识水平按1-10分评估，分值越高表明MTB成员间共识度越强。结果：七个MTB共讨论五例患者病例，产生34项治疗建议。各病例主要治疗方案的同意百分比分别为：主要治疗方案88.6%；手术方案51.8%；全身化疗建议53%。结论：本研究首次通过病例分析探讨国际多中心在儿童ACC治疗方面的共识度。中心间在治疗方案选择与排序方面共识度较低，而各MTB内部共识水平较高。这些发现提示各中心治疗标准存在显著且具临床意义的差异，凸显了国际协作及标准化诊疗建议的必要性，尤其对于罕见肿瘤。加强中心间基于病例的交流对减少当前显著差异、推进儿童ACC治疗标准化至关重要。
肿瘤(癌症)患者之家