Chatterjee Gaurav, DasGupta Shatavisha, Mukherjee Gautam, Sengupta Moumita, Roy Paromita, Arun Indu, Datta Chhanda, Mishra Prafulla Kumar, Banerjee Sugato, Chatterjee Uttara
Department of Pathology, IPGME&R, Kolkata, India,
Pediatr Surg Int. 2015 Jun;31(6):563-71. doi: 10.1007/s00383-015-3708-x. Epub 2015 Apr 17.
Adrenocortical tumors (ACT) occur rarely in pediatric age group. Pediatric ACTs behave differently from their histologically similar adult counterparts and standard adult criteria often cannot accurately predict their clinical behavior. The aim of the present study was to document the clinicopathologic spectrum of pediatric ACTs and to assess the utility of Wieneke scoring system in predicting clinical behavior of these tumors.
This multi-institutional study comprised of 13 cases of pediatric ACTs from January 2005 to May 2014. Clinical features and gross pathologic characteristics were obtained from records. Comprehensive analyses of microscopic features were performed. Each tumor was assessed according to criteria proposed by Wieneke et al. and was assigned to benign, intermediate for malignancy or malignant group. The standard adult Weiss criteria were also applied for comparison.
There were total 6 cases of adrenocortical adenomas and 7 cases of adrenocortical carcinomas. Most of the children (76.9%) presented with endocrine dysfunction. Lower age of presentation was significantly associated with better prognosis. Applying Wieneke criteria, there were 6 benign and 6 malignant cases and one case was assigned to intermediate for malignancy group. The clinical behavior of all the cases was consistent with Wieneke criteria categorization. Applying Weiss criteria, 3 cases with benign clinical behavior were assigned to malignant group.
Our study validates the reliability of Wieneke scoring system in predicting malignancy in pediatric ACTs. It is simple and easy to use and therefore useful in day-to-day practice.
肾上腺皮质肿瘤（ACT）在儿童年龄组中很少见。儿童ACT的行为与其组织学上相似的成人对应物不同，标准的成人标准通常不能准确预测其临床行为。本研究的目的是记录儿童ACT的临床病理谱，并评估Wieneke评分系统在预测这些肿瘤临床行为方面的效用。
这项多机构研究包括2005年1月至2014年5月期间的13例儿童ACT。从记录中获取临床特征和大体病理特征。对微观特征进行综合分析。根据Wieneke等人提出的标准对每个肿瘤进行评估，并分为良性、恶性中间型或恶性组。还应用标准的成人Weiss标准进行比较。
共有6例肾上腺皮质腺瘤和7例肾上腺皮质癌。大多数儿童（76.9%）表现为内分泌功能障碍。发病年龄较低与较好的预后显著相关。应用Wieneke标准，有6例良性和6例恶性病例，1例被归为恶性中间型组。所有病例的临床行为与Wieneke标准分类一致。应用Weiss标准，3例临床行为良性的病例被归为恶性组。
我们的研究验证了Wieneke评分系统在预测儿童ACT恶性程度方面的可靠性。它简单易用，因此在日常实践中很有用。
