■肾上腺皮质肿瘤是儿童的罕见肿瘤,缺乏结果数据,尤其是在资源受到挑战的环境中。
■进行了一项回顾性研究以评估其表现,管理,以及1992年1月至2019年12月到我们研究所就诊的肾上腺皮质肿瘤儿童的结局。
■在研究期间,包括28名儿童。9名儿童存在腺瘤,其余19名儿童患有肾上腺皮质癌。报告的中位年龄为48个月。将近一半的患有癌的儿童表现出肿块,而只有10%的腺瘤(P=0.049)。在60%的患者中存在病毒化,并且在大多数情况下与Cushingoid特征有关。3名儿童(15.7%)出现转移。所有儿童均接受了手术切除,其中6例溢出,两名患者残留。在平均11.8个月后,在5例患者中观察到复发。腺瘤患者的4年总生存率和无事件生存率分别为100%和75%和44%。分别,在癌症中。接受完全手术切除而没有任何溢出的儿童具有相对有利的结果。
■肾上腺皮质肿瘤是儿童的少见肿瘤。腺瘤有良好的结局。在不完全切除或转移性肿瘤中没有无病生存。肿瘤溢出在完全切除的肿瘤中也可能具有不良结果。
UNASSIGNED: Adrenocortical tumors are rare tumors in children with a paucity of outcome data, especially in a resource-challenged setting.
UNASSIGNED: A retrospective study was conducted to evaluate the presentation, management, and outcomes of children with
adrenocortical tumors who presented to our institute from January 1992 to December 2019.
UNASSIGNED: During the study period, 28 children were included. Adenoma was present in nine children and the remaining 19 children had
adrenocortical carcinoma. The median age of presentation was 48 months. Nearly half of the children with carcinomas presented with a mass compared to only 10% adenomas (P = 0.049). Virilization was present in 60% of the patients and in most cases was associated with Cushingoid features. Three children (15.7%) had metastasis at presentation. All children underwent surgical excision with a spill in 6 and gross residue in two patients. Recurrence was observed in five patients after a mean duration of 11.8 months. The 4-year overall survival and event-free survival were 100% in patients with adenomas compared to 75% and 44%, respectively, in carcinoma. Children who underwent complete surgical excision without any spill had a relatively favorable outcome.
UNASSIGNED: Adrenocortical tumors are uncommon tumors in children. Adenomas have a favorable outcome. There was no disease-free survival in incompletely resected or metastatic tumors. Tumor spill may also have an adverse outcome in completely resected tumors.