Jain Vishesh, Sehgal Mehak, Dhua Anjan, Bakhshi Sameer, Kandasamy Devasenathipathy, Mani Kalaivani, Sharma Rajni, Jain Vandana, Tandon Nikhil, Agarwala Sandeep
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
Department of Medical Oncology, Dr. B.R. Ambedkar Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.
J Indian Assoc Pediatr Surg. 2022 Mar-Apr;27(2):157-162. doi: 10.4103/jiaps.JIAPS_351_20. Epub 2022 Mar 1.
Adrenocortical tumors are rare tumors in children with a paucity of outcome data, especially in a resource-challenged setting.
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.
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 ( = 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.
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.
肾上腺皮质肿瘤在儿童中较为罕见，且缺乏预后数据，尤其是在资源有限的环境中。
进行了一项回顾性研究，以评估1992年1月至2019年12月期间在我院就诊的肾上腺皮质肿瘤患儿的临床表现、治疗及预后情况。
在研究期间，共纳入28例患儿。其中9例为腺瘤，其余19例为肾上腺皮质癌。发病的中位年龄为48个月。近一半的肾上腺皮质癌患儿表现为肿块，而腺瘤患儿仅有10%表现为肿块（P = 0.049）。60%的患者出现男性化，且在大多数情况下与库欣样特征相关。3例患儿（15.7%）在就诊时已有转移。所有患儿均接受了手术切除，6例有肿瘤播散，2例有肉眼残留。5例患者在平均11.8个月后出现复发。腺瘤患者的4年总生存率和无事件生存率分别为100%，而肾上腺皮质癌患者分别为75%和44%。接受完整手术切除且无肿瘤播散的患儿预后相对较好。
肾上腺皮质肿瘤在儿童中并不常见。腺瘤预后良好。不完全切除或转移性肿瘤无无病生存。肿瘤播散在完全切除的肿瘤中也可能产生不良后果。
