ORLANDO, Fla. — Older age at adrenocortical carcinoma symptom onset and higher stage disease are independently associated with overall survival in adolescents with adrenocortical carcinoma, according to research presented here.
“Adrenocortical carcinoma is a rare, but aggressive childhood tumor,” Nidhi Gupta, MBBS, a fellow in pediatric endocrinology at the Mayo Clinic, told Endocrine Today. “Clinical and histopathological features of adrenocortical tumors are distinct in adults and pediatrics. It is important to differentiate adrenocortical adenoma from adrenocortical carcinoma because management and prognosis for the two vary significantly. No definite histopathological criterion has been validated to distinguish adrenocortical adenoma vs. adrenocortical carcinoma in pediatrics. Findings from our study have the potential to help clinicians with management and prognostication of adrenocortical tumors in children.”
Gupta and colleagues evaluated 41 adolescents with adrenocortical carcinoma (median age at symptom onset, 15.7 years; median duration of symptoms before diagnosis, 2.7 months) to describe the clinicopathologic data and identify prognostic factors for survival.
The most common presentation was mixed symptomatology with more than one hormonal abnormality (54%) followed by virilization alone (17%). More than half of participants underwent total adrenalectomy (67%), and 54% received adjuvant therapy. Median tumor diameter and median tumor weight were lower in participants younger than 4 years compared with participants older than 12 years.
More than half of participants had metastatic disease (63%), but that was less common in participants younger than 4 years (20%) compared with participants older than 12 years (81%; P = .0005). The liver was the most common site of metastases (74%) followed by the lungs (67%). Most participants were diagnosed with stage IV adrenocortical carcinoma using the European Network for the Study of Adrenal Tumors system.
Twenty-four percent of participants experienced recurrent disease.
Data was available on 22 participants who died; 19 were older than 12 years at the onset of symptoms, 19 had stage IV adrenocortical carcinoma, and 14 were treated with surgery and adjuvant therapy.
Nearly half of all participants were alive at 1.8 years’ follow-up (46%); survival was 34.8% at 2 years and 26.5% at 5 years. Overall survival was independently associated with age at onset of symptoms and stage of disease. The most accurate predictor for death or recurrence of adrenocortical carcinoma was the Weineke scoring system with 100% sensitivity and 83.3% specificity.
“Our study provides evidence for close association of the Wieneke scoring system with patient outcomes in the pediatric population compared to the Weiss and modified Weiss scoring systems,” Gupta said. “Additionally, younger age at onset of symptoms (< 4 years) and less advanced stage of disease (stage I or II by ENSAT system) were noted to be favorable prognostic factors for survival in children with adrenocortical carcinoma.”
Gupta added that complete surgical resection of the tumor is key to disease management.
“Despite multimodality treatment strategies, including radiotherapy and chemotherapy with mitotane and other cisplatin-based regimens, adrenocortical carcinoma is associated with poor survival,” she said. “Due to rarity and heterogeneity of pediatric adrenocortical carcinoma, collaborative international prospective trials of various treatment regimens need to be pursued.” – by Amber Cox
Gupta N, et al. OR31-6. Presented at: The Endocrine Society Annual Meeting; April 1-4, 2017; Orlando, Fla.
Disclosures: The researchers report no relevant financial disclosures.