Abstract
ABSTRACTObjective: To analyze clinical, laboratory and histopathological features and the path to diagnosis establishment and treatment of patients with adrenal carcinoma (AC).Methods: Retrospective study with 13 patients assisted at the pediatric oncology service of Hospital das Clínicas, Universidade Federal de Minas Gerais, Brazil, between 2004 and 2015.Results: Age at diagnosis ranged from 1.0 to 14.8 years (median: 2.0 years). Manifestations of hypercortisolism were identified in all cases and virilization in all girls. All patients met the Weiss criteria to AC histopathological diagnosis. Immunohistochemistry was performed in 61.5% of the cases. Most patients had stage I disease (76.9%). All subjects were submitted to total tumor resection. Two patients (stages III and IV disease) received chemotherapy associated to mitotane. The only death case was that of a patient with stage IV disease. The probability of overall survival for the entire group up to 5.0 years was 92.3±7.4%. The median time between the onset of symptoms and diagnosis was 9.5 months, and 6.0 months between first visit and start of treatment.Conclusions: Low age at diagnosis, predominance of cases with localized disease and complete tumor resection - with only one case of tumor capsule rupture - can possibly explain the favorable evolution of the studied population. The long period between onset of symptoms and diagnosis highlights the importance of training pediatricians for early recognition of AC signs and symptoms.
Highlights
IntroductionAdrenal carcinoma (AC) accounts for 0.2% of childhood and adolescent malignancies, with annual worldwide incidence of 0.2 to 0.3 cases per million subjects.[1,2] It is more common in females, with 2:1 proportion.[3,4,5] Most symptomatic patients present virilization due to increased secretion of androgens or Cushing’s syndrome (hypercortisolism).[6]
Adrenal carcinoma (AC) accounts for 0.2% of childhood and adolescent malignancies, with annual worldwide incidence of 0.2 to 0.3 cases per million subjects.[1,2] It is more common in females, with 2:1 proportion.[3,4,5] Most symptomatic patients present virilization due to increased secretion of androgens or Cushing’s syndrome.[6]In Brazil, regions South and Southeast have 10 to 15 times higher incidence of AC compared to worldwide, reaching 4.2 cases per million inhabitants
This finding seems to be associated with the high prevalence of germline TP53 R337H mutation in the population, being identified in more than 90% of CA cases in these regions.[7,8]. The rarity of this disease, the differential diagnosis with other diseases commonly found in pediatric age groups, and frequent diagnosis delay justify the conduction of studies that contribute to the understanding of this neoplasm and increase its dissemination among pediatricians and specialists.[3]
Summary
Adrenal carcinoma (AC) accounts for 0.2% of childhood and adolescent malignancies, with annual worldwide incidence of 0.2 to 0.3 cases per million subjects.[1,2] It is more common in females, with 2:1 proportion.[3,4,5] Most symptomatic patients present virilization due to increased secretion of androgens or Cushing’s syndrome (hypercortisolism).[6]. In Brazil, regions South and Southeast have 10 to 15 times higher incidence of AC compared to worldwide, reaching 4.2 cases per million inhabitants. This finding seems to be associated with the high prevalence of germline TP53 R337H mutation in the population, being identified in more than 90% of CA cases in these regions.[7,8]. The rarity of this disease, the differential diagnosis with other diseases commonly found in pediatric age groups, and frequent diagnosis delay justify the conduction of studies that contribute to the understanding of this neoplasm and increase its dissemination among pediatricians and specialists.[3]
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