Adrenocortical carcinoma in children: First population-based clinicopathological study with long-term follow-up

Abstract

Abstract. Adrenocortical carcinoma (ACC) is rare in both adult and pediatric populations. Literature suggests significant differences between children and adults in presentation, histological properties and outcome. The aim of this first nationwide study on pediatric ACC was to describe the incidence, presentation, pathological characteristics, treatment and survival in The Netherlands. All ACC patients aged <20 years at diagnosis and registered in the population-based Netherlands Cancer Registry between 1993 and 2010 were included. Clinical data were extracted from medical records. Archival histological slides were collected via the Dutch Pathology Registry (PALGA). We compared our findings to all clinical studies on pediatric ACC that were found on PubMed. Based on the results, 12 patients were identified: 8 females and 4 males. The median age was 4

Adrenocortical Carcinoma in Children: A Clinicopathological Analysis of 41 Patients at the Mayo Clinic from 1950 to 2017

“…Consistent with previous studies [3, 9, 15, 16, 26, 34], mixed symptomatology (virilization, Cushing syndrome, and hyperaldosteronism) was the most common presentation of ACC across all age groups in our cohort. One patient presented at 5 months of age with lower extremity weakness and was diagnosed with ectopic extramedullary intradural ACC with 2 intact adrenal glands.…”

“…Results from the Surveillance, Epidemiology, and End Results (SEER) database indicated that overall 5-year survival for patients younger than 4 years was 91.1% (95% CI 74.8–97.1) and that it reduced significantly for patients aged 5–19 years to 29.8% (95% CI 16.8–44.1) [4]. Age less than 4 years was an important predictor of survival in our patients as well as in several other cohorts [3, 14-16]. This was likely related to a smaller tumor size (< 10 cm) [18], lower tumor weight (< 400 g), lower tumor volume (< 200 cm 3 ) [16], disease localized to adrenal gland [14, 18], less advanced disease (stage I/II) [15], and lower incidence of metastatic disease in younger children compared to older ones [14].…”

“…Mitotane treatment longer than 6 months and mitotane levels greater than 14 mg/L were found to be associated with significantly better survival. Radiation therapy alone or in combination with chemotherapy has rarely been used in previous studies [3, 15, 18]. …”

“…These studies included patients from Brazil [9, 15], the USA [14, 18], the Netherlands [3], Turkey [26], and other countries [27], thereby disputing any geographical pattern. A majority of our patients, which was primarily an American cohort, had onset of symptoms after 12 years of age.…”

“…Adrenocortical tumors (ACT) have been reported to have either early age of onset (< 4 years) [3, 9, 14-16] or biphasic age distribution (< 5 years and > 10 years) [18, 26]. These studies included patients from Brazil [9, 15], the USA [14, 18], the Netherlands [3], Turkey [26], and other countries [27], thereby disputing any geographical pattern.…”