Adrenocortical tumors in children

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

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“…Virilization and precocious puberty are the most common symptoms, reported in 80-100% of patients (3)(4)(5)(6).…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2014

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“…Almost all children present with hormonally functioning tumors, most commonly leading to virilization, with a mixed virilization-hypercortisolism pattern somewhat less common. [3][4][5] Most children have resectable disease at presentation, with distant metastases present in fewer than one-third. Stage of disease, based on tumor invasiveness and size, is a major predictor of outcome.…”

Section: Discussionmentioning

confidence: 99%

Delayed Diagnosis and False Relapse Due to Paternal Testosterone Use in Adrenocortical Carcinoma

2014

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The prognosis of pediatric adrenocortical carcinoma often depends on prompt diagnosis to begin treatment before metastatic progression. We discuss a girl who presented at 8 months of age with virilization, which was thought to be due to exposure to a topical testosterone preparation being used by her father. Her testosterone level did not decrease promptly after her father discontinued the medication, however, and when she followed up with signs of Cushing syndrome 5 months later, metastatic adrenocortical carcinoma was diagnosed. The patient was successfully treated with surgery and multiagent chemotherapy. Nine months after the end of treatment, her testosterone level was again found to be elevated. Testosterone precursors were now absent, however, and there were no imaging signs of recurrence. Further history showed that her father had restarted topical testosterone, and this time, exogenous exposure was correctly diagnosed. As use of topical testosterone becomes more prevalent, exogenous exposure must be considered in the differential diagnosis of childhood virilization. Any persistent testosterone elevation after exposure ceases or signs of hypercortisolism, however, are inconsistent with this diagnosis. We believe that the risk-benefit ratio favors abdominal ultrasound to rule out malignancy in all children presenting with virilization.

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“…Гормонально неактивные опухоли встречаются реже (менее 10 %) и, как правило, у детей старшего возраста [3]. Доопе-рационное обследование должно включать методы визуализации (УЗИ, магнитно-резонансную томогра-фию, КТ), которые необходимо использовать для ана-томической характеристики образования, оценки его распространенности, подтверждения наличия/отсут-ствия отдаленных метастазов, а также оценку гормо-нального профиля и консультацию эндокринолога, контроль артериального давления.…”

Section: рис 6 иммуногистохимическое исследование: уровень пролиферunclassified

A case of adrenal cortical adenoma in 14-year-old boy

Ускова¹,

Kletskaya²,

Shamanskaya³

et al. 2016

Ross. ž. det. gematol. onkol.

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Adrenocortical tumors in children

Cited by 121 publications

References 20 publications

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

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

Delayed Diagnosis and False Relapse Due to Paternal Testosterone Use in Adrenocortical Carcinoma

A case of adrenal cortical adenoma in 14-year-old boy

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