Adrenocortical tumours in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations

Virgone, Calogero; Roganović, Jelena; Vorwerk, Peter; Redlich, Antje; Schneider, Dominik T.; Janić, Dragana; Bień, Ewa; Almaraz, Ricardo López; Godziński, Jan; Österlundh, Gustaf; Stachowicz‐Stencel, Teresa; Brugières, Laurence; Brecht, Ines B.; Thomas‐Teinturier, Cécile; Fresneau, Brice; Surun, Aurore; Ferrari, Andrea; Bisogno, Gianni; Orbach, Daniel

doi:10.1002/pbc.29025

Cited by 27 publications

(53 citation statements)

References 58 publications

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“…For the first time, standardized therapy recommendations were published in 2012 on the basis of the German GPOH-MET registry data [ 1 , 95 ]. Recently, there have been efforts to publish international standardized diagnostic and therapeutic recommendations [ 150 , 177 ].…”

Section: Discussionmentioning

confidence: 99%

Adrenocortical Carcinoma in Childhood: A Systematic Review

Riedmeier¹,

Decarolis

Haubitz³

et al. 2021

Cancers

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Adrenocortical tumors are rare in children. This systematic review summarizes the published evidence on pediatric adrenocortical carcinoma (ACC) to provide a basis for a better understanding of the disease, investigate new molecular biomarkers and therapeutic targets, and define which patients may benefit from a more aggressive therapeutic approach. We included 137 studies with 3680 ACC patients (~65% female) in our analysis. We found no randomized controlled trials, so this review mainly reflects retrospective data. Due to a specific mutation in the TP53 gene in ~80% of Brazilian patients, that cohort was analyzed separately from series from other countries. Hormone analysis was described in 2569 of the 2874 patients (89%). Most patients were diagnosed with localized disease, whereas 23% had metastasis at primary diagnosis. Only 72% of the patients achieved complete resection. In 334 children (23%), recurrent disease was reported: 81%—local recurrence, 19% (n = 65)—distant metastases at relapse. Patients <4 years old had a different distribution of tumor stages and hormone activity and better overall survival (p < 0.001). Although therapeutic approaches are typically multimodal, no consensus is available on effective standard treatments for advanced ACC. Thus, knowledge regarding pediatric ACC is still scarce and international prospective studies are needed to implement standardized clinical stratifications and risk-adapted therapeutic strategies.

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Section: Discussionmentioning

confidence: 99%

Adrenocortical Carcinoma in Childhood: A Systematic Review

Riedmeier¹,

Decarolis

Haubitz³

et al. 2021

Cancers

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show abstract

“…RT seemed to be well tolerated and possibly contributed to improved survival. Thus, cooperative groups as established within the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) could be the platform for common randomized trials to further improve patient outcomes in pediatric patients [56,57]. Furthermore, the ENSAT consortium [58,59], which mainly covers adult ACT patients, has shown how centralization and networking can continuously improve patient outcomes, particularly in rare cancer entities.…”

Section: Discussionmentioning

confidence: 99%

Radiotherapy for pediatric adrenocortical carcinoma – Review of the literature

Wiegering

Riedmeier²,

Thompson

et al. 2022

Clinical and Translational Radiation Oncology

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“…Prognosis of ACT patients with intraoperative tumor spillage is inferior compared to complete tumor resection necessitating intensified treatment (64). Thus, in children and adolescents with adrenal masses in whom definite diagnosis cannot be established prior to surgery, microscopical complete tumor resection instead of biopsy must be performed.…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore, the biopsy comes with significant risks such as hemorrhage and the risk of tumor dissemination precluding a R 0 resection (63). The latter is a major prognostic factor in children and adolescents with ACT (58,64).…”

Section: Histological Diagnosismentioning

confidence: 99%

Adrenocortical Tumors and Pheochromocytoma/Paraganglioma Initially Mistaken as Neuroblastoma—Experiences From the GPOH-MET Registry

et al. 2022

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In children and adolescents, neuroblastoma (NBL), pheochromocytoma (PCC), and adrenocortical tumors (ACT) can arise from the adrenal gland. It may be difficult to distinguish between these three entities including associated extra-adrenal tumors (paraganglioma, PGL). Precise discrimination, however, is of crucial importance for management. Biopsy in ACT or PCC is potentially harmful and should be avoided whenever possible. We herein report data on 10 children and adolescents with ACT and five with PCC/PGL, previously mistaken as NBL. Two patients with adrenocortical carcinoma died due to disease progression. Two (2/9, missing data in one patient) patients with a final diagnosis of ACT clearly presented with obvious clinical signs and symptoms of steroid hormone excess, while seven patients did not. Blood analyses indicated increased levels of steroid hormones in one additional patient; however, urinary steroid metabolome analysis was not performed in any patient. Two (2/10) patients underwent tumor biopsy, and in two others tumor rupture occurred intraoperatively. In 6/10 patients, ACT diagnosis was only established by a reference pediatric pathology laboratory. Four (4/5) patients with a final diagnosis of PCC/PGL presented with clinical signs and symptoms of catecholamine excess. Urine tests indicated possible catecholamine excess in two patients, while no testing was carried out in three patients. Measurements of plasma metanephrines were not performed in any patient. None of the five patients with PCC/PGL received adrenergic blockers before surgery. In four patients, PCC/PGL diagnosis was established by a local pathologist, and in one patient diagnosis was revised to PGL by a pediatric reference pathologist. Genetic testing, performed in three out of five patients with PCC/PGL, indicated pathogenic variants of PCC/PGL susceptibility genes. The differential diagnosis of adrenal neoplasias and associated extra-adrenal tumors in children and adolescents may be challenging, necessitating interdisciplinary and multidisciplinary efforts. In ambiguous and/or hormonally inactive cases through comprehensive biochemical testing, microscopical complete tumor resection by an experienced surgeon is vital to preventing poor outcome in children and adolescents with ACT and/or PCC/PGL. Finally, specimens need to be assessed by an experienced pediatric pathologist to establish diagnosis.

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Adrenocortical tumours in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations

Cited by 27 publications

References 58 publications

Adrenocortical Carcinoma in Childhood: A Systematic Review

Adrenocortical Carcinoma in Childhood: A Systematic Review

Radiotherapy for pediatric adrenocortical carcinoma – Review of the literature

Adrenocortical Tumors and Pheochromocytoma/Paraganglioma Initially Mistaken as Neuroblastoma—Experiences From the GPOH-MET Registry

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