Universität zu Köln

Riedmeier, Maria, Decarolis, Boris ORCID: 0000-0002-1607-0543, Haubitz, Imme, Mueller, Sophie, Uttinger, Konstantin, Boerner, Kevin, Reibetanz, Joachim, Wiegering, Armin ORCID: 0000-0001-7777-0909, Haertel, Christoph, Schlegel, Paul-Gerhardt, Fassnacht, Martin ORCID: 0000-0001-6170-6398 and Wiegering, Verena ORCID: 0000-0002-2802-4034 (2021). Adrenocortical Carcinoma in Childhood: A Systematic Review. Cancers, 13 (21). BASEL: MDPI. ISSN 2072-6694

Full text not available from this repository.

Abstract

Simple Summary Pediatric adrenocortical tumors are rare. Little information is available on the incidence, risk factors, prognostic factors, treatment, and overall survival. In this systematic review, we performed a search of the current literature. The most common reported risk factors are age > 4 years, high pathological tumor score, and advanced stage in which prognosis is poor. Treatment options are surgery, radiation, or chemotherapy, but ongoing randomized trials are lacking. International prospective studies must be the next step to implement standardized clinical stratifications and risk-adapted therapeutic strategies. 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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Riedmeier, Maria UNSPECIFIED UNSPECIFIED UNSPECIFIED Decarolis, Boris UNSPECIFIED orcid.org/0000-0002-1607-0543 UNSPECIFIED Haubitz, Imme UNSPECIFIED UNSPECIFIED UNSPECIFIED Mueller, Sophie UNSPECIFIED UNSPECIFIED UNSPECIFIED Uttinger, Konstantin UNSPECIFIED UNSPECIFIED UNSPECIFIED Boerner, Kevin UNSPECIFIED UNSPECIFIED UNSPECIFIED Reibetanz, Joachim UNSPECIFIED UNSPECIFIED UNSPECIFIED Wiegering, Armin UNSPECIFIED orcid.org/0000-0001-7777-0909 UNSPECIFIED Haertel, Christoph UNSPECIFIED UNSPECIFIED UNSPECIFIED Schlegel, Paul-Gerhardt UNSPECIFIED UNSPECIFIED UNSPECIFIED Fassnacht, Martin UNSPECIFIED orcid.org/0000-0001-6170-6398 UNSPECIFIED Wiegering, Verena UNSPECIFIED orcid.org/0000-0002-2802-4034 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-573082
DOI:	10.3390/cancers13215266
Journal or Publication Title:	Cancers
Volume:	13
Number:	21
Date:	2021
Publisher:	MDPI
Place of Publication:	BASEL
ISSN:	2072-6694
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language ADRENAL-CORTICAL TUMORS; GERMLINE TP53 MUTATIONS; CLINICAL CHARACTERISTICS; IMMUNOHISTOCHEMICAL EXPRESSION; PROGNOSTIC-FACTORS; PEDIATRIC-PATIENTS; HOSPITAL VOLUME; UNITED-STATES; CHILDREN DATA; NEOPLASMS Multiple languages Oncology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/57308