Universität zu Köln

Riedmeier, Maria, Decarolis, Boris, Haubitz, Imme, Reibetanz, Joachim, Wiegering, Armin ORCID: 0000-0001-7777-0909, Haertel, Christoph, Schlegel, Paul-Gerhardt, Fassnacht, Martin ORCID: 0000-0001-6170-6398 and Wiegering, Verena (2022). Assessment of prognostic factors in pediatric adrenocortical tumors: a systematic review and evaluation of a modified S-GRAS score. Eur. J. Endocrinol., 187 (6). S. 751 - 764. OXFORD: OXFORD UNIV PRESS. ISSN 1479-683X

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Abstract

ObjectivePediatric adrenocortical carcinoma (pACC) is rare and prognostic stratification remains challenging. We summarized the clinical prognostic factors of pACC and determined the prognostic value of the pediatric scoring system (pS-GRAS) in adaption to the recommendation (S-GRAS) of the European Network for the Study of Adrenal Tumors for the classification of adult ACC. DesignAnalysis of pACC patients of 33 available retrospective studies in the literature. MethodsWe searched the PubMed and Embase databases for manuscripts regarding pACC. The pS-GRAS score was calculated as a sum of tumor stage (1 = 0; 2-3 = 1; 4 = 2 points), grade (Ki67 index/rate of mitosis 0-9%/low = 0; 10-19%/intermediate = 1; >= 20%/high = 2 points), resection status (R0 = 0; RX = 1; R1 = 2; R2 = 3 points), age (<4 years = 0; >= 4 years = 1 point), hormone-related symptoms (androgen production = 0; glucocorticoid/mixed/no hormone production = 1 point) generating 10 scores and 4 groups (1: 0-2, 2: 3-4, 3: 5, 4: 6-9). The primary endpoint was overall survival (OS). ResultsWe included 733 patients. The median age was 2.5 years and >85% of pACC showed hormone activity (mixed 50%, androgen 29%, glucocorticoid 21%). Androgen production was associated with a superior OS. Increasing age correlated with higher rates of inactive or only glucocorticoid-producing tumors, advanced tumor stage, and case fatality. Especially infants < 4 years showed more often low-risk constellations with an increased OS for all tumor stages. The pS-GRAS score correlated with clinical outcome; median OS was 133 months (95% CI: 36-283) in group 1 (n = 49), 110 months (95% CI: 2.9-314) in group 2 (n = 57), 49 months (95% CI: 5.8-278) in group 3 (n = 18), and 16 months (95% CI: 2.4-267) in group 4; (n = 11) P < 0.05). ConclusionThe pS-GRAS score seems to have a high predictive value in the pACC patients, may serve as a helpful tool for risk stratification in future studies, and should be evaluated prospectively in an international context.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Riedmeier, Maria UNSPECIFIED UNSPECIFIED UNSPECIFIED Decarolis, Boris UNSPECIFIED UNSPECIFIED UNSPECIFIED Haubitz, Imme 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 UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-667994
DOI:	10.1530/EJE-22-0173
Journal or Publication Title:	Eur. J. Endocrinol.
Volume:	187
Number:	6
Page Range:	S. 751 - 764
Date:	2022
Publisher:	OXFORD UNIV PRESS
Place of Publication:	OXFORD
ISSN:	1479-683X
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language ADRENAL-CORTICAL TUMORS; CLINICAL CHARACTERISTICS; CHILDREN; CHILDHOOD; NEOPLASMS; CARCINOMA; P53; MUTATION; CANCER; CLASSIFICATION Multiple languages Endocrinology & Metabolism Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/66799