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Clinical characteristics and outcome of 60 pediatric patients with malignant melanoma registered with the German Pediatric Rare Tumor Registry (STEP)


Offenmueller, Sonja; Leiter, Ulrike; Bernbeck, Benedikt; Garbe, Claus; Eigentler, Thomas; Borkhardt, Arndt; Friedrich Classen, Carl; Corbacioglu, Selim; Dirksen, Uta; Ebetsberger-Dachs, Georg; Heinzerling, Lucie; Jorch, Norbert; Kuhlen, Michaela; Lawlor, Jennifer; Niggli, Felix; Streiter, Monika; Schneider, Dominik T; Brecht, Ines (2017). Clinical characteristics and outcome of 60 pediatric patients with malignant melanoma registered with the German Pediatric Rare Tumor Registry (STEP). Klinische Pädiatrie, 229(6):322-328.

Abstract

BACKGROUND: Malignant melanoma (MM) is a common malignancy in adults while it is rare in children. Thus, information on clinical behavior of pediatric MM is incomplete.
PATIENTS: The German Pediatric Rare Tumor Registry (STEP) presents a prospective analysis of 60 childhood MM cases diagnosed between June 2006 and December 2014.
METHODS: Patients' ages ranged between 0 and 17 years at initial diagnosis (median age 9.6 years). Information on patient's and tumor characteristics was obtained by standardized documentation. Three-year overall survival (OS) and event-free survival (EFS) were estimated by the Kaplan-Meier test.
RESULTS: Follow-up ranged from 0 to 116 months with a median of 36.5 months, however, univariate analysis was performed for 46 cases with a follow-up > 3 months, only. Cases with spitzoid histotype (40%) did not show a significantly different outcome compared to cases with non-spitzoid MM. Breslow thickness ≤ 2.00 mm was identified in 30% of the cases and 18% were Clark level I to III. Adjuvant therapy was used in 45% of cases. OS at 3 years was 100%, EFS 95.2%.
CONCLUSION: We present a series of cases with a high number of spitzoid malignant melanoma and advanced pediatric melanoma, but surprisingly good overall survival rates. Spitzoid and non-spitzoid MM do not differ in clinical behavior and survival.

Abstract

BACKGROUND: Malignant melanoma (MM) is a common malignancy in adults while it is rare in children. Thus, information on clinical behavior of pediatric MM is incomplete.
PATIENTS: The German Pediatric Rare Tumor Registry (STEP) presents a prospective analysis of 60 childhood MM cases diagnosed between June 2006 and December 2014.
METHODS: Patients' ages ranged between 0 and 17 years at initial diagnosis (median age 9.6 years). Information on patient's and tumor characteristics was obtained by standardized documentation. Three-year overall survival (OS) and event-free survival (EFS) were estimated by the Kaplan-Meier test.
RESULTS: Follow-up ranged from 0 to 116 months with a median of 36.5 months, however, univariate analysis was performed for 46 cases with a follow-up > 3 months, only. Cases with spitzoid histotype (40%) did not show a significantly different outcome compared to cases with non-spitzoid MM. Breslow thickness ≤ 2.00 mm was identified in 30% of the cases and 18% were Clark level I to III. Adjuvant therapy was used in 45% of cases. OS at 3 years was 100%, EFS 95.2%.
CONCLUSION: We present a series of cases with a high number of spitzoid malignant melanoma and advanced pediatric melanoma, but surprisingly good overall survival rates. Spitzoid and non-spitzoid MM do not differ in clinical behavior and survival.

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Additional indexing

Other titles:Klinische Charakteristika von 60 Patienten mit malignem Melanom des Registers für Seltene Tumorerkrankungen in der Pädiatrie (STEP)
Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Children's Hospital Zurich > Medical Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:November 2017
Deposited On:02 Feb 2018 12:45
Last Modified:19 Feb 2018 10:58
Publisher:Georg Thieme Verlag
ISSN:0300-8630
OA Status:Closed
Publisher DOI:https://doi.org/10.1055/s-0043-118662
PubMed ID:29017184

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