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

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.

Method 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.

Zusammenfassung

Hintergrund Maligne Melanome (MM) zählen zu den häufigsten Malignomen im Erwachsenenalter. Im Gegensatz dazu sind sie eine seltene Diagnose im Kindes- und Jugendalter. Die Datenlage über das klinische Verhalten von pädiatrischen MM ist somit unvollständig.

Patienten Das deutsche Register für „Seltene Tumorerkrankungen in der Pädiatrie“ (STEP) stellt hier eine prospektive Analyse von 60 kindlichen Fällen maligner Melanome vor, die zwischen Juni 2006 und Dezember 2014 erstdiagnostiziert wurden.

Methoden Daten über Tumor- und Patientencharakteristika wurden durch standardisierte Dokumentation erhoben. Das Gesamtüberleben (OS) und ereignisfreie Überleben (EFS) zum Zeitpunkt von 3 Jahren wurden durch den Kaplan-Meier-Test ermittelt.

Ergebnisse Das Alter der Patienten lag bei Erstdiagnose zwischen 0 und 17 Jahren (mittleres Alter 9,6 Jahre). Verlaufskontrollen wurden bis zu 116 Monate nach Diagnose mit einem Median von 36,5 Monaten durchgeführt. Eine univariate Analyse wurde nur in 46 Fälle mit einer Nachbeobachtung von > 3 Monaten durchgeführt. Fälle mit spitzoider Histologie (40%) zeigten kein signifikant unterschiedliches Überleben im Vergleich zu Fällen mit nicht-spitzoider Histologie (60%). Eine Breslow Dicke ≤ 2,00 mm wurde in 30% der Fälle identifiziert und 18% wurden im Clark Level I bis III klassifiziert. Eine adjuvante Therapie wurde in 45% der Fälle verabreicht. Das OS zum Zeitpunkt von 3 Jahren betrug 100%, das EFS 95,2%.

Diskussion Wir zeigen eine Serie pädiatrischer MM mit einer hohen Anzahl von spitzoidem Subtyp und fortgeschrittenen Stadien, aber einem auffällig guten Gesamtüberleben. Spitzoide MM unterscheiden sich im klinischen Verhalten und Überleben nicht von den nicht-spitzoiden MM.

Key words

malignant melanoma - rare pediatric tumors - pediatric oncology - German Rare Pediatric Tumor Registry - orphan disease - STEP Registry

Schlüsselwörter

pädiatrische Onkologie - maligne Melanome - seltene kindliche Tumore - Register fur Seltene Tumorerkrankungen in der Pädiatrie - STEP Register

^* the authors contributed equally to this work

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