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TumorDiagnostik & Therapie 2014; 35(1): 28-35
DOI: 10.1055/s-0033-1356341
Thieme Onkologie aktuell
© Georg Thieme Verlag KG Stuttgart · New York

Testicular Germ Cell Tumors in Boys < 10 Years: Results of the Protocol MAHO 98 in Respect to Surgery and Watch & Wait Strategy

Keimzelltumoren des Hodens bei Jungen < 10 Jahre – Ergebnisse des Protokolls MAHO 98 unter Berücksichtigung der Operation und Nachbeobachtung
U. Göbel
1   Heinrich-Heine-Universität Düsseldorf, Germany
,
R. Haas
2   Pediatric Hematology and Oncology, Ludwig-Maximilians-University Munich, Germany
,
G. Calaminus
3   Pediatric Hematology and Oncology, University Münster, Germany
,
P. Botorek
1   Heinrich-Heine-Universität Düsseldorf, Germany
,
P. Schmidt
2   Pediatric Hematology and Oncology, Ludwig-Maximilians-University Munich, Germany
,
C. Teske
3   Pediatric Hematology and Oncology, University Münster, Germany
,
S. Schönberger
4   Pediatric Hematology and Oncology, University of Bonn, Germany
,
D. T. Schneider
5   Clinic of Pediatrics, Municipal Hospital Dortmund, Germany
,
D. Harms
6   Department of Pediatric Pathology, Christian Albrechts University, Kiel, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
28 January 2014 (online)

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Abstract

Background: In 1982 the GPOH opened the 1st protocol for germ cell tumors (GCTs) of the testis (MAHO 82). Here the results of the 5th version (MAHO 98) will be offered for boys < 10 year of age.

Objectives: In MAHO 98 watch and wait (w & w) strategy after inguinal tumororchiectomy was widened from 2 to 10-year-old boys with YST stage IA (group I); other invasive measures were omitted. Thus the prognostic impact of a non-recommended surgery like transscrotal operation +/− conventional biopsy (group II) can be evaluated.

Methods: Clinical diagnosis and staging by ultrasound and tumor marker. In blurry cases, a frozen section was recommended to confirm the diagnosis by histology intraoperatively. Indications for adjuvant chemotherapy were: YST stage IA without elevated AFP, YST stage > IA and all mixed malignant GCTs.

Results: From 1998 till 2005 128 boys < 10 years with a testicular GCT were registered. Histology: YST n = 76, teratoma n = 46, mixed malignant GCT n = 6. Tumor stage IA: n = 101. All teratoma patients survive event-free. At all, only 19/82 patients with a malignant GCT received chemotherapy including 5 patients with a tumor progress after w&w (2/49 group I and 3/15 group II patients, respectively) and 1 patient (YST IIIA) with relapse after adjuvant chemotherapy. Transscrotal surgery (n = 18) or tumorenucleation (n = 6) remained without event. Indeed all patients survived.

Conclusions: Prognosis of boys < 10 year with a testicular GCT is excellent as ~ 80% will be cured by high inguinal tumororchiectomy alone. w&w is feasible and safe even after not recommended surgery if suitable follow-up is assured at least in stage IA cases.

Zusammenfassung

Hintergrund: 1982 öffnete die GPOH das Protokoll MAHO 82 zur Diagnostik und Therapie testikulärer Keimzelltumoren (GCTs). Die 5. Version folgte 1998, deren Ergebnisse für Jungen < 10 Jahre mitgeteilt werden.

Ziele: In MAHO 98 wurde nach Tumororchiektomie durch hohe inguinale Inzision die Surveillance-Strategie (w&w) für Jungen mit Dottersacktumor (YST) im Stadium IA von 2 auf 10 Jahre erweitert und auf weitere Operationen verzichtet (Gruppe I). Die Prognose nach nicht protokollgemäßer Tumorresektion (Gruppe II) wird evaluiert.

Methoden: Diagnose klinisch durch Ultra­schall und Tumormarker; evtl. intraoperativ Schnellschnittdiagnostik. Chemotherapie bei YST Stadium IA mit normalem Serum-AFP oder YST Stadium > IA, bzw. gemischtem malignen GCT. Zur Gruppe II zählen u. a. Patienten mit transskrotaler Operation  +/− üblicher Biopsie.

Ergebnisse: Von 1998 bis 2005 wurden 128 Jungen < 10 Jahre gemeldet. Histologie: YST n = 76, Teratom n = 46, gemischter maligner GCT n = 6. Stadium IA: n = 101. Alle Patienten mit Te­ratom überleben ereignisfrei. Insgesamt nur 19 von 82 Patienten mit einem malignen GCT erhielten Chemotherapie, darunter 5 Patienten mit Tumorprogress unter w&w (2/49 der Gruppe I und 3/15 der Gruppe II) sowie 1 Patient (YST IIIA) mit Rückfall; dieser ist in anhaltender Zweit­remission. Transskrotale Operation (n = 18) oder Tumorenucleation (n = 6) waren ohne Ereignis. Alle Patienten überleben.

Schlussfolgerungen: Die Prognose bei Jungen < 10 Jahre mit GCT des Hodens ist exzellent; allein durch hohe inguinale Tumororchiektomie werden ~ 80% geheilt. Bei YST Stadium IA ist w&w auch bei nicht protokollgemäßer Operation möglich und sicher, sofern eine adäquate Nachsorge – inklusive Tumormarker – gewährleistet ist.

Key words

germ cell tumor - yolk sac tumor - teratoma - watch and wait - testicular tumor - tumororchiectomy

Schlüsselwörter

Keimzelltumor - Dottersacktumor - Teratom - Nachsorge - Hodentumor - Tumororchiektomie
 

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