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

Abstract

Background:

In 1982 the GPOH opened the 1^st protocol for germ cell tumors (GCTs) of the testis (MAHO 82). Here the results of the 5^th 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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