Intracranial Germ Cell Tumors: A Comprehensive Update of the European Data

G. Calaminus; M. Bamberg; M. C. Baranzelli; Y. Benoit; L. Cordero di Montezemolo; F. Fossati-Bellani; H. Jürgens; H. J. Kühl; H. G. Lenard; M. Lo Curto; J. R. Mann; C. Patte; A. Pearson; G. Perilongo; D. Schmidt; R. Schober; U. Göbel

doi:10.1055/s-2008-1071577

Neuropediatrics 1994; 25(1): 26-32
DOI: 10.1055/s-2008-1071577

Original article

Intracranial Germ Cell Tumors: A Comprehensive Update of the European Data

G. Calaminus^1* , M. Bamberg^2* , M. C. Baranzelli³⁺ , Y. Benoit⁴ , L. Cordero di Montezemolo^5# , F. Fossati-Bellani⁶ , H. Jürgens^7* , H. J. Kühl^8* , H. G. Lenard¹ , M. Lo Curto^9# , J. R. Mann^10o , C. Patte¹¹⁺ , A. Pearson^12o , G. Perilongo^13# , D. Schmidt^14* , R. Schober¹⁵ , U. Göbel^1*

¹Children's Hospital, University of Düsseldorf, Germany
²Clinic for Radiotherapy, University of Tübingen, Germany
³Dept. of Pediatr. Oncol., Centre Oscar Lambret, Lille, France
⁴Dept. of Pediatr. Oncol., University of Gent, Belgium
⁵Dept. of Pediatr. Oncol., Hospital Regina Margerita, Torino, Italy
⁶Dept. of Pediatr. Oncol, Istituto Nazionale dei Tumori, Milano, Italy
⁷Dept. of Pediatr. Hematol./Oncol., University of Münster, Germany
⁸Dept. of Pediatr. Hematol./Oncol., University of Würzburg, Germany
⁹Dept. of Pediatr. Oncol., Clinica Pediatrica I, Palermo, Italy
¹⁰Dept. of Pediatr. Oncol., Children's Hospital, Birmingham, UK
¹¹Dept. of Pediatr. Oncol., Institut Gustave-Roussy, Villejuif, France
¹²Dept. of Pediatr. Oncol., University of Newcastle-upon-Tyne, UK
¹³Dept. of Pediatr. Oncol., Università di Pauda, Italy
¹⁴Institute for Paidopathology, University of Kiel, Germany
¹⁵Institute for Neuropathology, University of Düsseldorf, Germany

* Representing the German GPOHo Representing the U.K. Children's Cancer Study Group+ Representing the French SFOP# Representing the Italian AIEOP

Abstract

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Abstract

Intracranial germ cell tumors are rare tumor entities in childhood and adolescents. Extra- and intracranial germ cell tumors are identical in their histologic pattern and occur in preferential midline localizations such as the pineal and the suprasellar region. Germ cell neoplasms show increasing incidence rates over the last 30 years.

The majority of intracranial germ cell neoplasms are germinomas. About 90% of the patients with pure germinomas can be salvaged by radiotherapy alone according to modern protocols. Non-germinomatous malignant CNS-germ cell tumors are considered to have a poor prognosis. In order to improve the survival of patients affected by these tumors different treatment approaches adding chemotherapy to conventional surgery and radiotherapy have been initiated by various study groups throughout the world.

Due to the rarity of these neoplasms only a very limited number of patients has been enrolled in each study. In 1993 an international working group on these tumors was established by the International Society of Pediatric Oncology (SIOP).

The data of the national groups of France, Italy, the United Kingdom and Germany are reviewed to compare the results of the different protocols, to analyse the specific problems of therapy of these neoplasms and to discuss further treatment possibilities in respect to established protocols.

For germinomas the data of 87 patients are evaluated. The life-table analysis shows an event-free survival (EFS) of 90% for patients who received radiotherapy only (46/87), which was craniospinal irradiation in most of the cases, whereas in patients who were treated with a combination of platinum-based chemotherapy and irradiation (30/87) the event-free survival was 92%. For nongerminomas the combination of platinum-based chemotherapy and craniospinal radiation leads to encouraging results. In respect to the cumulative platinum dosage the life-table analysis shows an EFS of 86% for patients with 400 mg/m² platinum (17/49). In patients treated with 200 mg/m² platinum (10/49) the EFS decreases to 56%.

Key words

CNS germ cell tumors - Combination therapy - Irradiation - Platinum

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