Verbesserte Prognose intracranialer Keimzelltumoren durch intensivierteTherapie: Ergebnisse des Therapieprotokolls MAKEI 89

Abstract

Germ cell tumors of the central nervous system are histological identical to the extracranial tumor sites. According to the localisation germ cell tumors of the CNS are different in symptoms, diagnostic approaches, kind and location of metastases and stratification of therapy. Since 1986 patients with intracranial germ cell tumors are registered in the ongoing study for non-testicular germ cell tumors (MAKEI) of the German Society of Pediatric Oncology and Hematology, and are treated in accordance to therapy guidelines for extracranial sites. In MAKEI 89 therapy strategy was revised with a reduction of radiotherapy and an increased cumulative cisplatinum dose from 200 mg/m2 to 400 mg/m2. Patients with germinoma receive after histologic diagnosis radiotherapy consisting of 30 Gy craniospinal irradiation and 15 Gy tumorboost. Malignant non-germinoma receive after diagnosis by tumor marker in CSF and/or serum 2 courses bleomycin 15 mg/m2 day 1-3, Etoposide 150 mg/m2 day 1 + 2 and cisplatinum 20 mg/m2 days 4-8 (BEP), continued by 2 courses Vinblastine 3 mg/m2 day 1 + 2, Ifosfamide 1500 mg/m2 days 1-5 and cisplatinum 20 mg/m2 days 1-5 (VIP), followed by 30 Gy craniospinal irradiation and 20 Gy tumor boost. In teratoma first line therapy is complete resection. In incomplete resected cases adjuvant chemotherapy according to histological grading is administered. Until 31st January, 1993 101 patients (pts) were registered, containing 69 protocol pts. Diagnosis in protocol pts was teratoma in 8 cases, 2 pts died postnatal because of extended disease, 2/8 pts relapsed, but were salvaged by chemotherapy. 40 pts offered germinomas.(ABSTRACT TRUNCATED AT 250 WORDS)

Combined treatment modality for intracranial germinomas: results of a multicentre SFOP experience

“…The low progression rate in our report is qualified by a short follow-up in a disease where late recurrences are not uncommon (Ono et al, 1994). However, the 42-month follow-up for the whole group does not differ from other large prospective series (Gobel et al, 1993;Balmaceda et al, 1996). Chemotherapy in our experience did allow avoidance of prophylactic craniospinal radiotherapy in patients with localized germinomas.…”

“…Craniospinal radiotherapy is still the standard treatment for many centres, but some radiotherapists favour local radiotherapy and oncologists are increasingly interested in giving chemotherapy in order to reduce either the dose or the extent of radiotherapy. The SFOP group has prospectively explored the feasibility of a combined approach that can be compared to other prospective multicentre trials such as the MAKEI trials (Gobel et al, 1989(Gobel et al, , 1993 and the First International Central Nervous System Germ Cell Tumor Study (Balmaceda et al, 1996).…”

Aktuelle und zukünftige Strategien in der interdisziplinären Therapie von Medulloblastomen, supratentoriellen PNET und intrakraniellen Keimzelltumoren im Kindesalter

“…Mit alleiniger Strahlenbehandlung der gesamten Neuroachse konnte in der MAKEI-89-Studie bei 49 Patienten eine rezidivfreie 5-Jahres-Überlebensrate von 92% (von fünf Ereignissen lagen zwei Fälle eines metachronen Keimzelltumors, zwei Fälle einer Metastasierung außerhalb des Zentralnervensystems und nur ein Rückfall innerhalb des Zentralnervensystems vor) erzielt werden gegenüber 34% nach kombinierten Radiochemotherapien oder alleinigen Operationen [9,19,40] (Abbildung 5). Die Patienten mit Rückfällen konnten einer erfolgreichen Chemotherapie zugeführt werden [3,9,19].…”

“…Anschließend erfolgt die Strahlentherapie. Die 2-Jahres-Überlebensraten (krankheitsfrei) konnten durch kombinierte Behandlung (Chemotherapie/Strahlenbehandlung des Liquorraums) von nur 6% (Operation/Strahlenbehandlung) auf über 80% gesteigert werden [19,40]. Die verabreichte Dosis Cisplatin scheint ausschlaggebend für das therapeutische Resultat zu sein.…”

Demaskierung einer sekundären Nebennieren-rindeninsuffizienz und eines Diabetes insipidus centralis durch eine hochfieberhafte akute Hantaan-Virus-Infektion

“…Die Erkrankung tritt ª zwischen dem siebten und 20. Lebensjahr auf, Jungen erkranken doppelt so h~iufig wie Mfidchen [5]. Die Diagnose gelingt meist erst nach Auftreten der Leitsymptome [9].…”