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DOI: 10.1055/s-2005-836505
© Georg Thieme Verlag Stuttgart · New York
Supratentorieller primitiver neuroektodermaler Tumor: Erfahrungen in einem Zentrum im Vergleich zur Literatur
Supratentorial Primitive Neuroectodermal Tumor: A Single Center Experience and Comparison with the LiteraturePublikationsverlauf
Publikationsdatum:
27. April 2005 (online)
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Zusammenfassung
Ein supratentorieller, primitiver neuroektodermaler Tumor (stPNET) ist ein seltener hochmaligner Tumor. Wir betreuten innerhalb von drei Jahren sechs Kinder mit stPNET. Bei vier der sechs Kinder gelang eine makroskopisch vollständige Exstirpation. Alle erhielten kraniospinale Bestrahlung und Chemotherapie nach dem HIT-91 Therapieoptimierungsschema. Die beiden Kinder mit nicht vollständig entferntem Tumor starben nach 7 und 10 Monaten in Folge Tumorprogression. Bei 2 der 4 Kinder mit komplett entferntem Tumor trat jeweils nach 8 Monaten ein Lokalrezidiv auf. Sie starben nach 14 bzw. 18 Monaten. Bei einem Kind trat nach 12 Monaten eine diffuse meningeale Aussaat auf. Trotz (Hochdosis-)Chemotherapie systemisch und Mafosfamid intraventrikulär starb der Patient nach vorübergehender Remission 21 Monate nach Diagnose in Folge des Tumors. Nur eines der 6 Kinder lebt in kompletter Remission (7 Jahre). In Übereinstimmung mit der internationalen Literatur haben Kinder mit stPNET eine schlechte Prognose, wobei im Rahmen multimodaler Therapiekonzepte zunehmend von einem verbesserten Langzeitüberleben berichtet wird. Aufgrund der offensichtlichen Heterogenität der Tumoren wird eine molekularbiologische Aufarbeitung an frischem Tumormaterial hilfreich sein, um in kooperativen Protokollen eine Therapieoptimierung zu erreichen.
Abstract
Supratentorial primitive neuroectodermal tumors (stPNETs) are malignant tumors. We saw within three years six children with stPNETs. In four of the six children radical resection could be achieved. All had craniospinal irradiation and chemotherapy according to the HIT-91 protocol. The two children with incomplete resection died due to tumor progression after 7 and 10 months. Two of the 4 children with complete tumor resection had local relapses 8 months after diagnosis and died after 14 and 18 months. One child had a diffuse meningeal relapse 12 months after diagnosis. Despite (high-dose) systemic chemotherapy and intraventricular mafosfamide, he died 21 months after diagnosis due to tumor although remission could be achieved. Only one child is still in remission 86 months after diagnosis.
Schlüsselwörter
supratentorieller primitiver neuroektodermaler Tumor - Therapie - Prognose
Key words
supratentorial primitive neuroectodermal tumor - therapy - prognosis
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Dr. Irene Schmid
Kinderklinik und Kinderpoliklinik im Dr. von Haunerschen Kinderspital · Ludwig-Maximilians-Universität München
Lindwurmstr. 4
80337 München
Telefon: 49/89/51 60 28 42
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eMail: Irene.Schmid@med.uni-muenchen.de