Curative treatment for central nervous system medulloepithelioma despite residual disease after resection. Report of two cases treated according to the GPHO Protocol HIT 2000 and review of the literature.
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Curative treatment for central nervous system medulloepithelioma despite residual disease after resection. Report of two cases treated according to the GPHO Protocol HIT 2000 and review of the literature. / Müller, Klaus; Zwiener, Isabella; Welker, Helmut; Maass, Eberhard; Bongartz, Rudolf; Berthold, Frank; Pietsch, Torsten; Warmuth-Metz, Monika; von Bueren, André; Rutkowski, Stefan.
In: STRAHLENTHER ONKOL, Vol. 187, No. 11, 11, 2011, p. 757-762.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Curative treatment for central nervous system medulloepithelioma despite residual disease after resection. Report of two cases treated according to the GPHO Protocol HIT 2000 and review of the literature.
AU - Müller, Klaus
AU - Zwiener, Isabella
AU - Welker, Helmut
AU - Maass, Eberhard
AU - Bongartz, Rudolf
AU - Berthold, Frank
AU - Pietsch, Torsten
AU - Warmuth-Metz, Monika
AU - von Bueren, André
AU - Rutkowski, Stefan
PY - 2011
Y1 - 2011
N2 - Medulloepithelioma of the central nervous system (CNS) is an uncommon primitive neuroectodermal tumor (PNET) usually occurring in early childhood. It is characterized by highly malignant behavior with a propensity for progression, recurrence, and dissemination despite intensive therapy. Due to its rarity, the optimal management is still unknown. However, gross total resection (GTR) has been considered crucial to achieve cure. In this article, the authors report on 2 cases of CNS medulloepithelioma in which long-term survival (more than 6 years) could be achieved despite evidence of, or suspected postoperative residual disease with an otherwise dismal prognosis.The patients were treated according to different strata of the protocol for primitive neuroectodermal tumors (PNET) of the German-Austrian multicenter trial of the German Society for Pediatric Oncology and Hematology (GPOH) for childhood brain tumors (HIT 2000). Treatment included postoperative hyperfractionated radiotherapy of the craniospinal axis followed by a boost to the tumor site in combination with chemotherapy. A review of the 2 reported and 37 previously published cases confirmed GTR and older age as positive prognostic factors.
AB - Medulloepithelioma of the central nervous system (CNS) is an uncommon primitive neuroectodermal tumor (PNET) usually occurring in early childhood. It is characterized by highly malignant behavior with a propensity for progression, recurrence, and dissemination despite intensive therapy. Due to its rarity, the optimal management is still unknown. However, gross total resection (GTR) has been considered crucial to achieve cure. In this article, the authors report on 2 cases of CNS medulloepithelioma in which long-term survival (more than 6 years) could be achieved despite evidence of, or suspected postoperative residual disease with an otherwise dismal prognosis.The patients were treated according to different strata of the protocol for primitive neuroectodermal tumors (PNET) of the German-Austrian multicenter trial of the German Society for Pediatric Oncology and Hematology (GPOH) for childhood brain tumors (HIT 2000). Treatment included postoperative hyperfractionated radiotherapy of the craniospinal axis followed by a boost to the tumor site in combination with chemotherapy. A review of the 2 reported and 37 previously published cases confirmed GTR and older age as positive prognostic factors.
KW - Humans
KW - Female
KW - Adolescent
KW - Survivors
KW - Child
KW - Follow-Up Studies
KW - Dose Fractionation
KW - Radiotherapy Dosage
KW - Neoadjuvant Therapy
KW - Cranial Irradiation
KW - Antineoplastic Combined Chemotherapy Protocols/therapeutic use
KW - Cerebellar Neoplasms/drug therapy/mortality/radiotherapy/surgery
KW - Chemoradiotherapy, Adjuvant
KW - Neoplasm, Residual/drug therapy/mortality/radiotherapy/surgery
KW - Neuroectodermal Tumors, Primitive/drug therapy/mortality/radiotherapy/surgery
KW - Skull Base Neoplasms/drug therapy/mortality/radiotherapy/surgery
KW - Humans
KW - Female
KW - Adolescent
KW - Survivors
KW - Child
KW - Follow-Up Studies
KW - Dose Fractionation
KW - Radiotherapy Dosage
KW - Neoadjuvant Therapy
KW - Cranial Irradiation
KW - Antineoplastic Combined Chemotherapy Protocols/therapeutic use
KW - Cerebellar Neoplasms/drug therapy/mortality/radiotherapy/surgery
KW - Chemoradiotherapy, Adjuvant
KW - Neoplasm, Residual/drug therapy/mortality/radiotherapy/surgery
KW - Neuroectodermal Tumors, Primitive/drug therapy/mortality/radiotherapy/surgery
KW - Skull Base Neoplasms/drug therapy/mortality/radiotherapy/surgery
M3 - SCORING: Journal article
VL - 187
SP - 757
EP - 762
JO - STRAHLENTHER ONKOL
JF - STRAHLENTHER ONKOL
SN - 0179-7158
IS - 11
M1 - 11
ER -
