Introduction: Medulloblastoma (MB) is the most common brain malignant tumor in children, conversely it is less frequent among adult population, accounting less 1% of adult intracranial tumors. Adult MB shows different characteristics and outcomes than pediatric MB. Areas covered: This article compares epidemiology, pathology, radiologic features, prognosis, risk stratification, molecular genetics, current and future treatment strategies of adult and pediatric medulloblastoma (MB). Expert opinion: The correct neuro-radiological diagnosis of adult MB remains a challenge, because of the lack of consensus about the choice of chemotherapeutic agents. Pediatric MB protocols have often limited application in adults, because of their less tolerance to chemotherapy. The added difficulty derives from the absence of a systematic description of the management and outcome of the relapses. Sonic Hedgehog Homolog (SHH) MB represents about 30% of all MB and is the first subgroup to be targeted by a molecularly focused therapy. Despite significant advances have been achieved in the development of SHH inhibitors, still the persistence of primary and acquired drug resistance represents an unresolved challenge. Prospective studies should be performed with international and multidisciplinary collaboration to guide future treatment choices.
Capozza, M. A., Trombatore, G., Triarico, S., Mastrangelo, S., Attina, G., Maurizi, P., Ruggiero, A., Adult medulloblastoma: an overview on current and future strategies of treatment, <<EXPERT OPINION ON ORPHAN DRUGS>>, 2019; 7 (9): 383-389. [doi:10.1080/21678707.2019.1663170] [https://hdl.handle.net/10807/237022]
Adult medulloblastoma: an overview on current and future strategies of treatment
Mastrangelo, Stefano;Maurizi, Palma;Ruggiero, Antonio
2019
Abstract
Introduction: Medulloblastoma (MB) is the most common brain malignant tumor in children, conversely it is less frequent among adult population, accounting less 1% of adult intracranial tumors. Adult MB shows different characteristics and outcomes than pediatric MB. Areas covered: This article compares epidemiology, pathology, radiologic features, prognosis, risk stratification, molecular genetics, current and future treatment strategies of adult and pediatric medulloblastoma (MB). Expert opinion: The correct neuro-radiological diagnosis of adult MB remains a challenge, because of the lack of consensus about the choice of chemotherapeutic agents. Pediatric MB protocols have often limited application in adults, because of their less tolerance to chemotherapy. The added difficulty derives from the absence of a systematic description of the management and outcome of the relapses. Sonic Hedgehog Homolog (SHH) MB represents about 30% of all MB and is the first subgroup to be targeted by a molecularly focused therapy. Despite significant advances have been achieved in the development of SHH inhibitors, still the persistence of primary and acquired drug resistance represents an unresolved challenge. Prospective studies should be performed with international and multidisciplinary collaboration to guide future treatment choices.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.