Adult Medulloblastoma: Updates on Current Management and Future Perspectives

Abstract

Medulloblastoma (MB) is a malignant embryonal tumor of the posterior fossa belonging to the family of primitive neuro-ectodermic tumors (PNET). MB generally occurs in pediatric age, but in 14–30% of cases, it affects the adults, mostly below the age of 40, with an incidence of 0.6 per million per year, representing about 0.4–1% of tumors of the nervous system in adults. Unlike pediatric MB, robust prospective trials are scarce for the post-puberal population, due to the low incidence of MB in adolescent and young adults. Thus, current MB treatments for older patients are largely extrapolated from the pediatric experience, but the transferability and applicability of these paradigms to adults remain an open question. Adult MB is distinct from MB in children from a molecular and clinical perspective. Here, we review the management of adult MB, reporting the recent published literature focusing on the effectiveness of upfront chemotherapy, the development of targeted therapies, and the potential role of a reduced dose of radiotherapy in treating this disease.

Unusual manifestation of cerebellopontine angle medulloblastoma with tinnitus and sensorineural hearing loss

“…The lesion presents moderate to strong contrast after intravenous administration of contrast agents. It is suggested to achieve diffusion-weighted images on which medulloblastoma appears with restricted diffusion because of the high cellularity of this tumor 43 . In our patient, the magnetic resonance imaging showed a nodular lesion with tissular isointense signal on T1 and T2, slightly hyperintense FLAIR localised on the left side of the Luschka foramen, with extension to the fourth ventricle and left cerebellar peduncle (Figure 1).…”

“…On CT, medulloblastoma may appear as a well-defined, hyperdense mass with edema around, on the midline in the cerebellar vermis and frequently compressing the fourth ventricle with obstructive hydrocephalus. Also, CT imaging may be useful in detecting mineralization and haemorrhage areas 43,45,46 .…”

“…Medulloblastoma stages (Chang classification) is based on pre-operative radiological characteristics and intraoperative ones to determine T stage and M stage 43 (Table 3).…”

“…If immediate surgery is not achievable, in hydrocephalus, at least an external ventricular drain is required. Before surgery, vasogenic tumour oedema should be decreased using corticosteroids 43,67,68 .…”

Immune microenvironment of medulloblastoma: The association between its molecular subgroups and potential targeted immunotherapeutic receptors

“…The standard protocols included different chemotherapeutic agents with long-term or maintenance dose-related regime including ifosfamide, etoposide, methotrexate, cisplatin, and cytarabine, lomustine, and vincristine[ 30 ]. The maintenance regimen has improved the overall survival compared to the sandwich approach among patients with M0 or M1 disease[ 30 , 31 ]. Nonetheless, the most frequent and current treatment strategy includes risk-adapted radiotherapy followed by 4 cycles of cyclophosphamide, and a high dose of chemotherapy such as cisplatin, vincristine, followed by autologous stem cell transplantation.…”