Patterns of care in adult medulloblastoma: results of an international online survey

Abstract

The literature on medulloblastoma in adults is generally limited to case reports and retrospective series, and there is no accepted standard of care. The Cooperative Trials Group for Neuro-Oncology (COGNO) sought to determine the range and consistency of clinicians' approaches to management as a basis for future trials. We aimed to identify current treatment strategies for adult medulloblastoma through an online survey launched at the 2012 Society of Neuro-Oncology meeting and by email invitation. Clinicians who had treated at least one adult patient with medulloblastoma, primitive neuroectodermal tumor (PNET), or pineoblastoma in the preceding year were asked about their most recent patient and invited to discuss their approach to a typical clinical scenario. Between November 2012 and January 2013, 45 clinicians (11 medical oncologists, 8 radiation oncologists, 5 pediatric oncologists, and 21 others) from Australia (24), United States (3), Europe (4) and other countries (14) completed the survey. Responding clinicians had treated 54 cases in the past 12 months. The most common histological type was medulloblastoma (64%), then PNET (20%). Most patients were male (68%), and had high-risk disease (65%). Complete surgical resection in 56 and 32% had molecular testing. Radiotherapy was predominantly cranio-spinal (92%) and given mostly post-resection (80%). Combination chemotherapy was more common than single-agent chemotherapy. The choice of chemotherapy varied considerably. There is substantial variation in the treatment of adult medulloblastoma, most pronounced in the choice of chemotherapeutic agents, highlighting the need for further collaborative research to guide evidence-based treatment strategies.

Clinical and mutational profiles of adult medulloblastoma groups

“…Due to their rarity, prospective trials on adult medulloblastomas are limited [40]. The management of adult medulloblastomas is adapted from paediatric protocols, often resulting in dose-limiting toxicities [12].…”

Whole Lung Irradiation in Adults with Metastatic Ewing Sarcoma: Practice Patterns and Implications for Treatment

“…Practice variation across adult and pediatric providers has been reported in other soft tissue cancers as well. For example, a survey of adult and pediatric oncologists on the treatment of adult medulloblastoma patients similarly demonstrated substantial variation in practice, particularly with the choice of chemotherapeutic agents, likely reflecting the limited data available relevant to older adult population [ 22 ]. Likewise, population studies have demonstrated less aggressive protocols for diagnosis, staging, and treatment for adults with Wilms' Tumor relative to their pediatric counterparts and cited this less aggressive approach as an explanation for inferior survival outcomes among adults [ 23 ].…”

Molecular stratifications, biomarker candidates and new therapeutic options in current medulloblastoma treatment approaches

“…Due to the relatively low incidence of adult MBs, there are no randomized clinical trials upon which to base treatment recommendations. After maximal safe resection, adult patients are treated with normofractionated CSI of 30-36 Gy followed by a tumor bed boost to 54-55.8 Gy and occasional chemotherapy, particularly for high-risk disease, with unknown outcomes [45,46]. However, such conventional radiation therapy is associated with progressive neurotoxicity, leading to the degradation of the quality of life.…”