Survival and recurrence factors in adult medulloblastoma: The M.D. Anderson Cancer Center experience from 1978 to 1998

Abstract

Medulloblastoma is a rare adult primary brain tumor for which limited retrospective studies are available to elucidate natural history or to guide therapy. A retrospective chart and imaging review of adult patients (aged >18 years) with medulloblastoma was performed to identify survival and prognostic factors. Fifty-seven patients were evaluated at the University of Texas M.D. Anderson Cancer Center from 1978-1998. Statistical analysis of prognostic factors and overall survival was performed for a subgroup of 28 patients who were followed exclusively at our institution from the time of diagnosis until death or last follow-up. These 28 patients had an overall survival of 91% at 3 years and 84% at 5 years, whereas median survival was not reached after a median follow-up of 168 weeks (range, 9-602 weeks). Progression-free survival for all patients was 68% at 3 years and 62% at 5 years, and was not statistically different between poor- and standard-risk patients. Univariate analysis of clinical features, such as age, sex, extent of local disease, extent of resection, and use of adjuvant chemotherapy, did not identify any prognostic variables for survival among the 28 patients. Patterns of recurrence revealed that the posterior fossa was the most common site (56%), followed by bone marrow (25%). Adult medulloblastoma appears to have a favorable prognosis after treatment with maximally surgically feasible resection followed by craniospinal irradiation. Optimal treatment remains to be clarified, as both standard-risk and poor-risk patients have prolonged disease-free survival. The marked difference between survival and progression-free survival suggests that salvage therapy, usually with combination chemotherapy in this cohort of patients, is of benefit. More formal analysis of the survival benefit was not possible, however, because of the small number of patients treated at recurrence with any one therapeutic regimen.

Long-term Outcomes and Role of Chemotherapy in Adults With Newly Diagnosed Medulloblastoma

“…Anderson Cancer Center, 28 patients had a 5-year OS of 84%. 7 A large, updated French multicenter experience involving 253 patients had a reported 5-year OS of 72%. 20 Our results appear to be similar to those in that report, as our patients had a 5-year OS of 74% and a 5-year DFS of 59%.…”

“…However, other authors have not found a difference in outcome by risk stratification. 7 Some series have demonstrated the prognostic significance of M stage or extent of surgery. 1,3,21 Riffaud et al 21 defined high risk as having less than a GTR or as having disseminated disease.…”

“…2-5 Some institutions have reported 5-year overall survival (OS) for adults as high as 84%, whereas other analyses, including a recent update from the Surveillance, Epidemiology and End Results (SEER) program within the National Cancer Institute, showed a 5-year OS of only 65%. 6,7 The data have also varied about the effects of residual disease, metastatic disease, adjuvant chemotherapy, histologic subtype, and tumor location on patient outcomes. 1-3,8-11 …”

Central Nervous System Cancers

“…Headache, ataxia, and nausea are commonly observed symptoms. 110 All PNETs of the brain are WHO grade IV, because they are invasive and rapidly growing. They also have the tendency to disseminate through the cerebrospinal fluid (CSF).…”

“…Complete resection can be achieved in half of the patients 110,113,114 and is associated with improved survival. 113,115 In addition, surgical placement of a ventriculoperitoneal shunt can be used to treat hydrocephalus.…”