Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor

Abstract

Central nervous system primitive neuroectodermal tumors (CNS PNETs) predominantly occur in children and rarely in adults. Because of the rarity of this tumor, its outcomes and prognostic variables are not well characterized. The purpose of this study was to evaluate clinical outcomes and prognostic factors for children and adults with CNS PNET. The records of 26 patients (11 children and 15 adults) with CNS PNET from 1991 to 2011 were reviewed retrospectively. Disease-free survival (DFS) and overall survival (OS) were estimated with the Kaplan-Meier method, and relevant prognostic factors were analyzed. For the cohort, both the 5-year DFS and the OS were 46 %. For pediatric patients, the 5-year DFS was 78 %; for adult patients, it was 22 % (P = 0.004). Five-year OS for the pediatric and adult patients was 67 and 33 %, respectively (P = 0.07). With bivariate analysis including chemotherapy regimen (high dose vs. standard vs. nonstandard) or risk stratification (standard vs. high) and age, the increased risk of disease recurrence in adults persisted. A nonsignificant tendency toward poorer OS in adult patients relative to pediatric patients also persisted. High-dose chemotherapy with stem cell rescue was associated with a statistically significant improvement in OS and a tendency toward improved DFS, although the findings were mitigated when the effect of age was considered. Local recurrence was the primary pattern of treatment failure in both adults and children. Our results suggest that adult patients with CNS PNETs have inferior outcomes relative to the pediatric cohort. Further research is needed to improve outcomes for CNS PNET in populations of all ages.

Embryonal Tumors of the Central Nervous System: From the Radiologic Pathology Archives

“…With regard to CNS PNETs as a group, clinical outcomes are worse than for medulloblastomas, with 5-year relative survival of 46% versus 73% based on Surveillance, Epidemiology, and End Results (SEER) data from 2000-2013 (29% for AT/RT) (5). Poor prognostic factors include CSF dissemination/metastases and either young (age < 3 years) or adult presentation; typical treatment is trimodal with maximum safe resection, adjuvant chemotherapy, and craniospinal irradiation (often withheld under 3 years of age owing to its neurotoxicity) (90). In one study of outcomes in 11 children with ependymoblastoma/ETMR registered to multicenter brain tumor trials between 1994 and 2006, there were four survivors, all of whom received craniospinal radiation therapy and/or high-dose chemotherapy with autologous stem cell rescue (91).…”

A primitive neuroectodermal tumor in an adult

“…[ 1 ] Central PNET mostly occurs in children and accounts for only 1% of primary CNS tumors. Ventricular primary PNET is extremely rare; to the best of our knowledge, only 4 cases of recurrence have been reported in the English literatures [ 2 – 5 ] In this study, we present the first case of the PNET occurring in a 36-year-old man in his ventricles. The standard treatment for CNS PNETs consists of major surgical resection when feasible, followed by radiotherapy and chemotherapy, but survival rates are consistently lower in spite of multimodal therapy.…”

Embryonale Tumoren des Zentralnervensystems

“…Die Behandlung ist typischerweise trimodal mit maximal sicherer Resektion, adjuvanter Chemotherapie und kraniospina-ler Bestrahlung. Auf Letztere wird bei Kindern unter 3 Jahren angesichts ihrer Neurotoxizität oft verzichtet [90]. In einer Auswertung der Verläufe bei 11 Kindern mit Ependymoblastom bzw.…”