Relative survival of childhood and adult medulloblastomas and primitive neuroectodermal tumors (PNETs)

Abstract

BACKGROUND: Medulloblastomas are 1 of the most common brain tumors in children but can affect individuals of all ages. For this report, the author investigated the impact of medulloblastomas/primitive neuroectodermal tumors (PNETs) on the US population with a focus on age differences. METHODS: Data from the Surveillance, Epidemiology, and End Results (SEER) database were used to describe cumulative relative survival (CRS) using crude, period, and longitudinal period approaches for patients diagnosed with all medulloblastoma subtypes and PNETs. CRS estimates were obtained using SEER expected mortality data and the Ederer II method for expected survival estimation. These data were applied to the construction of rational follow-up scheduling protocols. RESULTS: The 5-year period CRS for all patients who were followed between 2001 and 2006 was 69%. Adults had a worse overall prognosis, but this difference in excess hazard rates appeared only after 4 years of follow-up. Furthermore, the 5-year and 10-year CRS has improved a minimum of 11% in children, adolescents, and adults over the past 25 years. CONCLUSIONS: The survival difference between children, adolescents, and adults with medulloblastomas and PNETs depended on the length of follow-up, which was described in this report as an age-by-follow-up interaction and observed as a ''fork'' on Kaplan-Meier curves. Differences in survival between children and adults emerged only 4 years after diagnosis, and adults fared worse. There has been significant improvement in survival from medulloblastomas/PNETs since the late 1970s and early 1980s. Cancer 2012;118:1313-

Incidence and survival of children and young people with central nervous system embryonal tumours in the North of England, 1990–2013

“…In our study we did not find a difference in survival between the TYA age group and children aged 5-9 years. We did however observe a doubling of the risk of death for those aged under 5 years, consistent with other studies (3,5,(15)(16)(17)(18)(19)31) due to treatment strategies for infants and young children that defer or avoid radiation (7).…”

“…We compared our survival rates to those from other international studies and found childhood medulloblastoma rates are similar to those in France (16) and Sweden (15) (17). In our study we did not find a difference in survival between the TYA age group and children aged 5-9 years.…”

“…Central nervous system (CNS) tumours are the second most common site of neoplasms diagnosed in children, accounting for approximately one quarter of all childhood cancers (0-14 years), (1) and 14% of tumours diagnosed in teenagers and young adults (TYA) (15)(16)(17)(18)(19)(20)(21)(22)(23)(24) years) (2). They are the most common cause of cancer related death in children and TYA (1,2).…”

“…Population-based studies which have analysed survival rates separately for medulloblastoma and CNS PNET have shown that children diagnosed with medulloblastoma have better survival outcomes than those with CNS PNET (5-year survival rates range between 58-69% for medulloblastoma vs. 27-47% for CNS PNET) (3,(15)(16)(17)(18). The incidence of childhood CNS embryonal tumours increased in Europe between 1978 and 1997 (19).…”

Incidence, Trends, and Survival of Children With Embryonal Tumors

“…Male gender has been identified as a risk factor for medulloblastomas, PNETs, AT/RTs, neuroblastomas, and hepatoblastomas in this and other studies. [14][15][16][17][18] The exceptions to the male predominance were Wilms tumors, as shown by others as well, 19 and ganglioneuroblastomas. A strongly diverging median age at diagnosis between the genders was detected for PNET and medulloepitheliomas, which may reflect greater biological differences between the genders for these tumors than for the others.…”