Incidence and trends in pediatric malignancies medulloblastoma/primitive neuroectodermal tumor: A SEER update

Authors

  • Dawn Elizabeth McNeil MD,

    Corresponding author
    1. National Cancer Institute, Division of Cancer Epidemiology and Genetics, Genetic Epidemiology Branch, Rockville, Maryland
    • Genetic Epidemiology Branch, Division of Cancer Epidemiology and Genetics, NCI/NIH/EPS room 7125, 6120 Executive Boulevard, MSC 7236, Bethesda, MD 20892-7236.
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  • Timothy R. Coté MD, MPH,

    1. National Cancer Institute, Division of Cancer Control and Population Sciences, Cancer Statistics Branch, Rockville, Maryland
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  • Limin Clegg PhD,

    1. National Cancer Institute, Division of Cancer Control and Population Sciences, Cancer Statistics Branch, Rockville, Maryland
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  • Lucy Balian Rorke MD

    1. Children's Hospital of Philadelphia, Department of Pathology-Neuropathology, Philadelphia, Pennsylvania
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Abstract

Background

It has been suggested that cerebellar medulloblastoma (M) and primitive neuroectodermal tumors (PNET) arising elsewhere in the nervous system, represent a single entity (M/PNET), although this concept is controversial. Cancer registries permit population-based description of cases reported as medulloblastoma, those reported as PNET and description of the aggregate, M/PNET.

Procedure

We reviewed the 768 cases of M/PNET (633 diagnosed medulloblastoma and 135 diagnosed PNET) among persons under 20 years of age in the National Cancer Institute's Surveillance Epidemiology and End Results (SEER) database.

Results

The incidence of M/PNET rose 23%, from 4 per 106 person-years in 1973–77 to 4.9 per 106 person-years in 1993–98. Cases reported as PNET were more likely than those reported as medulloblastoma to be supratentorial (30.4% vs. 1.9%, P < 0.001) and to be female (42.2% vs. 35.4%, P = 0.13). The difference in 5-year survival between the 600 children with infratentorial medulloblastoma vs. the 49 children with infratentorial PNET was not statistically significant (55% vs. 43%). Regardless of reporting diagnosis, survival was poorer among children age 0–3 years and those with supratentorial tumors. Children diagnosed in the more recent period from 1985–1998 had a longer median survival than children diagnosed in 1973–84 (4.9 years vs. 10 years, P < 0.05). Rates were 42% higher among Whites compared to Blacks (4.5/106 person-years vs. 3.1/106 person-years, P < 0.01).

Conclusions

We found M/PNET is increasing in incidence and more frequent among Whites. Given that medulloblastoma and PNET are histologically identical and have similar epidemiologic profiles, future studies should provide analyses that combine these entities. Med Pediatr Oncol 2002;39:190–194. Published 2002 Wiley-Liss, Inc.

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