The incidence of brainstem primitive neuroectodermal tumors of childhood based on SEER data

Citation:
Chamdine, O., Elhawary G. A. S., Alfaar A. S., & Qaddoumi I. (2018).  The incidence of brainstem primitive neuroectodermal tumors of childhood based on SEER data. Child's Nervous System. 34(3), 431 - 439.

Abstract:

PURPOSE Incidence of BS primitive neuroectodermal tumors (BS-PNET) in children is not reported to date. Our main objectives were to estimate the incidence and report the outcome of BS-PNET in children. METHODS Data were collected using the Surveillance Epidemiology and End Results cancer registry. RESULTS From 1973 to 2013, we identified 83 pediatric patients (aged 0-21 years). Patients were divided into two age groups (0-3 years and 4-21 years). Median overall survival was 53 months. Patients in the older age group had a significant survival advantage (P < 0.001), as did those who received three modalities of therapy (surgery, chemotherapy, and radiation therapy) (P < 0.001) and patients with gross or subtotal tumor resection (P < 0.001). CONCLUSIONS This study presents the first estimate of incidence and the largest cohort of pediatric BS-PNETs to date. A high index of suspicion of BS-PNET in similar cases is crucial for diagnosis, treatment, and outcome.

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