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. 2018 Mar;34(3):431-439.
doi: 10.1007/s00381-017-3687-4. Epub 2018 Jan 3.

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

Omar Chamdine  1 , Ghada Ahmad Saad Elhawary  2 , Ahmad Samir Alfaar  3 , Ibrahim Qaddoumi  4
Affiliations

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

Omar Chamdine et al. Childs Nerv Syst. 2018 Mar.

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.

Keywords: Biopsy; Brainstem tumor; Embryonal tumor; Outcome.

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Conflict of interest statement

Conflict of Interest: The authors have no conflicts of interest relevant to this article to disclose.

Figures

Fig. 1
Fig. 1
Flow chart of the study cohort.
Fig. 2
Fig. 2
Overall survival of the patients compared to other groups and within the study groups. (A) Study group compared to tumors of other brain sites. (B) compared to PNET from other brain regions. (C) Comparing age groups within PNET. (D) Comparing patients according to the year of diagnosis. (E) According to therapeutic modality. (F) According to number of therapeutic modalities used.
See this image and copyright information in PMC

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