Dysembryoplastic Neuroectodermal Tumor: An Analysis from the Surveillance, Epidemiology, and End Results Program, 2004-2013

Abstract

Background: Dysembryoplastic neuroectodermal tumor (DNT) is a rare neoplasm. Though the pathology is commonly considered benign, there have been various reports documenting rapid growth, recurrence/progression, sudden death, and malignant transformation. Most studies have addressed outcomes regarding seizure control, but limited data exist regarding incidence and survival. Consequently, we explore the Surveillance, Epidemiology, and End Results (SEER) database to explore the epidemiology of DNT.

Methods: From the SEER-18 registry database, information from all patients diagnosed with intracranial DNT between 2004 and 2013 was extracted, including age, sex, race, marital status, tumor location, tumor size, receipt of surgery, extent of primary surgery, receipt of radiation, and follow-up data. Age-adjusted incidence rates and overall survival (OS) were calculated. A Cox proportional hazards model was used to assess relationships between various demographic/treatment variables and OS.

Results: A total of 381 cases were identified in the SEER-18 database. The incidence of DNT within the large subset of the United States population represented by SEER was 0.033 per 100,000 person-years (95% confidence interval [CI], 0.030-0.037). The median duration of follow-up was 50 months. The median OS was not attained. The 3-, 5-, and 9-year OS was 99.363% (95% CI, 97.428%-99.844%), 97.993% (95% CI, 95.168%-99.174%), and 96.296% (95% CI, 91.834%-98.341%), respectively. Seven of the 381 patients passed at their last follow up. Of all demographic/treatment factors, only receipt of radiation demonstrated a significant relationship with OS (hazard ratio, 0.051; 95% CI, 0.01-0.267; P < 0.01).

Conclusions: Although the prognosis for DNT is generally favorable, the pathology can lead to poor outcomes in rare cases. Common demographic factors, treatment with surgery, and the extent of surgical resection did not show significant associations with OS. In contrast, treatment with radiation was associated with poorer OS.

Keywords: DNET; Dysembryoplastic neuroectodermal tumor; SEER.