A Constellation of Atypical Findings in a Nine-Year-Old Child With Dysembryoplastic Neuroepithelial Tumors: A Case Report and Review of the Literature

Abstract

Dysembryoplastic neuroepithelial tumors (DNETs) are rare, generally benign, mixed neuronal-glial neoplasms occurring most often between 10 and 14 years of age. These lesions are classically cortically based and solitary, found preferentially in the temporal lobe, and most commonly present with seizures. On magnetic resonance imaging (MRI), these lesions are generally cystic and have variable contrast enhancement, which, when present, often involves the periphery. Rarely, lesions followed radiographically may demonstrate delayed contrast enhancement. Here, we present a case of multifocal DNETs involving the cerebellum that demonstrated delayed contrast enhancement. In addition, these occurred in a patient with Noonan syndrome (NS), a "RASopathy" disorder associated with low-grade glial and glioneuronal tumors. We present a summary of all previously reported cases of cerebellar DNETs. Our patient was successfully treated surgically and is doing well clinically, now one year status post his last procedure, and is being closely monitored with serial MRIs for progression. Gross total resection is often curative without adjuvant therapy for most DNETs. Our case emphasizes the importance of radiographic surveillance, as multifocality and recurrence may necessitate more than one procedure. Lastly, clinicians should be suspicious for DNETs and other low-grade glial tumors when treating patients with NS, acknowledging their predisposition for multifocal involvement and atypical presentations.