Recurrence and histological evolution of dysembryoplastic neuroepithelial tumor: A case report and review of the literature

Abstract

Studies of recurrent dysembryoplastic neuroepithelial tumors (DNTs) are distinctly rare. The present study reports the case of a 15-year-old female with a temporal lobe DNT, which recurred and transformed into an astrocytoma (WHO grade II) five years after an initial gross total resection (GTR). Furthermore, all the previous studies on recurrent DNT are reviewed. Although the majority of DNT cases demonstrate benign behavior, recurrent DNTs have been observed following a GTR of the tumor. Patients do not appear to benefit from post-operative adjuvant therapy, and inappropriate radiotherapy or chemotherapy may result in tumor recurrence or malignant transformation. The prognosis is favorable if a GTR of the recurrent tumor is achieved. The use of regular imaging examinations and the maintenance of a long-term follow-up is of importance following a tumor resection.

Salvage gamma knife radiosurgery in the management of dysembryoplastic neuroepithelial tumors: Long-term outcome in a single-institution case series

“…Some groups have even hypothesized a possible relation between adjunctive treatment (radiation and chemotherapy) and secondary malignant transformation. [ 9 35 ]…”

“…in 1988. [ 9 14 ] These rare tumors usually develop within the supratentorial cortex,[ 9 ] with preferred location in the temporal lobe (62%) and the frontal lobe (31%), although more unusual locations such as the caudate nucleus, the cerebellum, the pons, the septum pellucidum, the thalamic region, and the ventricular walls have also been reported. [ 3 13 21 33 40 ] The majority of patients are below 30 years of age at the time of diagnosis, with a peak incidence within the two first decades[ 3 5 25 33 ] and male gender predominance with a 1.4:1 male-to-female ratio.…”

“…[ 11 ] DNTs are World Health Organization (WHO)-grade I tumors[ 21 24 ] frequently associated with different grades of focal cortical dysplasia (FCD)[ 44 ] and usually result in epilepsy, often drug-resistant and intractable. [ 9 29 37 ] DNTs may account for 0.8–6.8% of all intractable epilepsy cases. [ 9 ] 65% of tumors collected at the European Epilepsy Brain Bank are reported to be DNTs and gangliogliomas combined.…”

“…[ 9 29 37 ] DNTs may account for 0.8–6.8% of all intractable epilepsy cases. [ 9 ] 65% of tumors collected at the European Epilepsy Brain Bank are reported to be DNTs and gangliogliomas combined. [ 3 ] Because of their complex nature, thorough histopathological analysis and advanced imaging is often necessary to achieve the correct diagnosis.…”

“…Microsurgery is widely accepted as the only treatment option, although cases of postsurgical recurrence and malignant transformation have been reported. [ 2 9 13 25 31 35 41 ] Adjuvant conventional radiotherapy and systemic treatments are generally regarded as ineffective,[ 9 35 ] although reports exist on the successful use of radiosurgery in selected cases. [ 23 38 ] We present our experience with long-term outcome using gamma knife radiosurgery (GKRS) as salvage therapy in this patient category, together with a review of the current literature.…”