Dysembryoplastic neuroepithelial tumors (DNETs) are frequently seen in children and young adults with intractable epilepsy, and are typically located in the temporal cortex. Extracortical location of DNET is a very rare occurrence. The authors report on a child with a unique extracortical location of DNET with an extensive involvement to the supracallosal cistern and callosum, septum pellucidum, and lateral ventricle ependyma. The authors discuss the radiological and pathological characteristics of the lesion and reiterate the difficulty in differentiating the histological characteristics of central neurocytoma and DNET in extracortical locations and its significance for further management planning.
Oguz Cataltepe, Paul Marshall, and Thomas W. Smith
James T. Rutka
Oguz Çataltepe, Güzide Turanli, Dilek Yalnizoglu, Meral Topçu, and Nejat Akalan
Object. Slow-growing, low-grade temporal lobe tumors are one of the most common causes of epilepsy in children. Although there are numerous consistent features in this patient group, consensus about the management and surgical approach is lacking. In this study the authors review the clinical, pathological, and radiological features as well as outcome data obtained in 29 pediatric patients with temporal lobe tumor—related epilepsy and discuss the surgical treatment strategies.
Methods. In patients who presented with intractable seizures secondary to mass lesions and underwent comprehensive epilepsy workup, the tumor was resected and the diagnosis confirmed by pathological examination. A minimum follow-up period of 16 months was required. Medical records were reviewed for details of seizure type and duration, electrophysiological data, imaging studies, operative notes, pathological examination reports, and follow-up data. The surgical approach was as follows. The lesionectomy with/without cortical resection was performed in all cases of lateral temporal tumors. Lesionectomy was performed with/without cortical resection in cases of basal temporal tumors if the mesial structures were radiologically normal. Mesial temporal tumors were excised, as were the remaining mesial temporal structures in the nondominant hemisphere; however, if the tumor was in the dominant hemisphere, lesionectomy was performed only if the remaining mesial structures were radiologically normal.
Twenty-nine patients between 2 and 18 years of age were identified. Most tumors were located in the mesial temporal lobe. All patients underwent resection of the tumor with or without mesial and cortical structures. The most common pathological entity was dysembryoplastic neuroepithelial tumor. Sixty-nine percent of the patients remained seizure free (Engel Class I) and 14% experienced significant improvement (Engel Class II) after surgery. Outcome was better in the patients who underwent gross-total tumor resection.
Conclusions. Mesially located low-grade neoplasms were the most frequently observed mass lesions in children with temporal tumor—related epilepsy in this series. Resection of the tumor with or without amygdalohippocampectomy provides a high rate of seizure-free outcome. It is the author's opinion that temporal lobe tumors should be managed based on the subgroups defined by their anatomical locations. If the tumor is located in or in proximity to eloquent cortex, we recommend functional magnetic resonance imaging and invasive monitoring techniques to map the eloquent cortex and epileptogenic zone, thereby tailoring the resection.
Case report and review of the literature
Melike Mut, Oğuz Çataltepe, Figen Söylemezoğlu, Nejat Akalan, and Tunçalp Özgen
✓ Malignant triton tumor (MTT) is a variant of malignant peripheral nerve sheath tumors. The authors report a case of radiation-induced MTT in a patient with severe cervicothoracic cord compression and review the related literature. This 36-year-old man presented with pain and weakness in his left arm. His medical history was significant for a biopsy procedure involving the sampling of an aneurysmal bone cyst located at T1–3 near the left lung apex; this was performed 6 years prior to presentation and was followed by radiotherapy. Neurological examination demonstrated radicular findings involving the left C-8 and T-1 nerve roots. Neuroimaging studies revealed a large mass lesion extending from C-6 to T-2 along the vertebral column, invading the upper thoracic cavity and the adjacent lung apex, and infiltrating the paravertebral muscles. A subtotal resection was performed, but the tumor regrew extensively within a short time. It invaded the spinal canal and caused significant cord compression. The patient underwent surgery two more times for tumor debulking and to relieve progressive airway and spinal canal compromise. He eventually became quadriplegic, however, and died 13 months after diagnosis of MTT. This is the seventh case of radiation-induced MTT and the fifth of MTT with spinal canal involvement to be reported in the literature.
Oguz Cataltepe, Deborah Liptzin, Lindsey Jolley, and Thomas W. Smith
Diffuse villous hyperplasia of the choroid plexus (DVHCP) is a very rare cause of hydrocephalus in children. Only 12 cases of DVHCP have been reported in the literature. In this report the authors describe a new case of a patient with DVHCP who was diagnosed prenatally with hydrocephalus. In a comprehensive literature review and discussion, the authors also discuss radiological and histological characteristics of the reported cases, treatment approaches, and surgical modalities that have been used in the treatment of these patients.