The authors report on a case of a full-term infant with lumbar myelomeningocele who was found to have an intramedullary mass at the time of surgical repair of the defect. The intramedullary mass was consistent with a dermoid tumor both macroscopically and microscopically. This case provides evidence that dermoid tumors occurring at the site of previous surgical myelomeningocele repair are not always a consequence of incomplete excision of the dermal elements.
Edwin Ramos, Arthur E. Marlin and Sarah J. Gaskill
Edwin Ramos, Selim Benbadis and Fernando L. Vale
The purpose of this study was to identify the causes of failed temporal lobe resection in patients with mesial temporal sclerosis (MTS) and the role of repeat surgery for seizure control.
This is a retrospective study of 105 patients who underwent temporal lobe resection for MTS with unilateral electroencephalographic findings. The mean follow-up duration was 36 months (range 24–84 months). Surgeries were all performed by the senior author (F.L.V.).
Following initial surgical intervention, 97 patients (92%) improved to Engel Class I or II (Group A), and 8 (8%) did not have significant improvement (Engel Class III or IV; Group B). These 8 patients were restudied using video-electroencephalography (EEG) and MR imaging. All major surgical failures occurred within 1 year after initial intervention. Reevaluation demonstrated 3 patients (37.5%) with contralateral temporal EEG findings. Five patients (62.5%) had evidence of ipsilateral recurrent discharges. Four patients underwent extended neocortical resection along the previous resection cavity. Their outcomes ranged from Engel Class I to Class III. Only 1 patient (12.5%) who failed to improve after initial surgery was found to have incomplete resection of mesial structures. This last patient underwent reoperation to complete the resection and improved to Engel Class I.
Failure of temporal lobe resection for MTS is multifactorial. The cause of failure lies in the pathological substrate of the epileptogenic area. Complete seizure control cannot be predicted solely by conventional preoperative workup. Initial surgical failures from temporal lobe resection often benefit from reevaluation, because reoperation may be beneficial in selected patients. Based on this work, the authors have proposed a management and treatment algorithm for these patients.
Juan Santiago Uribe, Edwin Ramos-Zapata and Fernando Luis Vale
Juan S. Uribe, Edwin Ramos, Sammy Youssef, Fernando L. Vale and Ali A. Baaj
Tony Goldschlager, Nicolas Dea, Michael Boyd, Jeremy Reynolds, Shreyaskumar Patel, Laurence D. Rhines, Ehud Mendel, Marina Pacheco, Edwin Ramos, Tobias A. Mattei and Charles G. Fisher
Giant cell tumors (GCTs) of the spine are rare and complex to treat. They have a propensity for local recurrence and the potential to metastasize. Treatment is currently surgical and presents unique challenges due to the proximity of neural structures and the need for reconstruction. Denosumab has been shown in clinical trials to be an effective treatment for GCT, but has not yet been studied specifically in GCT of the spine or as a surgical adjunct. To the authors' knowledge this is the first such reported series.
A multicenter, prospective series of 5 patients with GCT of the spine treated with denosumab were included. Patient demographic data, oncological history, neurological status, tumor staging, treatment details and adverse events, surgical procedure, complications, radiological and histological responses, and patient outcome were analyzed.
All patients were women, with a mean age of 38 years, and presented with pain; 2 patients had additional neurological signs and symptoms. The mean duration of symptoms was 62 weeks. No patient had a prior tumor or metastatic disease at presentation. All patients had Enneking Stage III tumors and were treated with monthly cycles of 120 mg of denosumab, with initial additional loading doses on Days 8 and 15. Patients were given daily supplements of calcium (500 mg) and vitamin D (400 IU). There were no denosumab-related adverse events. All patients had a radiological response to denosumab. One patient failed to have a histological response to denosumab, with > 90% of tumor cells found to be viable on histological investigation.
This study reports the early experience of using denosumab in the treatment of spinal GCT. The results demonstrate a clinically beneficial radiological response and an impressive histological response in most but not all patients. Further experience with denosumab and longer patient follow-up is required. Denosumab has the potential to change the treatment paradigm for spinal GCT.