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Gregory W. Albert and Murat Gokden

Solitary fibrous tumors of the spine are rare lesions. Their description in the literature is limited to case reports and small case series. While generally benign lesions, they can recur and occasionally occur as malignancies. Here the authors present the case of a 10-year-old boy, the youngest patient and first preadolescent reported thus far, with this condition. In addition, they perform a comprehensive review of all previously published cases of spinal solitary fibrous tumors.

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Jeffrey G. Ojemann, Christopher J. Moran, Murat Gokden, and Ralph G. Dacey Jr.

✓ Lesions involving the sagittal sinus typically present as masses compressing the sinus externally. The authors describe two cases of lesions entirely within the lumen of the sagittal sinus. In one of the cases, syncope was the presenting symptom and surgical resection of the cyst was performed. An entirely intraluminal cyst, consistent with a dural cyst, was resected, followed by reconstruction of the sinus and resolution of symptoms. Entirely intraluminal lesions of the sagittal sinus have rarely been reported as incidental findings. This represents the first report of symptomatic occlusion of a venous sinus by an intraluminal cyst.

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Eric W. Sherburn, Mark M. Bahn, Murat Gokden, Daniel L. Silbergeld, and Keith M. Rich

Preoperative differentiation between dysembryoplastic neuroepithelial tumor (DNT) and low-grade glioma is often not possible. Dysembryoplastic neuroepithelial tumor is a recently described entity of uncertain origin; however, the diagnosis has important clinical implications. Clinical and radiological findings of DNT and low-grade glioma, especially oligodendroglioma, may be similar. Treatment options and prognosis differ significantly between these two lesions; consequently, accurate diagnosis is imperative. The authors describe two individuals who presented simultaneously at their institution: one patient with an oligodendroglioma and a second patient with DNT. The natural history, neurodiagnostic, and pathological features of each are reviewed with special emphasis on the potential utility of magnetic resonance spectroscopy in differentiating these lesions.

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Emad Aboud, Mohammad Abolfotoh, Svetlana Pravdenkova, Abdulkerim Gokoglu, Murat Gokden, and Ossama Al-Mefty

OBJECT

Epidermoid tumors arise from misplaced squamous epithelium and enlarge through the accumulation of desquamated cell debris. Optimal treatment consists of total removal of the capsule; therefore, giant and multicompartmental tumors are particularly challenging. A conservative attitude in handling the tumor capsule is common given concerns about capsule adherence to neurovascular structures, and thus the possibility of recurrence is accepted with the intent of minimizing complications. This study focuses on the outcome of surgery in patients with giant epidermoid tumors for which total capsule removal was the aim.

METHODS

The authors conducted a retrospective analysis of all patients with giant epidermoid tumors treated by the senior author (O.A.), who pursued total removal of the capsule through skull base approaches. Patients were divided into 2 groups: one including patients with de novo tumors and the other consisting of patients who presented with recurrent tumors.

RESULTS

Thirty-four patients had undergone 46 operations, and the senior author performed 38 of these operations in the study period. The average tumor dimensions were 55 × 36 mm, and 25 tumors had multicompartmental extensions. Total removal of the tumor and capsule was achieved with the aid of the microscope in 73% of the 26 de novo cases but in only 17% of the 12 recurrent tumor cases. The average follow-up among all patients was 111 months (range 10–480 months), and the average postsurgical follow-up was 56.8 months (range 6–137 months). There were 4 recurrences in the de novo group, and every case had had a small piece of tumor capsule left behind. One patient died after delayed rupture of a pseudoaneurysm. In the de novo group, the average preoperative Karnofsky Performance Scale (KPS) score was 71.42%, which improved to 87.14% on long-term follow-up. In the group with recurrences, the KPS score also improved on long-term follow-up, from 64.54% to 84.54%. In the de novo group, 3 cases (11.5%) had permanent cranial nerve deficits, and 4 cases (15.4%) had a CSF leak. In the recurrence group, 3 cases (25%) had new, permanent cranial nerve deficits, and 1 (8.3%) had a CSF leak. Two patients in this group developed hydrocephalus and required a shunt.

CONCLUSIONS

Total removal of the capsule of giant epidermoid tumors was achieved in 73% of patients with de novo tumors and was associated with improved function, low morbidity and mortality, and a lower risk of recurrence. Surgery in patients with recurrent tumors was associated with higher morbidity and persistence of the disease.

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Samer K. Elbabaa, Murat Gokden, John R. Crawford, Santosh Kesari, and Ali G. Saad

Object

Radiation-associated meningiomas (RAMs) arise after treatment with radiation to the cranium and are recognized as clinically separate from sporadic meningiomas. Compared with their sporadic counterparts, RAMs are often aggressive or malignant, likely to be multiple, and have a high recurrence rate. However, limited information exists about the clinical, pathological, and cytogenetic features of RAMs in pediatric patients. The authors report the findings in 9 children with meningiomas following therapeutic radiation to the cranium. In addition, they performed a critical review of the English language literature on pediatric RAMs.

Methods

Medical files were searched for patients who demonstrated meningiomas after a history of radiation to the brain. Only those patients in whom a meningioma occurred before the age of 18 years were included in this study. Clinical and demographic data along with the MIB-1 labeling index and cytogenetic studies were evaluated.

Results

The patients consisted of 5 males and 4 females with a median age of 5 years (range 2–10 years) at radiation therapy. The latency period was a median of 10 years after radiation therapy (range 6–13 years). The MIB-1 labeling index was a median of 6.6% (range 4%–10%). Five patients (55.6%) displayed multiple meningiomas at the first presentation. Histological types included clear cell meningioma in 1 patient, fibroblastic meningioma in 2, chordoid meningioma in 2, meningothelial meningioma in 7 (atypical in 2 cases), xanthomatous meningioma in 1, and chordoid meningioma in 1. Cytogenetic studies showed that the loss of 22q12.2 was the most common abnormality (3 patients), followed by complex cytogenetic abnormalities (2 patients) and rearrangements between chromosomes 1 and 12 (1 patient) and a 1p deletion (1 patient).

Conclusions

In contrast to RAMs occurring in adults, those in pediatric patients show an increased incidence of multiplicity on first presentation and unusual histological variants, some of which are described here for the first time. There was no difference in the MIB-1 labeling index in children with RAMs as compared with that in children with non-RAMs.