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Lipomatous, vascular, and chondromatous benign tumors of the peripheral nerves

Representative cases and review of the literature

Claude-Edouard Châtillon, Marie-Christine Guiot, and Line Jacques

Benign peripheral nerve lesions of lipomatous, vascular, and chondromatous origin are very rare. Only one previous case of brachial plexus involvement by such a tumor has been reported. The authors report on their experience with peripheral nerve tumors in three patients and review the available literature on these topics. The three cases discussed include a 44-year-old woman with an intraneural lipoma of the right middle trunk, a 40-year-old woman with an intraneural hemangioma infiltrating the right posterior cord, and a newborn male with a predominantly cartilaginous hamartoma originating from the right C-5 nerve root.

The literature review yielded six previous cases of intraneural lipoma, approximately 50 cases of lipofibromatous hamartoma, 13 cases of intraneural hemangioma, and no previous case of cartilaginous hamartoma originating from a nerve. Intraneural lipomas are well encapsulated, and gross-total resection can be achieved. Lipofibromatous hamartomas are diffusely infiltrative; decompressive debulking and neurolysis is often the most appropriate initial approach for patients with symptomatic lesions. Resection of intraneural hemangiomas can be achieved but may require nerve resection and repair in some cases. Debulking has been reported to provide prolonged symptomatic relief in these lesions, and preoperative embolization and postoperative radiotherapy were beneficial in the case presented here. To the authors' knowledge, this is the first reported case of a cartilaginous hamartoma infiltrating a peripheral nerve. Gross-total resection of symptomatic intraneural lipomas is feasible and apparently curative. The optimal treatment for lipofibromatous hamartomas and vascular and chondromatous lesions of the peripheral nerves is uncertain and should be guided by the severity of symptoms.

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Meric A. Altinoz, Carlo Santaguida, Marie-Christine Guiot, and Rolando F. Del Maestro

✓ The authors describe the case of a patient with von Hippel—Lindau (VHL) disease in which a spinal hemangioblastoma contained metastatic renal cell carcinoma (RCC). The literature on tumor-to-tumor metastasis associated with VHL disease of the central nervous system (CNS) is reviewed.

Midthoracic back pain developed in this 43-year-old man with a left-sided radicular component 2 years after he underwent resection of a left RCC. Radiological findings demonstrated a T6–7 intradural intramedullary lesion. A T5–8 laminectomy and gross-total resection of the spinal cord mass were performed. Light and electron microscopic examination showed features of hemangioblastoma, which contained metastatic foci of RCC. Genetic analysis demonstrated the presence of a deleting mutation in the first exon (nt. 394–406) of the VHL locus, truncating 16 amino acids (N61–77) from the first beta sheet in the VHL protein. A review of the literature revealed that RCC-to-CNS hemangioblastoma is the second most common donor—recipient tumor association among the tumor-to-tumor metastases.

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Jack Lam, Patricia Tomaszewski, Guillaume Gilbert, Jeremy T. Moreau, Marie-Christine Guiot, Steffen Albrecht, Jean-Pierre Farmer, Jeffrey Atkinson, Christine Saint-Martin, Pia Wintermark, Boris Bernhardt, Sylvain Baillet, and Roy W. R. Dudley

OBJECTIVE

The authors sought to assess the utility of arterial spin labeling (ASL) perfusion 3T-MRI for the presurgical evaluation of poorly defined focal epilepsy in pediatric patients.

METHODS

Pseudocontinuous ASL perfusion 3T-MRI was performed in 25 consecutive children with poorly defined focal epilepsy. ASL perfusion abnormalities were detected qualitatively by visual inspection and quantitatively by calculating asymmetry index (AI) maps and significant z-score cluster maps based on successfully operated cases. ASL results were prospectively compared to scalp EEG, structural 3T-MRI, FDG-PET, ictal/interictal SPECT, magnetoencephalography (MEG), and intracranial recording results, as well as the final surgically proven epileptogenic zone (EZ) in operated patients who had at least 1 year of good (Engel class I/II) seizure outcome and positive histopathology results.

RESULTS

Qualitative ASL perfusion abnormalities were found in 17/25 cases (68%), specifically in 17/20 MRI-positive cases (85.0%) and in none of the 5 MRI-negative cases. ASL was concordant with localizing scalp EEG findings in 66.7%, structural 3T-MRI in 90%, FDG-PET in 75%, ictal/interictal SPECT in 62.5%, and MEG in 75% of cases, and with intracranial recording results in 40% of cases. Eleven patients underwent surgery; in all 11 cases the EZ was surgically proven by positive histopathology results and the patient having at least 1 year of good seizure outcome. ASL results were concordant with this final surgically proven EZ in 10/11 cases (sensitivity 91%, specificity 50%). All 10 ASL-positive patients who underwent surgery had positive surgical pathology results and good long-term postsurgical seizure outcome at a mean follow-up of 39 months. Retrospective quantitative analysis based on significant z-score clusters found 1 true-positive result that was missed by qualitative analysis and 3 additional false-positive results (sensitivity 100%, specificity 23%).

CONCLUSIONS

ASL supports the hypothesis regarding the EZ in poorly defined focal epilepsy cases in children. Due to its convenience and noninvasive nature, the authors recommend that ASL be added routinely to the presurgical MRI evaluation of epilepsy. Future optimized quantitative methods may improve the diagnostic yield of this technique.

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Shakir I. Shakir, Luis Souhami, Kevin Petrecca, Jose João Mansure, Khushdeep Singh, Valerie Panet-Raymond, George Shenouda, Amal A. Al-Odaini, Bassam Abdulkarim, and Marie-Christine Guiot

OBJECTIVE

The optimal adjuvant management for atypical meningiomas remains controversial. The aim of this study was to review long-term outcomes to identify potential prognostic factors for disease progression.

METHODS

From August 1992 to August 2013, 70 patients with atypical meningioma were treated at the authors’ institution. Pathology revision was performed based on WHO 2007 criteria. Patients with multiple tumors, neurofibromatosis Type 2, or inadequate imaging follow-up were not eligible. The authors performed pre- and postoperative serial measurements of tumor volume from MRI. Age, sex, tumor location, bone involvement, brain invasion, mitotic figures, preoperative disease volume, extent of resection, tumor growth rates, use of adjuvant postoperative radiation therapy (PORT), and residual tumor volume at the time of radiation therapy (RT) were assessed by univariate and multivariate analysis to determine their potential impact on disease progression.

RESULTS

Forty patients (57%) underwent gross-total resection (GTR) and 30 (43%) underwent subtotal resection (STR). PORT was delivered to 12 patients (30%) with a GTR and in only 4 (13%) with an STR. The 5-year progression-free survival (PFS) rate for patients in the GTR group with or without PORT was 100% and 54.1%, respectively (p = 0.0058). PFS for patients in the STR group with or without PORT was 75% and 0%, respectively (p = 0.0026). On multivariate analysis, STR and PORT were the only independent significant prognostic factors for disease progression with hazard ratios of 5.4873 (95% CI 2.19–13.72, p = 0.0003) and 0.0464 (95% CI 0.0059–0.364, p = 0.0035), respectively. Based on Youden’s index statistic, a cutoff residual tumor volume of more than 8.76 cm3 at the time of RT was associated with worse PFS (13.6% vs 56%, p = 0.0079). Before receiving RT, the median relative and absolute growth rates and tumor doubling time for patients were 124.2%/year, 4.8 cm3/year, and 1.67 years, respectively. These indices changed after RT to 0.245%/year, −0.09 cm3/year, and −0.005 year, respectively (p < 0.05).

CONCLUSIONS

In atypical meningioma, the use of PORT is associated with improved PFS even in patients who undergo GTR. Patients with residual tumor volume larger than 8.76 cm3 have an increased risk of disease progression and should be considered for early RT.

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Hamdy El-Hateer, Luis Souhami, David Roberge, Rolando del Maestro, Richard Leblanc, Eman Eldebawy, Thierry Muanza, Denis Melançon, Petr Kavan, and Marie-Christine Guiot

Object

The authors reviewed their institutional experience with pure low-grade oligodendroglioma (LGO), correlating outcomes with several variables of possible prognostic values.

Methods

Sixty-nine patients with WHO-classified LGOs were treated between 1992 and 2006 at the McGill University Health Center. Clinical, pathological, and radiological records were carefully reviewed. Demographic characteristics; the nature and duration of presenting symptoms; baseline neurological function; extent of resection; Karnofsky Performance Scale score; preoperative radiological findings including tumor size, location, and absence/presence of enhancement; and pathological data including chromosome arms 1p/19q codeletion and O-methylguanine-DNA methyltransferase promoter gene methylation status were all compiled. The timing and dose of radio- and/or chemotherapy, date of tumor progression, pathological finding at disease progression, treatment at time of disease progression, and status at the last follow-up were also recorded.

Results

The median follow-up period was 6.1 years (range 1.3–16.3 years). The majority (78%) of patients presented with seizures; contrast enhancement was initially seen in 16 patients (25%). All patients had undergone an initial surgical procedure: gross-total resection in 27%, partial resection in 59%, and biopsy only in the remaining 13%. Fifteen patients received adjuvant radiotherapy. Data on O-methylguanine-DNA methyltransferase promoter gene methylation status was available in 47 patients (68%) and in all but 1 patient for 1p/19q status. Survival at 5, 10, and 15 years was 83, 63, and 29%, respectively. Multivariate analysis showed that seizures at presentation and the absence of contrast enhancement were the only independent favorable prognostic factors for survival. The 5-, 10-, and 15-year progression-free survival rates were 46, 7.7, and 0%, respectively.

Conclusions

This retrospective review confirms the indolent but progressively fatal nature of LGOs. Contrast enhancement was the most evident single prognostic factor. New treatment strategies are clearly needed in the management of this disease.