An odd and serious "disc bulging"!

BACKGROUND AND IMPORTANCE: Cauda equina syndrome is a common acute medical condition, usually caused by large degenerative disc herniation or metastatic lumbar disease. We describe a patient who presented with a lesion featuring both discal and tumoral characteristics. CLINICAL PRESENTATION: A 41-year-old woman presented with ongoing back pain, progressive lower-limb weakness and sphincter disorder. Magnetic resonance imaging showed a very odd-looking large anterior epidural lesion originating from the L3-L4 space and severely compressing the roots of the cauda equina. Partial surgical decompression was performed in emergency. At a later time, redo surgery was performed to maximize resection, and was unfortunately followed by several complications. After 6 surgical procedures including a ventriculo-peritoneal shunt insertion and intensive rehabilitation, the patient could walk independently with the aid of one crutch. Following collegial review, the diagnosis of low-grade chondrosarcoma of the intervertebral disc was suggested. CONCLUSION: We report on a very unusual and therapeutically challenging spinal tumor diagnosed as low-grade chondrosarcoma of discal origin, an entity never previously described.

Malignant primary diffuse leptomeningeal gliomatosis with histone H3.3 K27M mutation.

INTRODUCTION: Malignant primary diffuse leptomeningeal gliomatosis (MPDLG) are rare central nervous system neoplasms associated with a poor outcome. CASE REPORT: We report the case of a 40-year-old woman who presented with unusual worsening of bilateral sciatica, headaches, diplopia and a left proptosis. MRI of the head and spine showed multiple leptomeningeal lesions with no intra parenchymal involvement. The search for a primary tumor was negative. An open surgical biopsy of the prominent intradural lumbar tumor was performed within a week. Histopathology, immunochemistry and molecular analyses revealed a malignant glioma with histone H3.3 K27M mutation. The patient was referred to the neuro-oncologist for chemotherapy and craniospinal radiotherapy. Despite aggressive therapy, she died of disseminated tumoral progression, 18 weeks after the diagnosis. CONCLUSION: MPLG is a rare tumor which should be considered whenever a patient presents with diffuse or multinodular meningeal contrast-enhancing lesions. Some cases of MLPG share histological and immunophenotypical features with diffuse midline gliomas H3-K27M-mutant, a rapidly fatal disease. The diagnosis remains histopathological and, therefore a biopsy is obligatory without delay. Immunohistochemistry and/or molecular analyses are now currently essential for a formal classification and, to provide a better prediction of clinical outcome, particularly in this heterogeneous group of tumors.

Solitary fibrous tumours and haemangiopericytoma of the meninges. A retrospective study for outcome and prognostic factor assessment.

BACKGROUND: To report on the outcome of patients diagnosed with central nervous system haemangiopericytoma (HPC) or solitary fibrous tumours (SFT) and identify factors that may influence recurrence and survival. MATERIAL AND METHODS: Between January 1977 and December 2016, a retrospective search identified 22 HPCs/SFTs. The patients underwent a total of 40 surgical resections and 63.6% received radiotherapy. Median follow-up was 7.8 years. RESULTS: Six patients (27.3%) were re-operated for tumour recurrence. At the end of the study, 15 patients (68.2%) had no residual tumour on the last imaging. Surgical recurrence-free survival at 5 years was 77.4%, [95% CI: 60.1-99.8]. None of the investigated variables was associated with recurrence. At the end of the study, 5 patients were deceased (22.7%) and only 10 patients (45.5%) had no residual tumour on the last imaging and were alive. Overall survival at 5 years was 95%, [95% CI: 85.9-100]. None of the investigated variables was associated with overall survival. Patients who received radiotherapy demonstrated neither a reduced risk of surgical recurrence (P=0.378) nor a longer overall survival (P=0.405). CONCLUSION: SFTs/HPCs are associated with a significant risk of recurrence that may reduce the survival. Even if we could not demonstrate their benefit in this limited series, we believe that tailored maximal tumour resection on initial surgery is beneficial and that adjuvant RT is useful for tumours displaying grade II or III, even in case of complete removal.

Atypical meningioma. A study on recurrence and disease-specific survival.

BACKGROUND: To analyse the outcome of patients with WHO grade II meningioma and identify factors that may influence recurrence and survival. MATERIAL AND METHODS: Between January 2000 and October 2016, a retrospective search identified 215 WHO grade II meningiomas operated on at our institution. A survival analysis was conducted on clinical and histological criteria. RESULTS: Eighteen patients (8.4%) had a previous history of grade I meningioma. The cohort underwent a total of 302 surgical resections and 29.7% received radiotherapy. Forty-one patients (19.1%) had been re-operated on for a WHO grade II meningioma relapse. Median follow-up was 4.5 years. At the end of the study, 105 patients (53.6%) had no residual tumour on the last scan. Surgical recurrence-free survival at 5 years was 82%, 95% CI [75.9-88.5]. Secondary grade II meningioma (HR=4.27, P=0.001), Simpson resection grade 1 and 2 vs. 3, 4 and 5 (HR=0.25, P=0.001) and, Ki-67 index (HR=0.22, P<0.001) were independently associated with the surgical recurrence-free survival. Forty-four patients died from their tumours (20.5%). Cause-specific survival probability at 5 years was 83.2%, 95% CI [77.6-89.1]. Age at diagnosis (HR=0.31, P<0.001), Simpson resection grade 1 and 2 vs. 3, 4 and 5 (HR=0.32, P<0.001) and, redo surgery for recurrence (HR=2.39, P=0.010) were independently associated with the cause-specific survival. Patients who received radiotherapy did not demonstrate either a reduced risk of recurrence or a longer survival (P=0.280). CONCLUSION: In this large series, atypical meningioma recurrence correlated with progression from grade I to II, incomplete resection and high Ki-67 index; shorter survival with an older age, incomplete resection, and redo surgery for recurrence. We did not observe a significant improvement in any of the clinical outcomes after radiotherapy.

World Health Organization grade III meningiomas. A retrospective study for outcome and prognostic factors assessment.

BACKGROUND: To depict the outcome of patients with WHO grade III meningioma and identify factors that may influence the survival. MATERIAL AND METHODS: Between 1989 and 2007, a retrospective search identified 43 WHO grade III meningiomas after cases exclusion. Nine patients (39.5%) had a previous history of grade I or II meningioma. The patients underwent a total of 89 surgical resections and 83.7% received radiotherapy. Median follow-up was 7.4 years. RESULTS: At the end of the study, 35 patients were deceased (81.4%). One patient died of disseminated metastatic disease of a papillary meningioma. Median overall survival was 4.1 years, 95% CI [1.9, 8.3]. Overall survival probability at 1, 5 and 10 years were respectively: 81.4%, 95% CI [70.6, 93.9], 48.8%, 95% CI [36,66.3] and, 27.5%, 95% CI [16.9, 44.9]. In univariate Cox regression, a previous surgery for WHO grade I or II meningioma (HR=2.05, 95% CI [1.03, 4.07], P=0.04) and the mitosis count (HR=0.3, 95% CI [0.12, 0.79], P=0.02) were associated with the overall survival. However, neither macroscopic gross total resection (HR=0.87, 95% CI [0.4, 1.87], P=0.71), nor radiotherapy (HR=0.75, 95% CI [0.31, 1.83], P=0.53) was associated with an increased survival. CONCLUSION: This series highlights the poor prognosis associate with the diagnosis of malignant meningioma. Patients with primary WHO grade III meningioma demonstrating less than 14 mitosis per 10 high power fields may live longer. We could not confirm the usefulness of complete resection and adjuvant radiotherapy.

[Pial synangiosis for moyamoya disease. The Bordeaux experience]. / Traitement de la maladie de Moya-Moya par synangiose piale. L'expérience bordelaise.

BACKGROUND: Different surgical methods may be used for the treatment of moyamoya disease. The authors report their experience with pial synangiosis revascularization. MATERIAL AND METHODS: We reviewed the clinical, surgical as well as radiographic records of all patients who underwent cerebral revascularization surgery using pial synangiosis for a symptomatic moyamoya disease at a single institution, Bordeaux University Hospital. RESULTS: Over a 6-year period (2007-2013), 17 procedures were performed in 9 patients. Median age at presentation was 11.5 years and six patients presented with an ischemic event. As regards previous medical history, five patients were diagnosed with moyamoya syndrome. Median time between the symptoms onset and the first surgical procedure was 5.9 months. The average age of the first surgery was 11.8 years and the median time between the two synangiosis was 3.5 months. One patient died of a malignant ischemic stroke after the second procedure. At long-term follow-up, no patient experienced any symptoms recurrence. CONCLUSION: Pial synangiosis typically results in an increase in collaterals from the superficial temporal artery or middle meningeal artery to the brain. It is a simple and effective surgical technique to prevent further moyamoya manifestation and to stabilize or improve the neurologic prognosis.

Anterior trans-frontal endoscopic management of colloid cyst: an effective, safe, and elegant way of treatment. Case series and technical note from a multicenter prospective study.

Different management options are available for the treatment of colloid cysts. Goals of those procedures are to achieve a complete resection avoiding potential long-term recurrence along with CSF pathways restoration with minimal morbidity and mortality. The two main surgical options are endoscopic resection or direct removal by either transfrontal or transcallosal approach. The efficacy of endoscopic technique to achieve gross total colloid cyst excision has been well documented. In the present study, authors describe a series of 29 patients who underwent surgery by a variation of the standard worldwide implemented endoscopic technique. Using a more anterior approach, it is easier to reach the roof of the cyst, its possible adherences with the tela choroidea, plexus, and the internal cerebral veins. The described approach has shown to be safe, quick, and very effective with a total cyst removal rate of 86.2%.