Intracranial angiomatous meningiomas: A 15-year, multicenter study.

BACKGROUND: Angiomatous meningiomas (AMs) represent a rare subtype of meningiomas in which the vascular component prevail. They represent less than 1% of all intracranial tumors and approximately 2.1% of all meningeal tumors (Hasselblatt et al., 2004). The purpose of this study was to determine the clinical characteristics, radiological features and prognosis of AMs based on a Tunisian multicenter experience in the management of 58 successive cases of intracranial AMs. To the best of our knowledge, this is the largest series reported to date. METHODS: We retrospectively reviewed 58 patients of AM treated in the departments of Neurosurgery of The Tunisian National Institute of Neurology, Sahloul University Hospital and Fattouma Bourguiba University Hospital from January 2001 to December 2015. Clinical characteristics, radiographic features and treatment modality, in the form of radical surgery, were noted. Statistical analysis was done with regards to recurrence free survival (RFS) and overall survival (OS) using Kaplan-Meier survival analysis. RESULTS: The median age of the patients on admission was 53.0 years (8-78). Twenty-two patients were males and thirty-six were females. The median duration of symptoms prior to presentation was six months. Signs of increased intracranial pressure were the most common presenting symptom followed by seizures, motor weakness and cranial nerves paresis. Cystic changes were observed in 50% of cases, moderate to severe peritumoral edema in 81% of cases and multiple signal voids suggestive of blood vessels in 86.2% of cases. Forty-two patients underwent Simpson I excision (72.4%) while fourteen had Simpson II excision (24.1%). A Simpson III resection was realized in two patients (3.5%). The surgical resection was hemorrhagic in 63.8% of cases and in 13.8% of cases, several blood transfusions were necessary to maintain hemodynamic stability. No adjunctive treatment was administered. Six out of the fifty-six patients of the Simpson I/II groups recurred while one of the two patients with Simpson III resection had tumor regrowth. Median duration of RFS was 103 months. Second surgery was realized in all cases with no subsequent recurrence. The extent of tumor resection and the location were found to correlate with the risk of developing recurrences (P=0.001). CONCLUSIONS: AMs represent a rare subtype of meningioma characterized by variable cystic components, large peritumoral edema and multiple areas of vascular signal voids. The mainstay of the treatment is gross total resection, ideally following a preoperative embolization. The fate of the tumor remnant after incomplete tumor resection still needs to be evaluated and we do not recommend the systematic use of post-operative adjuvant RT in all cases. As local recurrence can develop many years after initial treatment, Long-term follow-up is mandatory.

Osteolytic clear cell meningioma of the petrous bone occurring 36 years after posterior cranial fossa irradiation: Case report.

OBJECTIVE AND IMPORTANCE: While bone invasion and hyperostosis are frequent phenomena in meningiomas, primary intraosseous meningiomas are rare and their occurrence in the skull base is an extraordinary exception. Moreover, radiation-induced meningiomas represent a unique clinical dilemma given the fact that patients with these tumors had often received a prior full course of radiotherapy. CLINICAL PRESENTATION: A 42-year-old man presented with a 3-month history of progressively worsening facial asymmetry. His medical history was consistent for a posterior cranial fossa irradiation at the age of 6 years for a non-confirmed brain stem tumor. On admission his Karnofsky performance status was graded as 50% and his neurological examination showed a complete right facial nerve paralysis and hearing impairment. Computed tomography and magnetic resonance imaging demonstrated an osteolytic tumor invading the whole right petrous bone without intracranial involvement. INTERVENTION: As the tumor reached the external auditory canal, a tissue sample was obtained locally. Pathological examination of the lesion identified a grade II clear cell meningioma and the patient was consequently addressed for an intensity modulated radiation therapy. His condition remained unchanged till the most recent follow-up examination, 8 months later. CONCLUSIONS: To the best of our knowledge, a radiation induced osteolytic clear cell meningioma of the petrous bone has not been previously reported. As little literature exists regarding the use of adjuvant therapies for these tumors, intensity modulated radiation therapy remains an attractive treatment option in case of pervious irradiation and general status alteration.

Congenital neuroblastoma presenting with paraplegia following spinal puncture in a neonate. Case report and review of the literature.

Neuroblastoma is the most common intraspinal solid tumor of childhood. Neurological deterioration due to an intratumoral hemorrhage following a spinal puncture is extremely rare. We report on the case of a 23-day-old neonate who was admitted to our institution for the onset of a paraplegia following a diagnostic lumbar puncture. The MRI showed an epidural tumor with massive intratumoral hemorrhage. Operatively and with histologic confirmation, the mass was determined to be a neuroblastoma. Following surgery, neurological function improved.

Syringomyelia following surgery for a spontaneous spinal subdural hematoma in a 13-year-old girl with congenital von Willebrand disease: case report and literature review.

BACKGROUND AND IMPORTANCE: Spontaneous spinal subdural hematomas are rare. Their occurrence in a child with congenital von Willebrand disease and the complication of their surgery by a large secondary syringomyelia have never been previously reported. CASE PRESENTATION: A 13-year-old girl with congenital von Willebrand disease presented to our emergency department in January 2011 for sudden onset of severe back pain centered in her thoracic spine rapidly aggravated by signs of acute myelopathy without any precipitating factor. MRI scan revealed a thoracic subdural collection anterior to the spinal cord at the T7-T9 level, hyperintense on T1- and T2-weighted sequences consistent with an acute spinal subdural hemorrhage. Evacuation of the subdural hematoma was realized immediately after hemostasis parameter correction, and post-operative course was uneventful with full functional recovery. One year later, the patient presented once again but with progressive and more severe myelopathy caused by a large syringomyelia extending from the T5 level to the conus medullaris. A syringopleural shunting was performed and the patient was unrolled under an intensive care and rehabilitation program. Her condition remarkably improved and she became able to walk independently within 2 weeks post-operatively. CONCLUSIONS: von Willebrand disease should be included as a possible factor of spontaneous spinal subdural hemorrhage. Surgery is advised in emergency and can be associated with remarkable recovery especially in children. Delayed syringomyelia can complicate the post-operative course and can be successfully addressed by syringopleural shunting. Long-term clinical and radiological follow-up is advocated.

Cavernous hemangioma of the skull and meningioma: association or coincidence?

Intraosseous cavernous hemangiomas of the skull are rare. Meningiomas are quite frequently encountered in a neurosurgical practice. The association between these two entities is nevertheless very uncommon. The authors present a case of a 72-year-old woman suffering from headache. The MRI showed a parietal meningioma with adjacent thick bone. The meningioma and the bone were removed. The histological examination confirmed the diagnosis of meningioma and revealed a cavernoma of the skull. The relationship between the lesions suggests more than a coincidental association. Several hypotheses are proposed to explain common causal connections.

Multiple spinal metastases from a well-differentiated liposarcoma of the iliac wing: a case report.

STUDY DESIGN: A case report. OBJECTIVES: To report an unusual case of multiple spinal metastases from an undiagnosed well-differentiated liposarcoma (WDLPS) of the iliac wing and to stress the need of a meticulous clinical examination and further screening of patients with chronic and asymptomatic bony lesions. SETTING: University of medicine of Monastir, Department of neurological surgery, Fattouma Bourguiba University Hospital, Monastir, Tunisia and University of Medicine of Tunis EL Manar, Department of neurological surgery, Tunisian National Institute of Neurology, Tunis, Tunisia. METHODS: A 39-year-old man presented with signs of spinal cord compression for the past 2 weeks. His medical history was consistent for an asymptomatic right iliac wing mass that appeared 3 years ago and for which he has not consulted. Magnetic resonance imaging revealed multiple bony lesions of the thoraco-lumbar spine associated with a 6-cm right paravertebral mass at the T4 level extending posteriorly through the intervertebral foramina to the spinal canal causing major spinal cord compression. An emergent T2-T6 laminectomy allowed for a complete resection of the epidural mass. Pathological examination confirmed the diagnosis of well-differentiated liposarcoma. Adjunctive radiation therapy was administered. RESULTS: The patient's neurological status improved remarkably under an intensive care and rehabilitation program. He was ambulatory without assistance in the second postoperative week. CONCLUSION: The case reported in this paper represents a genuine example of the possible metastatic potential of WDLPSs of the bone and underscores the importance of examining patients thoroughly, especially when they have chronic and asymptomatic lesions.

Cerebellar liponeurocytoma with extracranial extension: case report.

Cerebellar liponeurocytoma is a newly recognized, rare clinicopathological entity commonly described in the cerebellar hemispheres or the vermis. We present a rare case of cerebellar liponeurocytoma arising from the left cerebellar amygdala with extracranial extension. Such a condition has never been previously reported.