Primary bulbo-medullary glioblastoma in a child: case report.

Glioblastoma (GBM) of the spinal cord represents a rare entity in children and account for less than 1% of all central nervous system (CNS) cancers. Their biology, localization, and controversial treatment options have been discussed in a few pediatric cases. Here, we report a case of primary spinal cord glioblastoma in a 5-year-old girl having the particularity to be extended to the brainstem. This tumor has been revealed by torticollis and bilateral brachial paresis. The patient underwent subtotal resection; unfortunately, she died in reanimation 1 week later by severe pneumopathy. To the best of our knowledge, this is the first case in the literature reporting this particular localization in a child. Beyond their dismal prognosis, we discuss the rarity of the disease and describe the peculiar characteristics, management, and prognosis of this rare tumor in pediatric oncology. This case appears to be unusual for both the histological type and the extension to brain stern.

[Epidermoid cyst of the cisterna magna and the fourth ventricle: Report of four cases]. / Kystes épidermoïdes de la grande citerne et du quatrième ventricule : à propos de quatre cas.

INTRODUCTION: Epidermoid cyst is a congenital and benign tumor, developed from ectodermal inclusion. These cysts occur very rarely in the cisterna magna and the fourth ventricle. OBJECTIVE: To report four cases of epidermoid cyst of the cisterna magna and the fourth ventricle in the light of the data of literature. PATIENTS AND METHODS: We report a retrospective study of four cases of epidermoid cysts of the cisterna magna and the fourth ventricle. The data was collected from January 2000 to December 2006 from to series of 18 cases of epidermoid cysts of posterior cranial fossa (14 cases were localised at the cerebellopontine angle). All the patients had a physical examination and a complete neuroradiological imagery. The treatment was surgical. The follow-up was at least 9 months. RESULTS: There were two men and two women. The mean age was 47.75 years. All patients presented with cerebellar syndrome. Three patients had intracranial hypertension. Neuroradiological explorations showed a cystic lesion developed in the cisterna magna in two cases, in the fourth ventricle in one case and in the two locations in one case. Two patients had hydrocephalus. All patients had surgery with a posterior approach and one patient had first ventriculoperitoneal shunt. The diagnosis was confirmed by histological examination. Postoperatory outcome was favourable in all cases. One patient developed bilateral chronic subdural hematoma, which was surgically removed. A long time follow-up was good in all cases. CONCLUSION: Epidermoid cysts are characterized by a long evolution. The diagnosis is relatively characteristic in the imagery. The prognosis was favourable with a complete surgical resection.

[Solitary intrasellar plasmocytoma revealed by a diplopia: a case report]. / Plasmocytome solitaire intrasellaire révélé par une diplopie : à propos d'un cas.

INTRODUCTION: Plasmocytomas rarely invade the skull base. It can be solitary or multiple. The clinical presentation mainly consists in a progressive neuropathy involving many cranial nerves, or may simply present as an optochiasmatic syndrome. OBSERVATION: The authors report a case of a female patient aged 68 years presenting with an optochiasmatic syndrome with an anosmia and paralysis of the right sixth (VI) cranial nerve progressing over two years prior to her hospitalisation. Cerebral MRI showed a voluminous intrasellar lesion, isointense on T1 and hyperintense on T2, enhancing intensely after gadolinium injection with evidence of invasion of the sphenoid and cavernous sinuses. The endocrinologic assay was normal. A sphenoidal biopsy by the rhinoseptal route permitted the diagnosis of a plasmocytoma. A complete radiologic and laboratory assessment of the patient was accomplished, the patient benefited from local radiotherapy. DISCUSSION: Plasmocytomas are malignant tumors that are essentially osseous. The cervicocephalic region is rarely afflicted (1%). Extension to the cranial base seldom occurs. Less than 30 cases have been described in the literature simulating the other numerous neoplastic intrasellar lesions. CONCLUSION: Plasmocytomas of the cranial base revealing a myeloma represent a very rare entity. Nevertheless, whether solitary or multiple, a plasmocytoma must be considered amongst the differential diagnoses in the face of any invasive lesion of the sphenoid sinus.

Intracranial meningioma in children: different from adult forms? A series of 21 cases.

OBJECTIVE: Intracranial meningiomas are very rare in children, comprising only 0.4 to 4.1% of pediatric tumors and only 1.5 to 1.8% to all intracranial meningiomas. The goal of this study of pediatric meningiomas was to establish their epidemiological profile as well as their clinical and radiological features, to assess the long-term outcome, and compare this result with adult meningioma. PATIENT AND METHODS: We conducted a retrospective study from June 1983 to June 2007; during this period 521 patients underwent surgery for primary meningioma at the Rabat Hospital, Department of Neurosurgery. Twenty-one patients were under 16 years of age (4%). The clinical charts and imaging data were reviewed. RESULTS: The mean age was 10.3 years (range: 2 to 16 years), with 13 boys and eight girls. In one patient a neurofibromatosis was associated. The mean delay to diagnosis was 4.6 months (range: 1 to 12 months). The most common clinical sign was raised intracranial pressure (90%). Of the meningiomas diagnosed, 47% were convexity meningiomas while 24% were parasagittal and 19% were skull-base meningiomas; in two cases (9.5%) the location was intraventricular. The mean tumor diameter was 6.6 cm (range: 3 to 10 cm). A large cystic component was found in 24% of the cases. Surgery achieved a Simpson grade I resection in 47%; 62% of the tumors were grade I and 24% were grade II based on World Health Organization pathological classification. The mean follow-up period was 33 months (range: 6 to 120 months). The recurrence rate was 33%. CONCLUSION: Pediatric meningiomas are larger than those found in the adult population; there is a male predominance with high incidence of a cystic component and high-grade meningiomas, thus explaining the increased recurrence rate despite the multimodal treatment.

[Posterior fossa extradural and extracranial hydatid cyst]. / Kyste hydatique intra- et extracrânien de la fosse cérébrale postérieure (à propos d'un cas).

Hydatidosis is an endemic disease in Morocco. Cerebral echinococcosis is a relatively rare entity accounting for only 1-2% of all hydatid cysts in humans. Extradural hydatid cyst of the posterior fossa is a very uncommon site for the disease: only four cases have been reported in the literature. We report the case of a 37-year-old admitted for high intracranial pressure. Brain MRI showed an extradural and extracranial posterior fossa cyst without enhancement after contrast medium injection. Multiple hydatid cysts were removed and the histological examination of the tissue sample confirmed the diagnosis. The patient was given albendazole postoperatively with good follow-up 6 months later.

[Multiple intracranial ganglioglioma. Case report]. / Gangliogliomes multiples intracrâniens. A propos d'un cas.

Multiple intracranial ganglioglioma (GG) is an extremely rare condition, generally associated with van Recklinghausen neurofibromatosis. We report the case of a 26-year-old woman who presented with progressive tetraparesis, raised intracranial pressure and visual loss. The neuroradiologic workup demonstrated a multiple lesion involving the temporal lobe, the diencephalomesencephalon and the optical pathways. Computed tomography-guided stereotactic biopsy of a right temporal lesion led to the diagnosis of GG. In light of this case and the literature review, we discuss morphologic aspects, differential diagnosis and therapeutic options of GG. In patients with multiple cerebral tumors, search for multiple ganglioglioma is particularly important because of the therapeutic implications and the good prognosis of this tumor.

[Infected cervical epidural Ewing's sarcoma (case report)]. / Sarcome d'Ewing épidural cervical infecté (à propos d'un cas).

INTRODUCTION: Ewing's sarcoma is a relatively rare osseous malignant tumour. The age of onset is generally in the youth. Epidural localisation of the malignant osseous tumour Ewing's sarcoma is exceptional. Only 29 cases were reported in surgical literature including only one infected case. OBSERVATION: We report a case of a 24 years old patient who displayed a complete flaccid tétraplégia with T1 sensitive level. Spinal cord MRI documented a cervical posterior epidural process with peripheric gadolinium enhancement. Patient was operated in emergency. A C6 and C7 laminectomy allowed complete tumour resection with its purulent intratumoural collection revealed during the operation. A total tumour removal was performed. The immediate postsurgical evolution was remarkable, with a quasi complete recovery in the upper limbs. DISCUSSION: Firstly described by James Ewing in 1921, the spinal epidural locations of the Ewing's sarcoma are rare without specific clinical signs generally presenting as a radiculo-medullary compression. Our case is the second infected epidural Ewing sarcoma reported in the literature. However the cause remains unexplained. For this spinal epidural extraosseous tumour, X-ray radiography failed to show any osseous lesions. On MRI, the usually found aspect is a T1 and T2 isosignal with gadolinium enhancement. CONCLUSION: Through our observation, we underlined the scarcity of this pathology in the cervical epidural localization and the association with infection. We also emphasized on the complete removal, in order to give the best functional and vital outcome.

[Double localization of a cerebral germinoma. Case report]. / Double localisation d'un germinome cérébral. A propos d'un cas.

A germinoma located in both the pineal and suprasellar regions remains rare. The clinical expression can be polymorphic, although brain imaging reveals specific signs. We report the case of a 16-year-old boy who presented with diabetes insipidus of six months' standing, associated with visual loss and Parinaud's syndrome. Brain MRI showed a tumor in both the pineal and suprasellar regions. A stereotaxic biopsy targeting the infundibular growth confirmed the diagnosis of ectopic germinoma, which was successfully treated by radiotherapy and chemotherapy. The incidence of bifocal germinoma, its clinical and radiological characteristics as well as the therapeutic strategies to adopt are discussed.