Pediatric high grade gliomas: Clinico-pathological profile, therapeutic approaches and factors affecting overall survival.

INTRODUCTION: Pediatric high grade gliomas are rare tumors of the central nervous system. Treatment is multidisciplinary, comprising surgical excision followed by radiotherapy and/or chemotherapy. OBJECTIVES: describe these tumors' characteristics as seen in our institution, and identify factors associated with better overall survival. PATIENTS AND METHODS: We conducted a retrospective study of 30 cases of pediatric high grade glioma treated consecutively in our institution over a 20-year period. Brainstem tumors and patients aged more than 22years were excluded. Univariate analysis was conducted to determine factors associated with better overall survival. RESULTS: The series comprised 30 pediatric high grade gliomas: 27 glioblastomas and 3 anaplastic astrocytomas. The sex ratio was 1.7. Mean age was 13years. Tumors were mainly located in the cerebral hemispheres (63.3%). Median tumor size was 5cm. Glioblastomas were subdivided into 26 cases of classical subtype (96.3%) and 1 case of epithelioid subtype (3.7%). Surgical strategy consisted in tumor resection in 24 cases (80%). Twenty-one patients (70%) received postoperative radiotherapy. Therapeutic response at end of treatment was complete in 7 cases (23.3%). Postoperative radiation therapy and complete treatment response were significantly associated with improved overall survival in all high grade gliomas and also specifically in glioblastomas (P<0.001 and P=0.005, respectively). CONCLUSION: Our results suggest that postoperative radiotherapy and complete treatment response are predictive factors for better overall survival in pediatric high grade glioma.

[Choroid plexus carcinoma: a case report]. / Carcinome du plexus choroïde : à propos d'un cas.

OBJECTIVE: Choroid plexus carcinomas are rare and occur more frequently in children than in adults. The differential diagnosis includes choroid plexus papilloma and papillary ependymoma. The prognosis is generally poor. The objective of this paper is to discuss the epidemiological characteristics, diagnosis, treatment, and prognosis of this rare childhood tumor. CASE REPORT: A three-year-old child was hospitalized with a history of partial epilepsy and intracranial hypertension. A computed tomography scan showed an intracranial mass measuring 11cm in diameter, extending to the ventricle system with heterogeneous contrast enhancement. He was urgently operated for loss of consciousness. During the operation, the patient developed an abundant hemorrhage and died. The histopathological examination of the excised tumor identified a choroid plexus carcinoma. CONCLUSION: Choroid plexus carcinomas are rare and treatment is currently controversial. Radical excision is quite difficult and associated with excessive blood loss.

[Extra dural arachnoid cyst: case report]. / Kyste arachnoïdien extradural spinal: à propos d'un cas.

Extradural arachnoid cysts are uncommon expanding lesions in the spinal canal, which may communicate with the subarachnoid space. Usually located in the lower thoracic spine, they may cause symptoms by compressing the spinal cord or nerve roots. We report a case of an extradural thoracic arachnoid cyst revealed by progressive spinal cord compression. CT myelography and MRI enabled diagnosis. Rapid neurological improvement was observed after surgical resection.

[Multilevel costovertebral echinococcosis: effectiveness of a combined approach]. / Hydatidose costovertébrale pluriétagée: intérêt d'un abord combiné.

Hydatid disease generally involves the liver and the lung, but rarely can be encountered in bones. The disease predominantly occurs in vascularized areas, involving in descending order: vertebrae, long bones, ilium, skull, and ribs. Vertebral and rib hydatidosis may result from vascular or lymphatic migration of a fertile cell from a hepatic focus. In humans, the two main forms are due to Echinococcus granulosis and less frequently, E. multilocularis (alveolaris). The hydatid cysts usually grow slowly asymptomatically and compression of the involved or the neighboring organ causes clinical manifestations. In this patient, hydatid disease occurred in the chest wall with secondary spinal canal involvement successfully treated by removal of cysts via T7, T8, T9, T10, and T11 laminectomies via a posterior approach, followed by resection of the involved ribs. Total removal of cysts without rupture appears to provide effective protection against late recurrences.

[Ectopic prolactinoma of the sphenoidal sinus: case report]. / Prolactinome ectopique du sinus sphénoïdal.

A 34-year-old woman was hospitalized for exploration of amenorrhea and galactorrhea. She had experienced primary sterility, diagnosed 11 years earlier. Endocrine tests demonstrated hyperprolactinemia (serum prolactin 594 ng/ml). Magnetic resonance imaging (MRI) showed a soft tissue mass located within the posterior part of the sphenoidal sinus. The pituitary gland appeared normal. Medical treatment with bromocriptine was unsuccessful. Transsphenoidal tumor resection was performed. Histology reported adenoma, which was consistent with prolactin-producing tumor demonstrated by immunohistochemical staining for prolactin. Because of the persistence of hyperprolactinemia related to an intra-sphenoidal tumor remnant, bromocriptine (15 mg/d) was initiated. Menses ensued and prolactin level declined (49 ng/ml). One year later, the tumor remnant had disappeared on the MRI and the patient became pregnant. Ectopic adenomas of the sphenoidal sinus are of particular interest due to the diagnostic difficulties encountered. Nevertheless, the nature of the lesion can be determined from the endocrine manifestations. Some of these tumors respond to medical treatment, similar to intra sellar tumors. Surgery remains however the only means of obtaining definitive diagnosis and cure.

[Syringomyelobulbia associated with cervical spondylosis. Pathophysiology and therapeutic implications]. / Syringomyélobulbie et canal cervical étroit.

Spinal spondylosis is rarely implicated in syringomyelia. We report the case of a 70-year-old patient with a 10-year history of gait disturbance; constrictive pain of lower limbs and urinary incontinance. Physical examination disclosed spastic tetraparesis. In the upper limbs, deep tendon reflexes were abolished, with hyposthesia and hands amyotrophy. Brain and cervical MRI showed syringomyelobulbia with cervical spondylotic myelopathy. Extensive cervical laminectomy induced a mild clinical improvement. A second MRI performed 6 months after surgery depicted a complete disappearance of the bulbo-medullar cavitation with secondary atrophy. Extradural spondylotic compression of the spinal cord should be firmly considered as an etiology of syringomyelia. A purely extradural decompression could be sufficient to induce regression of the medullary cavitation.

Cavernous sinus tuberculoma: diagnostic difficulties in a personal case.

BACKGROUND: Tuberculoma of the brain is rare, and its location in the cavernous sinus is exceptional. Many factors make the diagnosis difficult, necessitating a surgical procedure. CASE DESCRIPTION: A 44-year-old woman complained of persistent headaches and diplopia. The examination revealed a right cavernous sinus syndrome. Radiologic investigation showed a growing process in the right cavernous sinus, overlapping the sella turcica. The diagnoses of metastasis, tuberculoma, and sarcoidosis were considered, but none of them could be confirmed. A biopsy of the lesion via a subtemporal approach revealed a tuberculoma. Antituberculous treatment led to complete resolution of her symptoms. CONCLUSION: The diagnosis of a cavernous sinus tuberculoma is difficult; it is confirmed by biopsy. However, a trial of medical treatment, if the results are favorable, may obviate the need for a surgical procedure.

[Hydatid cysts of the brain stem. Two cases]. / Les kystes hydatiques du tronc cérébral. A propos de deux cas.

Cerebral hydatid cysts represent 2-3% of all intracranial masses in endemic countries. Its incidence in posterior fossa is very rare. We report two cases of brainstem location. Clinically, the lesion exhibited signs of brainstem tumor. In two patients, CT scan showed a hypodense lesion. There was no enhancement after contrast administration. One patient was explored by MRI; on precontrast images, the lesion appeared homogeneous with hyposignal intensity and smooth limits. T2 weighted MRI and post contrast examination confirmed the cyst nature of the lesion. Surgery was performed in the two patients. The cyst was first aspirated and its membrane was then removed. Post operatively, one patient died, the other one is still alive but severely affected two years later. CT scan showed total disappearance of the cyst. The clinical presentation, radiological findings and surgical procedures are discussed.

[Teratoma and medulloblastoma of the cerebellum: a case]. / Tératome et médulloblastome du cervelet: un cas.

The majority of intracranial teratoma are localized in pineal and sellar regions. In cerebellum, the teratoma is quite rare, the association with medulloblastoma is exceptional and was differentiated from medullomyoblastoma. We report one case of 5 years old boy with intracranial hypertension for 3 months. The cerebral computed tomography showed a tumor in the fourth ventricle. The histologic study of surgical specimens found a proliferation of component of medulloblastoma adjacent to mature teratoma with smooth and striated muscles, chondroid component, adipose tissue and epithelial elements. Our objective is to discuss the diagnosis, the hitogenesis and the prognosis of this tumor.