1/58. craniopharyngioma of the pineal region.Craniopharyngiomas generally develop either in the suprasellar region or in both suprasellar and intrasellar regions. We report on a nontypical location of the craniopharyngioma in the pineal region. An 8-year-old boy was admitted to the department of pediatric neurosurgery in a grave condition. An MRI scan of the brain was performed after the neurological examination and revealed a large neoplasm situated in the posterior part of the III ventricle and in the pineal region, measuring 8.5x6.5x5 cm. The size of the tumor and its location meant it was occluding three ventricles, with subsequent hydrocephaly. Total removal of the tumor was carried out. Microscopic investigation of the tissue removed showed the typical structure characteristic for craniopharyngioma of the adamantinomatous type. Results of a consultation 6 months after the operation revealed that the patient was feeling well, attending school regularly and had finished the first semester with excellent results. On control MRI scan no tumor was found.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/58. Treatment of a symptomatic posterior fossa subdural effusion in a child.We describe the first observation of a child with a posterior fossa subdural effusion with secondary hydrocephalus and tonsillar herniation. We diagnosed this entity in a 14-month-old girl with no history of trauma or coagulation disorder. The patient presented in our emergency department with opisthotonus and raised intracranial pressure resulting from supratentorial hydrocephalus. An emergency ventriculo-peritoneal shunt was placed, which resolved the symptoms only temporarily. Eventually external drainage of the subdural fluid was performed. The collection gradually disappeared, and both the external subdural shunt and the ventriculo-peritoneal shunt were removed. The patient made a complete neurological recovery. We review the physiopathology and treatment of subdural effusions in general, and propose some guidelines for the management of symptomatic effusions occurring in the posterior fossa in particular.- - - - - - - - - - ranking = 865.06618545597keywords = supratentorial (Clic here for more details about this article) |
3/58. Ultrasound-guided puncture of a Dandy-Walker cyst via the lateral and III ventricles.Since the dandy-walker syndrome was first described by Dandy and Blackfan, Taggart and Walker, the many variants of posterior fossa anomalies, the appropriate management of these malformations and the clinical outcome have been the subjects of controversy. Surgery of the posterior fossa with membrane excision was initially the preferred method of treatment. Unfortunately, there was a high rate of complications, and many of the patients treated in this way still needed a shunting system. Ventricular-peritoneal and/or cysto-peritoneal shunting is commonly used to treat symptomatic posterior fossa cysts of Dandy-Walker malformations and hydrocephalus. Cysto-peritoneal shunt implantation only was associated with a high rate of complications, and most patients so treated needed a ventriculo-peritoneal shunt in addition. According to the literature, combined ventriculo-peritoneal and cysto-peritoneal shunting is needed for satisfactory decompression of Dandy-Walker cyst and hydrocephalus in between 16% and 92% of cases. We report on a young patient with a Dandy-Walker malformation who needed drainage of the posterior fossa and a ventricular shunt. We decided to drain the cyst and the supratentorial ventricles via a single, especially prepared, catheter with many perforations. The catheter was inserted under ultrasound guidance. The tube was inserted from the left lateral ventricle through the foramen of Monro into the III ventricle and downwards into the cyst. Intraoperatively, an immediate decrease in the size of the cyst and the supratentorial ventricles was observed. Postoperative MRI confirmed the exact position of the catheter and sufficient drainage of the posterior fossa cyst and the ventricles. Six months later the girl was seen in our outpatient department. Clinical examination showed no neurological deficit, and MRI demonstrated sufficient drainage of the ventricles and the Dandy-Walker malformation, and in addition hypoplasia of the corpus callosum.- - - - - - - - - - ranking = 1730.1323709119keywords = supratentorial (Clic here for more details about this article) |
4/58. Pure yolk sac tumors in genital and extragenital sites: study on three pedriatic cases, with cytological findings on two.Cytopathological findings on three pedriatic gonadic-extragonadic pure yolk sac tumor are reported: the highly variable histological patterns (reticular, solid, festooning or pseudopapillary, polyvesicular vitelline), reflecting differentiation towards extraembryonic yolk sac structures, are not appreciated in cytologic samples. Since the tumor's cytologic spectrum is very broad, the most diagnostic differential difficulty with yolk sac tumor is adenocarcinoma. The presence of intracellular and extracellular hyaline globules, that are periodic acid-Schiff stain (PAS) positive and diastase resistant and correspond to alpha-fetoprotein production, alpha-fetoprotein positivity of the neoplastic cells; showing nuclear pleomorphism and vacuolated "bubbly" cytoplasm are the most consistent cytologic hallmarks of this neoplasm, which diagnosis would be confirmed by histologic, immunocytochemical and clinical findings.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/58. Cerebellar neuroblastoma in an infant.A cerebellar neoplasm in an 8-month-old boy is reported. While this tumour was composed of small cells and had regions resembling desmoplastic medulloblastoma, it showed ultrastructural neuronal characteristics including bundles of microtubules in the cell processes, numerous synaptic vesicles, and occasional abortive or complete synapses. These characteristic features warranted the diagnosis of a neuroblastoma of the cerebellum. The nature of this rare intraparenchymal tumour in infants is also briefly discussed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/58. Primary meningeal rhabdomyosarcoma in a child with hypomelanosis of Ito.Intracranial rhabdomyosarcomas are rare neoplasms, and those thought to be primary meningeal tumors are even more rare. Hypomelanosis of Ito is a neurocutaneous disorder believed to involve a defect in cells of neural crest origin. We report the case of a 15-month-old boy with hypomelanosis of Ito who developed a primary meningeal rhabdomyosarcoma. The patient initially presented with hydrocephalus and 2 months later developed neurologic signs localizing to the spinal cord. Radiologic studies revealed widespread leptomeningeal enhancement with compression of the spinal cord at C5-C7. A brain biopsy revealed a tumor diffusely involving the meninges. Microscopically, the tumor was composed of rhabdomyoblasts, many of which showed prominent cross-striations on routine hematoxylin-eosin staining. To the best of our knowledge, this is the first reported case of meningeal rhabdomyosarcoma in a patient with hypomelanosis of Ito and the fourth reported case of a primary meningeal rhabdomyosarcoma reported in the world literature.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/58. Benign melanocytic tumor in infancy: discussion on a rare case and review of the literature.Meningeal melanocytoma is an infrequent neoplasm of the central nervous system (CNS), especially in childhood and infancy. It was first described as an entity different from pigmented meningiomas and schwannomas in 1972, and few cases have been published so far. In this article, a 5-month-old male patient with meningeal melanocytoma is presented. This midline lesion was localized in the posterior fossa and manifested by hydrocephalus. The entire dural origin and extradural growing pattern in addition to the destruction of the adjacent occipital bone were the unexpected presentations since these tumors usually tend to locate on leptomeninges and to extend into the adjacent neural compartment rather than the outside. On the other hand, this case is the only one which had identical lesions in both surrenal glands and the left renal capsule, the structures containing neural-crest-derived cells outside the CNS. The prognostic criteria, differential diagnosis and its embryological aspects are discussed with an extensive review of the related existing literature.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/58. Prolonged cerebral salt wasting syndrome associated with the intraventricular dissemination of brain tumors. Report of two cases and review of the literature.hyponatremia is a frequent event in neurosurgery practice and is usually associated with subarachnoid hemorrhage, head trauma, infections and neoplasms. The two common clinical manifestations are the inappropriate secretion of antidiuretic hormone (SIADH) and the cerebral salt wasting syndrome (CSWS), which were usually attributed to each other due to identical clinical presentation. In contrast to the better-recognized SIADH, there has not been a uniform consensus over the humoral and neural mechanisms of CSWS and functional aspects of renal response. In this article, we report on 2 cases of a primitive neuroectodermal tumor with prolonged CSWS manifested during the intraventricular dissemination of primary disease and the high catabolic stage.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/58. adrenocortical carcinoma with cerebral metastasis in a child: case report and review of the literature.OBJECTIVE AND IMPORTANCE: adrenocortical carcinoma (ACC) is rare in the pediatric population, and brain metastasis seldom occurs. CLINICAL PRESENTATION: The authors report a case of metastatic ACC to the brain in a 9-year-old patient who had an adrenal cortex neoplasm removed at 4 years of age, and was free of symptoms for 5 years. Two weeks before admission she complained of blurred vision in both eyes. INTERVENTION: Examination revealed bilateral papilledema, and a magnetic resonance imaging (MRI) of the brain revealed a mass in the left lateral ventricle with extensive vasogenic edema and hydrocephalus. The tumor was removed, and histopathologic examination demonstrated metastatic ACC. CONCLUSION: Although ACC is a rare neoplasm it must be considered in the differential diagnosis of cerebral lesions in patients with a history of this tumor. Periodic long-term brain imaging is suggested as part of the follow up in patients with adrenocortical neoplasms.- - - - - - - - - - ranking = 3keywords = neoplasm (Clic here for more details about this article) |
10/58. diagnostic imaging and clinical findings in rhombencephalosynapsis: case report and literature review.Rhombencephalosynapsis is a rare condition in which most cases are found in newborns and infants. Morphological findings are predominantly characterized by fusion of the cerebellar hemispheres and absence of the vermis with often associated supratentorial anomalies. We review the literature with emphasis on diagnostic imaging of this condition and present a case of a 2-year-old girl.- - - - - - - - - - ranking = 865.06618545597keywords = supratentorial (Clic here for more details about this article) |
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