Childhood spinal glioblastoma multiforme with intracranial dissemination. A case report.

Primary spinal cord tumors in children are uncommon and account for approximately 5% of all pediatric central nervous system tumors. Intracranial metastases from primary spinal cord tumors have rarely been reported. Spinal cord glioblastoma in children mainly involves the thoracic region, whereas involvement of the medullaris conus with intracranial dissemination has been described in six cases. This paper describes a pediatric case of thoracic glioblastoma multiforme with intracranial dissemination in the early stage of the clinical course and discuss the clinical and neuroradiological manifestations, the possible patterns of dissemination and finally to consider the therapeutic implications.

Occipital aneurysmal bone cyst secondary to eosinophilic granuloma.

We describe the case of a 2-year-old male patient with an aneurysmal bone cyst (ABC) of the occiput secondary to unifocal eosinophilic granuloma (EG). The lesion presented as a painless mass of the scalp which had grown rapidly over the 2 weeks prior to admission. Radiologically, the lesion was osteolytic and multicystic with fluid-fluid levels. On histology, the cyst wall contained hemosiderin-laden histiocytes, spindle cells, multinucleated giant cells, Langerhans' cells and eosinophils. Surgical treatment consisted of en bloc resection. Fourteen months after surgery, the patient was well with no local recurrence. Association between EG and ABC is rare. To our knowledge, this is the first case involving the skull.

Cerebrospinal fluid shunt infections in infants.

Infection remains a major cause of morbidity and mortality following CSF shunt procedures. In this study 191 shunt procedures carried out from January 1981 to December 1992 in a series of 81 infants (less than 6 months old) were retrospectively analyzed for possible risk factors. The overall surgical infection rate was 7.8%, with 15 infections occurring in 14 patients (17.2%). No significant difference in the rate of infections was found in relation to sex, birth weight, gestational age, and type of shunt procedure (primary insertion/revision). The occurrence of other infections during the period of shunt surgery did not influence the infection risk either. Intraventricular hemorrhage and central nervous system infections as causes of the hydrocephalus were found to correlate with septic risk. Young age (less than 6 months) seems to represent the main risk factor, and this is related both to the immunologic deficiency and to the particular features of residential bacterial flora in this age group.

Results of surgery in children with cerebral cavernous angiomas causing epilepsy.

Epilepsy is the most frequent presenting symptom of cerebral cavernous angiomas or cavernomas, and surgical removal of these vascular malformations is considered the treatment of choice in patients with intractable or long-standing seizures, or in those with poor compliance to medical therapy. In this paper the results of surgical treatment in 11 children with seizures from cerebral cavernomas are reported. Surgery for deep-seated cavernomas was aided in 3 cases by a stereotactic localization technique, that allowed a limited approach with minimal brain damage. Major morbidity and mortality were absent; follow-up ranged between 1 to 16 years. Improved seizure control was seen in all the patients: eight (72%) became seizure-free with the same preoperative therapy; one became seizure-free with a lower drug dosage, and two (18%) were seizure-free without medical therapy. The removal of cavernomas prevented the risk of haemorrhages or further deficits from growth and, above all, avoided spreading and autonomization of the epileptogenic area induced by the cavernoma.

Surgical management of cerebral cavernous angiomas causing epilepsy.

Epilepsy is the most frequent presenting symptom in patients with cerebral cavernous angiomas. Surgical removal of these vascular malformations causing epilepsy is usually recommended for patients with disabling, intractable seizures, while conservative management is indicated for neurologically intact patients with well controlled seizures. In this paper is reported a retrospective series of 36 patients suffering epilepsy from cerebral cavernous angiomas, and admitted at our Institution between 1975 and 1992. All patients were surgically treated; mortality and morbility were absent. The mean follow-up period was 5.9 years, since the 36 patients were alive and available for a control review in December, 1993. Seizure outcome of the patients resulted in a complete cure of the epileptic disorders in 9 (25%), improved seizure control with decreased medication in other 11 (30.5%), and epilepsy control with the same preoperative therapy in the remaining 16 (44.5%) patients. Furthermore, patients with preoperative neurological signs associated to epilepsy resulted improved. The authors conclude that surgery, where it is unlikely to cause neurological deficits, may allow a definitive cure of epilepsy in patients with cerebral cavernous angiomas, preventing the possible risks from hemorrhage and mass effect.

Surgical management of cavernous angiomas in children.

Cavernous angiomas are vascular malformations affecting any part of the central nervous system (CNS). The management of asymptomatic cavernous angiomas is still debated due to their poorly understood natural history, although more data are now available regarding results of surgical treatment in symptomatic cases. The authors report their surgical experience with 18 pediatric patients operated on for symptomatic CNS cavernous angiomas. The children ranged in age from 10 months to 17 years, without a relevant sex difference. Cavernous angiomas were intracranial in 17 cases: 15 being in the supratentorial compartments and two in the cerebellum. Clinical manifestations were as follows: seizures in 11 cases, focal neurologic deficits in five, and headache in one. The 18th case was observed in a girl showing paraparesis in the spinal subdural-extramedullary space at T8-T9 level. Excision of four deep cerebral lesions was performed after stereotactic localization through non-eloquent cortex. Pathologic confirmation of cavernous angiomas was obtained in all patients. Mortality from surgical procedures was absent in this series. The follow-up period ranged from 1 to 16 years. All 11 epileptic patients obtained seizure control; improvement or stabilization of neurologic symptoms was observed in the remaining seven patients.

Regressing intracranial carotid occlusions in childhood.

Though detected with increasing frequency, intracranial carotid artery dissection remains less common in infancy. We report on 3 otherwise healthy children aged 8, 12 and 15 years who presented with focal headache and stroke secondary to intracranial carotid occlusive disease consistent with arterial dissection. In 2 cases this was precipitated by strenuous physical exertion. The protean angiographic configuration included long tapered narrowing with focal stenosis, beaded narrowing with Moya Moya vascular network and 'string sign'; occlusion of the anterior cerebral artery was always present. Control angiograms revealed complete or partial recanalization in all cases suggesting self-healing dissection. The clinical course was smooth in all patients, and at long-term follow-up (5, 3, and 2 years) they remain in good neurological condition. Although intracranial carotid dissection has a poor reputation, regression to normal and fair outcome may sometimes occur as in the extracranial counterpart, suggesting the existence of benign forms of the disease. Surgical procedures should be weighed against the spontaneous resolution of the lesion.

Primary intrasellar germinoma: case report.

Intracranial germinomas arising primarily within the sella turcica are extremely rare. Preoperative diagnosis is difficult to establish even with sophisticated procedures. Diabetes insipidus is the main clinical manifestation. The authors report a case of an apparently primary intrasellar germinoma causing subclinical pituitary apoplexy in a 12-year-old boy. The transsphenoidal approach and appropriate radiotherapeutic management were employed with a good outcome.

Establishment of a human medulloblastoma cell line (BO-101) demonstrating skeletal muscle differentiation.

A permanent cell line, BO-101, was derived from a classic vermian medulloblastoma in a 9-year-old child. This line grew in vitro in adherent cultures and grew in athymic mice as serially transplantable intracranial and subcutaneous xenografts. Intracranial neoplasms grew as masses of small cells, which focally showed large cells with intense immunoreactivity for desmin, myoglobin and alpha-striated actin. The rhabdomyoblastic nature of these cells was confirmed ultrastructurally. The primary neoplasm showed immunoreactivity for synaptophysin, neuron-specific enolase and vimentin. A large panel of monoclonal antibodies and antisera against neuronal and glial antigens failed to show glial and neuronal immunoreactivity in the cell culture and xenografts. Despite the marked genotypic and phenotypic differences, the original neoplasm and the cell line share a common chromosomal marker del (12) (p 13.1). The BO-101 line differs phenotypically and genotypically from previously established medulloblastoma cell lines and further supports the heterogeneous biologic properties of the cell populations that constitute these neoplasms.

N-myc and c-myc oncogenes amplification in medulloblastomas. Evidence of particularly aggressive behavior of a tumor with c-myc amplification.

N-myc and c-myc amplification was investigated in 27 medulloblastomas. DNA was extracted from 19 formalin fixed and paraffin embedded tumors and from fresh frozen tumor tissue in 8 other cases. The results showed no evidence of amplification of N-myc oncogene and only 1 case had a 27 fold amplification of c-myc. Cytogenetically, this neoplasm presented numerous double minute chromosomes (DMs). Moreover, it had an unusual rapidly aggressive course with massive cerebrospinal fluid dissemination unresponsive to intrathecal chemotherapy. Our results indicate a low incidence of N-myc and c-myc gene amplification in medulloblastomas, suggesting that the oncogenic mechanism in these neoplasms is not closely related to DNA gene amplification. C-myc amplification, although not frequently observed, may however provide a growth advantage for medulloblastoma cells in vivo, favoring their rapid dissemination. Medulloblastomas with c-myc activation may represent a subgroup of tumors with a more aggressive behavior.

Intracranial tumors during the 1st year of life.

Twenty-eight patients with intracranial tumors symptomatic during the 1st year of life were managed from January 1970 to March 1988. Supratentorial location (19 cases) was dominant over the infratentorial. The most common histological type was astrocytoma; choroid plexus papilloma and ependymal tumors followed in frequency. Twenty-two infants (78.6%) had associated hydrocephalus. Macrocrania, vomiting, delayed milestones, and behavioral disturbances were the chief clinical manifestations. Four patients were admitted in extremely deteriorated condition and died preoperatively. Twenty cases underwent surgery for tumor removal with a 1-month mortality rate of 20%. Tumor excision provided permanent relief of hydrocephalus in the majority of the surviving cases. Five patients received conventional radiotherapy. Stereotactic brachytherapy yielded an excellent result in 1 case. Overall 13 cases are currently alive; 6 of them have survived longer than 6 years. Fourteen of the 15 patients who were dead at follow-up succumbed within 1 year after diagnosis.

High concentrations of catecholamines in human hypothalamic-hypophysial blood.

While the hypothalamic-hypophysial portal system has been extensively studied in laboratory animals, equivalent studies have not been performed in humans. Here, we present an experimental procedure for collecting suprapituitary blood in man. To solve the question on the origin of such blood we investigated specific markers of hypothalamic secretory activity: the catecholamines (CAs). We found (a) norepinephrine (NE), dopamine (DA), and epinephrine (E) concentrations from approximately 1.5 to 2.5, 3.5 to 4.5, and 6- to 10-fold higher, respectively, in suprapituitary than peripheral blood, (b) different NE/DA and NE/E ratios in favor of DA and E in suprapituitary blood, and (c), a complete (100%) group separation (suprapituitary vs. peripheral) when discriminant analysis included only DA and E. These data indicate that suprapituitary blood composition is different from that of the peripheral blood, and is particularly rich in CAs and claimed differences between DA and E release on one hand and NE release on the other in suprapituitary blood also are observed. We advance the hypothesis of a hypothalamic source of such amines draining via median eminence into portal vasculature, and name this blood "hypothalamic-hypophysial blood." Besides serving as "classical" neurotransmitters, CAs may also have a direct neurohormonal role in the regulation of the human hypothalamic-hypophysial function.

Bilateral traumatic third nerve palsy.

Bilateral palsy of the third cranial nerve in a head-injured patient is described. Delayed computed tomography scanning demonstrated a midline necrotic lesion within the mesencephalon ventral to the aqueduct. The lesion, possibly the sequela of a focal contusion, involved both third nerve nuclear complexes and caused paralysis of their voluntary as well as autonomic functions. Gaze mechanisms and long tracts appeared to be less heavily damaged. The literature dealing with third nerve palsy, particularly bilateral cases, from traumatic and other origins is briefly reviewed.

Arachnoid cysts of the middle cranial fossa: experience with 77 cases treated surgically.

Arachnoid cysts of the middle cranial fossa (Sylvian cysts), represent the most common type of intracranial leptomeningeal malformation. Among the 102 intracranial arachnoid cysts operated on at the authors' institution from January 1970 to August 1986, the 77 cases (75%) located in the middle cranial fossa are reviewed. The higher incidence in the first two decades of life (51 cases) as well as the marked predilection for the male sex (60 cases) and the left hemisphere (55 cases) are confirmed in the authors' experience. As for clinical presentation cranial deformities, symptoms of raised intracranial pressure and epilepsy constituted the most frequent features. In 13 patients a complicating lesion was associated: subdural or intracystic haematomas in 7 cases, subdural hygromas in 4 cases and, extradural haematomas in 2 cases. Based on the appearance at CT scan and the results at CT cisternography the authors proposed a classification into three basic types of increasing severity and different pathophysiologic conditions. All the patients underwent craniotomy, excision of the cyst walls and perforation into the basal cisterns. There was one postoperative death (mortality rate of 1.3%) due to meningitis. The remaining clinical results were gratifying in all three types of lesion; on follow-up CT scans the cysts of type I. and II. exhibited a steady tendency to reduction or obliteration while cerebral reexpansion seemed less evident in the third, most severe, type. The authors compare and discuss the options of radical open surgery versus shunting procedures.

Craniopagus twins. An unsuccessful separation and a clinical review of the entity.

Craniopagus twinning is an extremely uncommon birth defect with an estimated incidence of 4-6 every 10 million births. The most complex and challenging issue is the feasibility of surgical separation, which involves not only technical but also socioethical problems and requires strict multidisciplinary cooperation between pediatricians, neuroradiologists, anesthesiologists, and plastic and neurological surgeons. The authors report a case in which separation was followed by the death of both twins and stress the importance, from the surgical and prognostic viewpoints, of the degree of vascular connections between the major dural sinuses. We propose a classification into three types according to severity.

Haemangioblastomas of the brain stem.

Six cases of capillary haemangioblastoma involving the brain stem are presented. Some clinical and pathological peculiarities of this series are outlined. Four patients (two operated radically) showed good or excellent outcome, whereas two (one partial and one complete removal) died shortly after the operation. The chances of direct surgical approach in brain stem haemangioblastomas are considered and the pertinent literature summarized.

Extradural hematoma complicating middle fossa arachnoid cyst.

Two cases of post-traumatic extradural hematoma complicating an arachnoid cyst of the middle cranial fossa in children are described. While subdural and intracystic hemorrhages are well-known complications from this malformation, the association with extradural hematoma has never been previously reported in the literature. The pathogenetic mechanisms are discussed and the particular vulnerability of intracranial arachnoid cysts is stressed.

Benign cerebellar hematomas in children.

Cerebellar hematoma is generally regarded as a rapidly progressive condition which necessitates prompt evacuation in most cases. Unlike adults, where hypertension is the most common etiological factor, children generally have underlying structural lesions (angiomas, tumors) that per se demand surgical intervention. While several reports describe nonsurgical management of cerebellar hematomas in adults, the spontaneous resolution of juvenile cerebellar hematomas is almost unknown. This paper describes a 16-year-old boy with a cerebellar hematoma of obscure etiology that was managed conservatively. This report indicates that nonsurgical treatment of cerebellar hematomas, once structural lesions have been excluded, may be attempted in neurologically stable children.