Clear-cell meningioma in a 22-month-old male: a case report and literature review.

We report a case of spinal clear-cell meningioma occurring in a 22-month-old male who presented a right limp and then refused to walk. Spinal magnetic resonance imaging demonstrated a large, intradural tumor from T(11) to L(4), which was totally excised. The patient's postoperative recovery was uneventful and 5 months after surgery he began walking again. The latest follow-up magnetic resonance imaging of the brain and spine, obtained 42 months after diagnosis, was negative for tumor recurrence. Though clear-cell meningioma is a rare form of meningioma, it should be considered in the differential diagnosis of any space-occupying lesion of the spine arising in very young children. Complete surgical removal is necessary because it is potentially aggressive and may recur. After surgery, an accurate follow-up is warranted.

[Process of communication in learning groups: a multiprofessional experience]. / Processo de comunicação em grupos de aprendizagem: uma experiência multiprofissional.

A multiprofessional experience carried out during a pedagogic course for health professionals, most of them dentists, to act on the in-service formation of middle level personnel is reported. The course was developed in group, and its task was to learn the problem posing pedagogy. The study was analyzed according to the Pichon-Rivière's theoretical framework. Data analysis has evidenced the close relationship among operativeness and the explicitness of the human relations ghosts, learning difficulties and communication disorders, evidencing the interdependence among communication and learning.

Analyses of prognostic factors in a retrospective review of 92 children with ependymoma: Italian Pediatric Neuro-oncology Group.

The principal aim of this report is to present the results of multivariate analyses conducted to identify clinical prognostic factors in 92 children aged < 16 years with ependymoma (EPD) retrospectively collected in seven Italian centres. They were treated over a 16-year period (1977-1993). Treatment modalities varied. Surgery and radiotherapy (RT) was the "gold standard" management method for the majority of these children. Only in the late 1980s did some of them receive chemotherapy (CT), mainly with vincristine, lomustine (CCNU) and prednisone. The median follow-up of the entire study population is 36 months (average 43 months; range 12 to 214 months). The 10-year overall (OS) and the progression-free survival (PFS) of the study population were 55.5% (CI 41.4-69.4%) and 34.7% (CI 21.4-47.8%), respectively. Age (< 5 years; > 5 years), sex, site (infratentorial vs. supratentorial), histology (anaplastic/malignant vs. non-anaplastic/non-malignant), type of resection (complete vs. incomplete); use and fields of RT, and of CT employed were entered in a multivariate regression model to test their impact on OS and PFS. On univariate analysis, radical surgery, the use of RT and age more than 5 years at the time of diagnosis achieved statistically significant values for predicting long-term OS and PFS. Histology reached marginal statistical significance but only for PFS. When those variables were entered in a multivariate analysis only radical resection (P = 0.00142 and 0.0001) resulted a significant factor for predicting long-term OS and PFS, while the use of RT reached a marginal statistical significance, but only for PFS (P = 0.05). Children who had the tumour completely resected did significantly better than all the others who had less than a complete resection, with a 10-year OS and PFS for the two groups of patients of 69.8% (CI 53-86.5%) and 57.2% (CI 40.3-75%) and of 32.5% (CI 8.5-57.6%) and 11.1% (0-24.4%), respectively. These findings suggest that, for childhood EPD, radical resection should be pursued as much as reasonably possible. Thus, it seems justified proposing for future trials, patient stratification by entity of surgical resection.

Medullocytoma (lipidized medulloblastoma). A cerebellar neoplasm of adults with favorable prognosis.

This study describes three cases of neuroectodermal cerebellar neoplasms occurring in adults, characterized by a monomorphic population of round cells with scanty cytoplasm and focal areas of lipid accumulation. Astrocytic and neuronal differentiation was confirmed in these cells by glial fibrillary acidic protein and synaptophysin immunoreactivity. Electron microscopy performed in two cases showed neuritic processes, synapses, and dense-core granules. Patients included two men and one woman, and the age at diagnosis was 36, 37, and 57 years, respectively. Two patients refused any postoperative treatment. One of these had two surgically removed recurrences after 10 and 11 years and died postoperatively from intracranial hemorrhage. The second had two recurrences after 10 and 15 years and is alive and in good health at the last follow-up. The third patient received postoperative radiotherapy and is alive and well after 2 years. Review of the literature revealed seven cases of cerebellar neoplasms with histological features similar to those observed in our series. These lesions have been considered a variant of medulloblastomas. The age of patients ranged from 42 to 77 years (mean age, 51 years); four were women, 3 men. Follow-up information available in two cases indicates a 5-year survival with surgery alone. These data indicate that these cerebellar neuroectodermal neoplasms have morphologically unique features and indolent biologic behavior that distinguish them from the highly aggressive medulloblastoma; the term medullocytoma for this form is suggested.

CNS-85 trial: a cooperative pediatric CNS tumor study--results of treatment of medulloblastoma patients.

Between 1985 and 1989, 38 children with newly diagnosed medulloblastoma entered our therapeutic protocol. After surgery and postoperative staging assessments, patients were assigned to risk groups. Eleven with "standard-risk" (SR) tumors were treated with radiation therapy alone, while 27 with "high-risk" (HR) tumors received radiation therapy plus adjuvant chemotherapy with vincristine, methotrexate, VM-26, and 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU). After a minimum follow-up of 5 years (range 5-9 years) 21/38 children had developed a recurrence or progression of their disease and 19/38 patients had died. Five-year event-free survival rates and 5-year total survival rates for all 38 patients were 47.4% and 50% respectively. The event-free survival rates at 5 years for SR and HR patients separately were 27.3% and 55.6%, respectively. The corresponding 5-year total survival rates were 27.3% and 59.3%. The differences were not statistically significant. Univariate analysis showed age at diagnosis to be the most important prognostic factor. Infants aged 5 years or less had a significantly shorter event-free survival time than older patients (P = 0.00897). Similar effects were found when total survival time was considered. There were significant differences in outcome in patients receiving different doses of radiation, suggesting a dose-response relationship. A Cox stepwise multivariate analysis showed age at diagnosis as the only independent prognostic factor. Variables relating to treatment entered the model, suggesting that chemotherapy could play an important role in determining outcome.

Primary T-cell lymphoma of the central nervous system.

A series of 6 patients with primary T-cell lymphomas of the central nervous system (PTCLCNS) is presented. The clinical, histological and immunohistological findings are shown. The lesions were infratentorial in the 34% and sopratentorial in the 66% of the cases. Of the 5 cases with an available follow-up only one died in the first year after diagnosis and one is alive and well after 42 months. These data agree with those of others series of PTCLCNS in the literature. In conclusion our data and those of the literature suggest that immunophenotyping should routinely be carried in primary non-Hodgkin's lymphomas of the CNS (PNHLCNS), because the T-cell forms run a more favourable course than the B-cell varieties.

Non-Hodgkin lymphomas of the central nervous system. Clinico-pathologic and immunohistochemical study of 147 cases.

We report on data gathered from five European centres regarding 147 primary non-Hodgkin Lymphomas (NHLs) of the Central Nervous System (CNS) in HIV-negative patients. The results lead us to make the following considerations: i) there has been a significant and progressive increase in the frequency of observation of this pathology during the course of the last two decades; ii) the pathology lacks specific characteristic symptoms; iii) the radiological profile, as observed by CAT and/or MNR, most frequently corresponds to an isodense or slightly hyperdense lesion which has clear margins and is capable of assuming the contrast medium homogeneously; iv) the tumour most often has a single supratentorial localisation; v) high grade B-cell lymphomas account for 66% of the observations, low grade B-cell varieties being relatively rare and cases of T-cell derivation exceptional (6/147); vi) immunohistochemistry allows the differential diagnoses with respect to primitive or secondary non-lymphoid tumours, and provides confirmation of the histogenetic assessment made on morphological grounds; vii) the course of the disease is not significantly influenced by the histotype, the phenotype, the number of lesions present or the chemotherapy regimen, but rather by the employment of combined surgery and radio- or radiochemotherapy. This study represents the largest series of CNS NHLs so far reported, and as such, provides precise clinico-pathological indications which were only partially obtainable from the relatively small previously published series. Some concluding remarks are made as to the genesis of CNS NHLs, along with some practical suggestions for reaching a better understanding of their complex biology.

Large-cell medulloblastomas. A distinct variant with highly aggressive behavior.

We present four cases of infantile cerebellar neoplasms composed of cells with large vesicular nuclei with prominent nucleoli. All four cases were strongly immunoreactive for synaptophysin, and one case showed immunoreactivity for neurofilaments. Filter hybridization for N-myc and c-myc oncogenes showed a 27-fold c-myc amplification in one case. The cytogenetic analysis in this case showed Double-Minutes and isochromosome 17q. An intracerebral xenograft in nude mice obtained from one such tumor showed a similar morphology to that of the original tumor as well as strong immunoreactivity for synaptophysin and neurofilaments. All the neoplasms were characterized by highly aggressive behavior leading to early cerebrospinal fluid dissemination despite radiotherapy and chemotherapy. We conclude that large-cell medulloblastoma represents a distinct and more aggressive variant of medulloblastoma that requires more aggressive therapy.

Cytogenetic t(11;17)(q13;q21) in a pediatric ependymoma. Is 11q13 a recurring breakpoint in ependymomas?

Cytogenetic studies on a supratentorial ependymoma from a 1-year-old boy showed a t(11;17)(q13;q21). This is the second ependymoma reported with a rearrangement at 11q13; to our knowledge the 11q13 is the first recurring breakpoint reported in ependymoma.

Primary intrasellar coccidioidomycosis simulating a pituitary adenoma.

The case of a 68-year-old woman who had relatively acute, unilateral ophthalmoplegia is reported. Radiological studies indicated a mass lesion involving the pituitary gland and left cavernous sinus. Pathological tissue obtained by the transsphenoidal approach revealed the presence of a Coccidioides granuloma. This pathological entity should be considered when evaluating patients with a pituitary mass and ophthalmoplegia.

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.

[Ischemic cerebrovascular accidents in childhood. Problems of neuroradiological diagnosis]. / Incidenti cerebro-vascolari ischemici in età pediatrica. Problemi di diagnosi neuroradiologica.

The authors present 19 cases of stroke with subsequent hemiplegia in children from 5 months to 15 years of age. These cases are not strictly correlated with haematologic alterations. The aetiopathogenesis remains uncertain in the most of patients, in which predisposing causes are absent and angiographic pictures do not demonstrate anatomical lesions or occlusions of cerebral vessels. In our series, cerebral angiography was performed in 16 children and showed vascular alterations only in 6 cases. CT scan some time after the acute hemiplegia, demonstrated low-density areas in 12 cases over 17 examined, with the characteristic evolution of ischemic lesions.

Multiple meningiomas.

The authors discuss the criteria of differential diagnosis for a correct use of the term "multiple meningiomas". Reviewing a series of 934 meningiomas, of which 834 were intracranial and 100 were spinal, they found 14 cases of multiple meningiomas, i.e. an incidence of 1.5%. The study shows that multiple meningiomas are quite similar to solitary meningiomas in all their biological characteristics. Furthermore, the possible presence of more than one meningioma must always be kept in mind in the clinical and radiological evaluation of a patient. The aetiology of multiple separate meningiomas is discussed: the hypothesis which suggests their origin from multicentric neoplastic foci activated by a supposed "tumour-producing factor" appears to be the most reliable.

An unusual onset of a spinal cord tumour: subarachnoid bleeding and papilloedema. Case report.

A rare case of intracranial hypertension associated with subarachnoid bleeding from a spinal tumour is reported. Clinical features, review of the literature and pathogenetic factors are discussed.

Tapia's syndrome caused by a neurofibroma of the hypoglossal and vagus nerves: case report.

Tapia's syndrome is characterized by unilateral paralysis of the tongue and vocal cord, and is caused by a lesion of the 10th and 12th cranial nerves below the nodose ganglion, without involvement of the pharyngeal branches of the 10th nerve. The authors report the case of a 25-year-old man who presented with a 4-year history of progressive glossolaryngeal paralysis. Operation through the right laterocervical region allowed complete removal of a neurofibroma involving the 10th and 12th nerves at their crossing below the nodose ganglion.

[Spinal epidural empyema. Study of a clinical case]. / Empiema epidurale spinale. Studio di un caso clinico.

A case of epidural spinal abscess is reported. This rare lesion provoked a syndrome of thoracic spinal compression without biohumoral or anamnestic findings of phlogosis. After a brief review of literature, the Authors discuss the usefulness of cortical evoked responses in presence of clinical cross section of the spinal cord.

Pituitary abscesses: report of two cases and review of the literature.

Two cases of pituitary abscess are reported. Bacteriological and histological findings of both cases are discussed, together with data available from the literature.

Anterior optic gliomas with precocious or pseudoprecocious puberty.

5 patients with gliomas of the anterior optic pathways, histologically assessed or with strong diagnostic evidence, who developed precocious or pseudoprecocious puberty (2 cases) before the operation or after radiation treatment, are presented. Only 2 patients presented familial and/or personal evidence of neurofibromatosis. The production mechanism of these sexual changes is discussed.

[Value of computerised tomography in the diagnosis of colloid cyst of the third ventricle (author's transl)]. / Validità della tomodensitometria computerizzata nella diagnosi della cisti colloide del terzo ventricolo.

The so called colloid cyst of the third ventricle is believed to originate from the neuroepithelium in the diencephalic roof. The diagnosis is notoriously difficult and contributes significantly to the high mortality. A case in an eleven-year-old boy is reported. Computed tomography, also because not invasive, is chiefly indicative in this lesion, reducing the overall mortality.