Accessible online at: www.karger.com/pne Dear Sir, We would like to give an update on the clinical history of the patient with clear cell meningioma (CCM) whom we recently reported [1]. Briefly, a 22-month-old male was referred to our child neurology division for walking impairment persisting for the previous 4 months. Spinal magnetic resonance imaging (MRI) revealed a large, intradural mass extending from T11 to L4 and involving the intravertebral foramina from L2 to L4. The tumor was removed completely and histopathology revealed a CCM. The patient’s postoperative recovery was uneventful. His motor skills progressively improved after surgery, and 5 months later, he started walking again. Five years later, the patient complained of headache and back stiffness; the headache occurred daily, usually in the morning, with spontaneous resolution after 1–2 min. The

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 T11 to L4, 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.

Mechanical obstruction or infection are the commonest and most widely feared complications of ventriculoperitonenl shunting. Nowadays third ventriculostomy (TVS) constitutes an alternative to shunt revision in patients with third ventricular hydrocephalus. We describe five children treated by TVS with a satisfactory outcome.

Il malfunzionamento degli shunt da cause meccaniche o infettive rappresenta la complicanza più frequente e temibile delle derivazioni ventricolo-peritoneali. La terzoventricolostomia (VTS) rappresenta oggi un'alternativa alla revisione dello shunt, nei pazienti con idrocefalo triventricolare. Vengono descritti cinque casi in era pediatrica trattati con TVS con soddisfacenti risultati.

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.

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.

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.

La patologia midollare, sia di tipo malformativo che espansivo, può presentare nella prima infanzia una sintomatologia subdola, talora atipica, che può rendere difficile la diagnosi eziologica. Riportiamo 2 nuovi casi di cisti midollari in età pediatrica, non associate a malformazioni spino-vertebrali, che hanno presentato difficoltà diagnostiche sia cliniche che neuroradiologiche.

Spinal malformation or compressive lesions in infancy may present with insidious, sometimes atypical symptoms which hinder accurate diagnosis.We describe two children with intraspinal cysts unaccompanied by spinovertebral deformity which were difficult to diagnose clinically and neuroradiologically.Two 19-month-old infants were referred to us. One had worsening pain in the head and neck and was forced to lie on his side; the other infant had non-epileptic atonic seizures in his legs, flexion contractions in the head and arms and subsequent spastic paraparesis in the course of meningococcal sepsis without meningitis. The first infant had a cervical extramedullary intradural neuroenteric cyst; the second had an isolated extramedullary intradural subarachnoid cyst in the thoracic region. In both cases diagnosis was established by combined MR and myelo-CT investigation. Surgical removal resolved symptoms in both children.Though rare, spinal cysts should be suspected when infants present with spinal pain and/or impairment of uncertain origin. MR with or without myelo-CT is useful in identifying these lesions.

Dopo aver passato in rassegna le diverse classificazioni cliniche, anatomo-patologiche e radiologiche ritrovabili in letteratura, gli autori propongono una nuova classificazione neuroradiologica di tipo semeiologico delle raccolte liquorali intracraniche. Essa è basata essenzialmente sull'analisi della situazione encefalica nel suo complesso, con particolare riferimento al parenchima cerebrale, identificando 5 gruppi di patologie: a) raccolte liquorali da variante di sviluppo di strutture parenchimali; b) raccolte liquorali da malformazione primitiva del parenchima cerebrale; c) raccolte liquorali da perdita di tessuto parenchimale già formato; d) raccolte liquorali da anomalie neuroepiteliali; e) raccolte liquorali da malformazione primitiva delle membrane meningee. Sull'inquadramento generale cosi effettuato si inseriscono poi i segni diretti e indiretti derivati dalla semeiotica tradizionale, riconoscibili per tutti i tipi di lesione, che tengono conto sia delle caratteristiche di segnale (RM) o densità (TC) inerenti alla tecnica usata, sia della situazione di riequilibrio o scompenso della raccolta liquida in rapporto con le altre strutture: scatola cranica, parenchima cerebrale, comparto ventricolo-cisterno-sulcale. L'analisi cosi effettuata dovrebbe meglio mettere in sintonia il quadro neuroradiologico con quello clinico e anatomo-patologico.

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.

Un neonato, che l'ecografia fetale aveva già indicato affetto da idrocefalo, e stato studiato dopo la nascita mediante ultrasonografia transfontanellare che ha rilevato una cisti liquorale in fossa cranica posteriore, non comunicante col IV ventricolo, ed un idrocefalo triventricolare. Le ecografie successive dimostravano un progressivo aumento delle dimensioni dei ventricoli e della cisti. La TC cerebrale confermava la grossa cisti sottotentoriale che comprimeva posteriormente il cervelletto ipoplasico. Il piccolo è stato operato mediante unishunt cisto-peritoneale. I controlli ecografici seriati hanno mostrato una sorprendemente rapida riduzione delle dimensioni della cisti e dei ventricoli cerebrali, fino a completa remissione del quadro patologico. Questo lavoro vuole sottolineare come l'ecografia cerebrale sia del tutto affidabile per la diagnosi delle cisti liquorali in FCP e rappresenti il metodo ideale per seguire l'evoluzione delle cisti e dell'eventuale idrocefalo prima e dopo il trattamento chirurgico.

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.