Suprasellar/pineal bifocal germ cell tumors.

PURPOSE: Intracranial germ cell tumors (GCT) arise from embryonal rests of germinal cells. The aim of this report is to analyze a small group of GCT located simultaneously in the suprasellar and pineal regions without seeding either between both tumors or to other places. We named this group as suprasellar/pineal bifocal germ cell tumors (SPBT). METHODS: A retrospective review of a series of 25 GCT showed a) 16 cases of unifocal non-disseminated pineal or sellar GCT, b) one case of unifocal disseminated pineal GCT, c) three cases with suprasellar and pineal double tumors with dissemination, and d) five cases with SPBT. The analysis is focused on the latter group. RESULTS: The series includes four pure germinomas and one germinal non-germinoma. MRI and endoscopic exploration were necessary to define SPBT. Endocrine, ocular, and increased intracranial pressure syndromes were identified and related to the size of the tumors. Chemotherapy and radiotherapy were performed in all SPBT. Radical or partial resection of SPBT offered no benefits over biopsy. Prognosis for bifocal groups was similar to unifocal tumors of the same histological type. Complete remission without recurrence and mortality were achieved in all cases. CONCLUSIONS: SPBT seem to be an entity defined by a) one tumor in the suprasellar and another in the pineal region, b) GCT with predominance of PG, but not exclusively, and c) MRI and endoscopy without any dissemination. The presence of two tumors does not indicate dissemination; SPBT were non-disseminated but focal tumors, and spinal radiotherapy was not necessary.

Lesiones intramedulares que simulan tumor / Spinal cord lesions that mimic neoplasms

Objetivo. Analizar las características clínicas y neurorradiológicas de las distintas lesiones intramedulares con la finalidad de poder diferenciar las lesiones no-tumorales de las tumorales y reservar la exploración quirúrgica sólo para aquellos pacientes sin diagnósticoy con sintomatología rápidamente progresiva. Material y métodos. Se revisaron las historias clínicas de 44 pacientes operados consecutivamente con diagnóstico presuntivo de tumor intramedular entre enero de 1988 y diciembre de 2007. No se incluyeron en el análisis los tumores bulbomedulares ni los del cono y filum ni tampoco los lipomas, teratomas y quistes dermoides.Resultados. Se identificaron 4 pacientes (9.1%), 3 masculinos y 1 femenino, con edad media de 9 años, que presentaban deterioro neurológico progresivo e IRM espinal compatible con tumor intramedular. El resultado histopatológico confirmó en los cuatro casos lesiones no tumorales: infiltrado inflamatorio inespecífico, edema, micosis y gliosis respectivamente.Conclusiones. El minucioso análisis clínico, de laboratorio y el estudio de las imágenes en IRM puede ayudar a la correcta distinción entre tumores y lesiones no-tumorales evitando la exploración quirúrgica que sólo está justificada en los casos muy dudososacompañados de rápido deterioro neurológico funcional del paciente.(AU)

Objective. To analyze the clinic and neuro-imaging characteristicsof the intra spinal lesions in order to differentiate the non-neoplasticlesions from neoplastic lesions and to spare surgical exploration only for non-diagnosed patients with rapid deterioration. Materials and method. Fourty-four medical charts of patientsoperated on spinal cord lesions between January 1988 and December 2007 have been analyzed. Results. In four patients (3 male, 1 female ¹ mean age: 9 years)who were operated with diagnosis of intra-spinal tumor, their lesions turned out to be non-neoplastic: non-specific inflammation, edema, mycosis and gliosis, respectively. Conclusions. The thorough clinical and laboratory investigation together with the study of the imaging can help distinguishbetween non-neoplastic lesions from neoplastic lesions in order to avoid surgical exploration which is only suitable in very doubtful cases with rapid deterioration.(AU)

Lesiones intramedulares que simulan tumor / Spinal cord lesions that mimic neoplasms

Objetivo. Analizar las características clínicas y neurorradiológicas de las distintas lesiones intramedulares con la finalidad de poder diferenciar las lesiones no-tumorales de las tumorales y reservar la exploración quirúrgica sólo para aquellos pacientes sin diagnósticoy con sintomatología rápidamente progresiva. Material y métodos. Se revisaron las historias clínicas de 44 pacientes operados consecutivamente con diagnóstico presuntivo de tumor intramedular entre enero de 1988 y diciembre de 2007. No se incluyeron en el análisis los tumores bulbomedulares ni los del cono y filum ni tampoco los lipomas, teratomas y quistes dermoides.Resultados. Se identificaron 4 pacientes (9.1%), 3 masculinos y 1 femenino, con edad media de 9 años, que presentaban deterioro neurológico progresivo e IRM espinal compatible con tumor intramedular. El resultado histopatológico confirmó en los cuatro casos lesiones no tumorales: infiltrado inflamatorio inespecífico, edema, micosis y gliosis respectivamente.Conclusiones. El minucioso análisis clínico, de laboratorio y el estudio de las imágenes en IRM puede ayudar a la correcta distinción entre tumores y lesiones no-tumorales evitando la exploración quirúrgica que sólo está justificada en los casos muy dudososacompañados de rápido deterioro neurológico funcional del paciente.

Objective. To analyze the clinic and neuro-imaging characteristicsof the intra spinal lesions in order to differentiate the non-neoplasticlesions from neoplastic lesions and to spare surgical exploration only for non-diagnosed patients with rapid deterioration. Materials and method. Fourty-four medical charts of patientsoperated on spinal cord lesions between January 1988 and December 2007 have been analyzed. Results. In four patients (3 male, 1 female – mean age: 9 years)who were operated with diagnosis of intra-spinal tumor, their lesions turned out to be non-neoplastic: non-specific inflammation, edema, mycosis and gliosis, respectively. Conclusions. The thorough clinical and laboratory investigation together with the study of the imaging can help distinguishbetween non-neoplastic lesions from neoplastic lesions in order to avoid surgical exploration which is only suitable in very doubtful cases with rapid deterioration.

Neuroendoscopia guiada por neuronavegación / Neuroendoscopy guided by neuronavigation

Objetivo. Describir y analizar una serie de pacientes en los que se utilizó neuroendoscopía guiada por neuronavegación para la resolución de diferentes patologías neuroquirúrgicas. Material y método. Se realizó una análisis retrospectivo de las historias clínicas de los pacientes en los que se combinaron la utilización del neuroendoscopio de óptica rígida de 0° o 30° STORZ-RECQ y de los neuronavegadores (BRAIN LAB Vectorvisión II ó Electra). Resultados. Desde abril de 2001 hasta diciembre de 2006, operamos 14 pacientes combinando ambas tecnologías. Las edades de los pacientes fueron de entre 10 meses y 10 años (promedio 7.5 años). Las patologías que motivaron la cirugía fueron: seis quistes aracnoideos próximos a ó dentro de ventrículos pequeños, cuatro tumores hipotalámicos, tres ventrículos excluidos y una cavidad porencefálica. La precisión del procedimiento de neuronavegación fue siempre inferiora 1mm, sin embargo no pudimos acceder al sistema ventricular en uno de los catorce pacientes. En los trece restantes se logró el planeamiento prequirúrgico en forma adecuada. Conclusión. La neuroendoscopia guiada por estereotaxia sin marco con neuronavegador es una técnica precisa y segura cuando la patología es solucionable endoscópicamente y los ventrículos son pequeños.

Pinealoblastomas in children.

INTRODUCTION: Series of pinealoblastomas (PB) usually comprise small number of cases as this tumor type is extremely rare and occurs mainly in childhood (especially under 9 years of age). Frequently, PB are reported together with others pineal parenchymal tumors (PPT) or pineal tumors, making characterization far from adequate. MATERIALS AND METHODS: Our series of CNS pediatric tumors comprises 1,350 cases of whom 16 are PPT, 12 PB, two pineocytomas (PC), and two mixed or transitional tumors (PC/PB). We have only analyzed the PB considering clinical features, treatment strategy, prognosis, recurrences, and mortality. RESULTS: PB represented 0.89%. Mean age was 7 years. Male-female ratio was 8/4. All patients complained of increased intracranial pressure, eight presented ocular symptoms, two cerebellar, and one endocrine disturbances. Patients underwent CT scans and/or MRI. All children had negative serum and CSF markers and only one case had positive tumor cells in the CSF on admission. Hydrocephalus (12/12) was treated with ventriculoperitoneal shunt in 11/12 and endoscopic third ventriculostomy (ETV) in 1/12. We performed 11 surgical procedures (seven by occipital transtentorial approach) and one endoscopic biopsy. Total removal was achieved in two, partial removal (50-90%) in seven, and biopsy in three patients or <50%. Adjuvant therapy included radiotherapy and chemotherapy. Recurrences appeared in 8/12 cases (mean time of recurrence=27.28 months). Six patients died (mean survival=29.55 months). Mean follow up for the six patients alive was 54 months and mean follow up for all 12 children was 38.7 months. CONCLUSION: In our opinion, PB have a poor prognosis and are very aggressive, especially in small children. Survival rate at 1 and 5 years in the present series is 66.6% (8/12) and 50% (6/12), respectively. We propose an algorithm for the treatment of pediatric patients with PB.

Subependymal giant cell astrocytoma in children with tuberous sclerosis.

METHODS: Out of 105 patients with tuberous sclerosis (TS) admitted to the Hospital Nacional de Pediatría "Juan P. Garrahan" (Buenos Aires, Argentina), we surgically treated 17 children between January 1988 and December 2000. Two patients were operated on because of epilepsy and 15 patients because of an intraventricular tumor (subependymal giant cell astrocytoma [SGCA]). In this report we focus on tumors. Twelve of the 15 patients presented with hydrocephalus but none of them had a preoperative shunt. All tumors were surgically resected using frontal transventricular or transcallosal routes. Total removal was achieved in 12 out of 15 and subtotal removal in 3 out of 15 patients (resection of 70-95%). RESULTS: Exeresis of the tumor was not accompanied by significant morbidity and there was no perioperative mortality. Seizures and mental retardation did not improve after tumor resection. It was necessary to insert a postoperative shunt a long time after surgery in only one patient. There were no recurrences of SGCA that were totally removed after a mean follow-up of 51.7 months. CONCLUSIONS: We encourage surgery as soon as a lesion is diagnosed as a tumor. The "transformation" of subependymal nodules (SEN) into tumors (SGCA) may be considered controversial.

Pediatric granulomatous cerebral amebiasis: a delayed diagnosis.

We present four cases of cerebral amebae infection treated at our neurosurgical department. Patient 1 was a 12-year-old male with skin lesions of 2 years' progression involving the midface. He received a corticosteroid course, and, after that, he presented a right body hemiparesis. Patient 2 was a 5-year-old male, with a past surgical history of fibula fracture and osteomyelitis of 1-year evolution, associated with lesions of the surrounding skin that presented with partial seizures. Patient 3 was a 3-year-old female who presented with a stroke-like episode and with partial seizures. Patient 4 was a 6-year-old male who had ulcerative lesions in the face of 1-year evolution. After a corticosteroid course, he presented with right-body hemiparesis. All patients were human immunodeficiency virus-negative and died 1 month or less after surgery because of progressive evolution of the disease. Histopathology revealed granulomatous amebic encephalitis. All patients revealed infection from Balamuthia mandrillaris (Leptomyxiidae). Treatment consisting of pentamidine, clarithromycin, fluconazole, and 5-fluorocytosine was ineffective. Although extremely uncommon, granulomatous amebic encephalitis should be considered in the differential diagnosis of cerebral lesions while nonspecific, associated granulomatous skin lesions support the diagnosis of amebiasis.