Surgical Treatment of Rosai-Dorfman Intracranial Disease: Case Report and Literature Review

Rosai-Dorfman disease is a benign lymphoproliferative disease with rare occurrence in the overall population. The characteristic findings of the disease are emperipolesis on microscopy and S-100 protein positivity on immunohistochemistry. The present report describes the case of a 57-year-old man who presented with repeated seizures, urinary retention, and ataxia. Satisfactory results were obtained with surgical treatment. The diagnosis was confirmed by immunohistochemistry.

Primary Central Nervous System ALK Positive Anaplastic Large Cell Lymphoma with Predominantly Leptomeningeal Involvement in an Adult

A 31-year-old Korean male presented with altered consciousness and severe headache. Brain MRI delineated focal leptomeningeal enhancement without any intracerebral lesions. Diagnosis was made based on a brain biopsy showing anaplastic large cell lymphoma (ALCL), immunohistochemical stains revealing positivity for anaplastic lymphoma kinase (ALK) and an absence of involvement in any other organs; specifically, the primary central nervous system ALK+ALCL. Complete remission was achieved following 5 cycles of systemic chemotherapy with a high dose of Methotrexate and a simultaneous 7 cycles of intrathecal triple chemotherapy. Diagnosis of primary leptomeningeal ALK+ALCL is challenging given its rarity and non-specific symptoms along with non-pathognomonic radiologic findings. We present the first case of primary leptomeningeal ALK-positive ALCL where the clinical course, pathologic characteristics and treatment modality are described as well as a review of literature.

F-18 Fluorodeoxyglucose PET/CT and Post Hoc PET/MRI in a Case of Primary Meningeal Melanomatosis

Primary meningeal melanomatosis is a rare, aggressive variant of primary malignant melanoma of the central nervous system, which arises from melanocytes within the leptomeninges and carries a poor prognosis. We report a case of primary meningeal melanomatosis in a 17-year-old man, which was diagnosed with 18F-fluorodeoxyglucose (F-18 FDG) PET/CT, and post hoc F-18 FDG PET/MRI fusion images. Whole-body F-18 FDG PET/CT was helpful in ruling out the extracranial origin of melanoma lesions, and in assessing the therapeutic response. Post hoc PET/MRI fusion images facilitated the correlation between PET and MRI images and demonstrated the hypermetabolic lesions more accurately than the unenhanced PET/CT images. Whole body F-18 FDG PET/CT and post hoc PET/MRI images might help clinicians determine the best therapeutic strategy for patients with primary meningeal melanomatosis.

Trigone ventricular meningiomas: surgical approaches / Meningiomas do trígono ventricular: abordagens cirúrgicas

OBJECTIVE: Report our experience with trigone ventricular meningiomas and review the surgical approaches to the trigone. METHOD: From 1989 to 2006, six patients with meningiomas of the trigone of the lateral ventricles underwent microsurgical resection. Their clinical features, image, follow up, and surgical approaches were retrospectively analyzed. RESULTS: Five patients presented with large and one with small volume meningioma. Unspecific symptoms occurred in three patients; intracranial hypertension detected in three patients; homonymous hemianopsy in three; and motor deficit present in one patient. Three patients were operated by transparietal transcortical approach, two by middle temporal gyrus approach, and one by parieto-occipital interhemispheric precuneus approach. Total resection was achieved in all patients without additional deficits. CONCLUSION: Judicious preoperative plan, adequate knowledge of anatomy, and use of correct microsurgical techniques are fundamental in achieving complete resection of trigone meningioma with low morbidity.

OBJETIVO: Relatar a experiência com seis meningiomas do trígono ventricular e discutir as várias vias de abordagem para o trígono descritos na literatura. MÉTODO: Seis pacientes com meningiomas do trígono ventricular operados entre 1989 e 2006 foram analisados quanto às suas características clínicas, de imagem, evolução e às vias de abordagem. RESULTADOS: Cinco pacientes apresentaram meningiomas de grande volume e um pequeno. Sintomas inespecíficos ocorreram em três pacientes, hipertensão intracraniana em outros três pacientes; hemianopsia homônima em três e déficit motor em um paciente. Três pacientes foram operados por via transcortical transparietal, dois através do giro temporal médio, e um por abordagem interhemisférica precuneus. A ressecção total foi possível em todos os pacientes, sem défices adicionais. CONCLUSÃO: Planejamento operatório cuidadoso aliado ao uso de técnicas microcirúrgicas são fundamentais na ressecção completa dos meningiomas do trígono com baixa morbidade.

Intradural Extramedullary Tuberculoma Mimicking En Plaque Meningioma

A 24-year-old man with tuberculosis meningitis developed acute paraplegia and sensory disturbances 5 weeks after receiving conventional antituberculous therapy. Magnetic resonance imaging revealed an intradural extramedullary long segmental mass mimicking en plaque meningioma at the T2-T6 vertebrae levels. Prompt surgical decompression was performed. A histology examination of the mass revealed a tuberculoma. After surgery, the patient showed improved motor power and a normal bladder function. Intradural extramedullary tuberculoma of the spinal cord is rare complication of tuberculosis meningitis, which can occur as a response to conventional antituberculous therapy.

Meningiomas malignos / Malignant meningiomas

La mayoría de los meningiomas son benignos, aunque el 15 por ciento de todos ellos son considerados malignos, caracterizados por invasión cortical y metástasis a distancia. El objetivo de nuestro trabajo es caracterizar el comportamiento de esta entidad en nuestro centro en cuanto a sus aspectos clínicos, radiológicos, terapéuticos y evolutivos. Realizamos un estudio descriptivo retrospectivo en el período comprendido entre 1997 y el 2007. Nuestra muestra quedó conformada por 7 pacientes, 5 varones y 2 mujeres, con una edad media de 54 años. Las alteraciones clínicas encontradas fueron la cefalea asociada a déficit motor, la epilepsia, el déficit motor, la epilepsia relacionada con el déficit motor y la amaurosis derecha; todos los pacientes se diagnosticaron con tomografía computarizada y sólo a dos de ellos se les realizaron resonancia magnética, 4 meningiomas se localizaron en la convexidad de los hemisferios cerebrales, 2 en la región parasagital y uno en el ala menor del esfenoides, todos los pacientes se trataron quirúrgicamente seguidos por la observación clínica. Se concluye que en nuestra muestra, los meningiomas malignos fueron más frecuentes al final de la quinta década de la vida y en el sexo masculino. La cefalea junto al déficit neurológico focal fueron las manifestaciones clínica más encontrada. La localización en la convexidad cerebral fue la más frecuente. El proceder terapéutico empleado fue la cirugía asociada a la observación clínica. Nuestra recurrencia fue alta, con una sobrevida media de 5 años.

Intracranial Fibroxanthoma in an Infant: a Case Report

A primary fibroxanthoma of the central nervous system is very rare. We present a case of an infantile fibroxanthoma that arose from the cranial dura mater in a six-month-old girl with US, MRI and PET/CT features that mimicked a meningioma. The tumor appeared as a large, well-circumscribed echogenic mass in the right parieto-occipital area on US. The tumor was seen as isoattenuated to slightly hypoattenuated on pre-contrast CT scan and as hypometabolic on PET/CT. As seen on T2-weighted image, the mass was heterogeneously hyperintense to the gray matter. The mass was isointense on T1-weighted image and homogeneously strongly enhanced on contrast enhanced T1-weighted image.

Meningioma do nervo óptico simulando progressão de dano axonal glaucomatoso: relato de caso / Optic nerve meningioma mimicking progression of glaucomatous axonal damage: a case report

O objetivo é relatar um caso de meningioma da bainha do nervo óptico localizado na região do canal óptico simulando progressão de dano axonal glaucomatoso. Paciente de 60 anos em tratamento para glaucoma primário de ângulo aberto apresentou perda progressiva do campo visual e aumento da escavação do disco óptico à esquerda. Devido à rapidez e à assimetria da progressão do dano axonal, por causa da redução da acuidade visual e do aparente bom controle pressórico uma lesão compressiva do nervo óptico foi suspeitada. A tomografia computadorizada de órbitas foi normal, entretanto a imagem por ressonância magnética evidenciou um meningioma da bainha do nervo óptico na região do canal óptico. Afecções compressivas da via óptica anterior são causas incomuns de escavação do disco óptico, entretanto podem simular uma neuropatia glaucomatosa e devem ser lembradas nos pacientes com suspeita de glaucoma de baixa pressão intra-ocular ou em tratamento para glaucoma que apresentem evolução atípica da perda visual.

To report a case of an optic nerve sheath meningioma located at the optic canal area mimicking the progression of a glaucomatous axonal damage. A 60-year-old female patient developed progressive visual field loss and enlargement of the optic disc cup in the left eye while in treatment for primary open-angle glaucoma. Because of the rapid progression of axonal loss, the asymmetry of the visual field defect and the fact that intraocular pressure had been under control, a compressive optic neuropathy was suspected. A computerized tomography scan of the orbit was normal but a high-resolution magnetic resonance imaging demonstrated an optic nerve sheath meningioma located at the optic canal area. Compressive lesions of the anterior visual pathways are uncommon causes of optic disc cupping but should be considered in patients suspected of or undergoing treatment for glaucoma that present with atypical progression of visual loss.

Multiple intracranial meningiomas: diagnosis, biological behavior and treatment / Meningiomas intracranianos múltiplos: diagnóstico, comportamento biológico e tratamento

Multiple intracranial meningiomas (MIM) are a rare pathology when not associated with neurofibromatosis. The prevalence rate of those tumors varied from 2.3 to 8.9 percent of all intracranial meningiomas. OBJECTIVE: To present, analyze and discuss the cases of seven patients diagnosed with multiple intracranial meningiomas, describing their clinical and histological characteristics, as well as their biological behavior. METHOD: The patients records, surgical descriptions, imaging studies and the histopathological exams were retrospectively reviewed. This is the largest series of MIM publisted in Brazil. RESULTS: This sample consists of five women and two men. The age varied from 42 to 84 (average 53.8). Twenty-two meningiomas were identified and varied from 1.5 to 192 cm³, with an 62.8 cm³ average volume. The number of procedures varied from 1 to 5 per patient. No surgical death occurred in this series. CONCLUSION: Despite the multiplicity of tumors, number of recurrences, the new tumors, repeated operations and re-operations, radiotherapy and a number of postoperative complications, the patients experienced a long survival in conjunction with a fairly good quality of life.

Os meningiomas intracranianos múltiplos são tumores raros quando não associados a neurofibromatose, correspondem de 2,3 a 8,9 por cento de todos os meningiomas intracranianos. OBJETIVO: Apresentar, analisar e discutir 7 pacientes com diagnóstico de meningioma intracraniano múltiplo, descrevendo as características clínicas, histológicas e o comportamento biológico desses tumores. MÉTODO: Trata-se de revisão retrospectiva incluindo a análise dos prontuários, das descrições cirúrgicas, dos estudos de imagem e do material histopatológico. Esta série é a maior série de meningiomas intracranianos múltiplos publicada no Brasil. RESULTADOS: A casuística é composta de 5 mulheres e 2 homens. A idade oscilou entre 42 e 84 anos, com média de 53,8 anos. Identificamos 22 meningiomas que variaram de 1,5 a 192 cm³, com volume médio de 62,8 cm³. O número de procedimentos oscilou de 1 a 5 por paciente. Não ocorreu mortalidade cirúrgica. CONCLUSÃO: Apesar de vários tumores, de recorrências, do surgimento de novas neoplasias, de várias cirurgias, re-operações, do tratamento com radioterapia e de inúmeras complicações pós-operatórias, esses pacientes obtiveram uma longa sobrevida com boa qualidade de vida.

Unusual dissemination patterns of low-grade astrocytomas in childhood

CONTEXT: Low-grade astrocytomas are intracerebral lesions of relatively high frequency in the under-18 pediatric population. They often present indolent behaviour, and complete surgical resection is the choice treatment. In cases where the surgery is not possible, chemotherapy and radiotherapy may be used. Medical reports do not recommend examination of the spinal cord at diagnosis or during treatment, since the risk of dissemination of the lesion to the spine is minimal according to medical experience. We describe here four cases of children with low-grade astrocytoma with aggressive dissemination to the neuroaxis.

CONTEXTO: Gliomas de baixo grau de malignidade são lesões intracerebrais relativamente freqüentes na população pediátrica menor de 18 anos de idade. Eles freqüentemente são indolentes em seu comportamento e a ressecção cirúrgica completa é o tratmento de eleição. Nos casos em que a cirurgia não é possível, a quimioterapia e a radioterapia podem ser utilizadas. Relatos da literatura não recomendam a avaliação radiológica da coluna espinhal ao diagnóstico ou durante o tratamento, desde que o risco de disseminação destas lesões para a coluna é considerado mínimo. Descrevemos aqui quatro casos de crianças com gliomas de baixo grau de malignidade com disseminação agressiva para o neuroeixo.