Cerebrospinal fluid dissemination of anaplastic intraventricular meningioma: report of a case presenting with progressive brainstem dysfunction and multiple cranial nerve palsies

Abstract

BackgroundIt is extremely rare to see cerebrospinal fluid dissemination of intraventricular meningioma, particularly with the development of acute, progressive brainstem/cerebellar dysfunction with an absence of mass formation in the corresponding anatomical sites.Case presentationAn 81-year-old man was admitted because of double vision, right facial nerve palsy and truncal ataxia. Brain magnetic resonance imaging showed normal findings except for a tumor mass in the left lateral ventricle, which had been noted over 6 months previously. The patient developed hiccups, hyperventilation, and drowsiness, which worsened progressively, and did not respond to corticosteroid or intraventricular immunoglobulin therapy. Cerebrospinal fluid study revealed a mild elevation of protein, and cytology was negative. The patient died and an autopsy was performed. Postmortem investigation disclosed a malignant transformation of benign fibroid meningioma with cerebrospinal fluid dissemination of the malignant cells, diversely involving the surface of brainstem, cerebellum, and spinal cords, secondarily resulting in extensive ischemia in the brain parenchyma by vessel occlusion.ConclusionIf a patient with an intraventricular tumor develops acute, progressive neurological symptoms, the possibility that it is be caused by cerebrospinal fluid dissemination of tumor cells, after malignant transformation, should be considered.

Anaplastic Intraventricular Meningioma with Rhabdoid Features: An Unusual Tumor with Usual Clinical Presentation

“…Few cases of anaplastic meningiomas have been reported in literature. 5 , 15 - 18 All these anaplastic or grade 3 meningiomas reported have found to be arising from lateral ventricle and commonly from right trigone. This contrasts with the radiological location of present case.…”

“The Silk Road” via subarachnoid cisterns

“…It is well known that neoplastic meningitis is more common with solid carcinoma such as lung, breast and gastrointestinal cancer (46), but literature also reported few malignant meningioma cases with CSF dissemination into the brain ventricles (47). There were only eight cases (47,48) with intracranial or intraspinal malignant meningioma arising from lowgrade meningiomas, which disseminated throughout the CSF (47). In neoplastic meningitis, CSF dissemination of malignant meningioma cells may cause a variety of neurological symptoms such as disturbances of multiple cranial nerves, hydrocephalus, cerebellar dysfunction and multiple spinal nerve roots or cauda equine symptoms (49).…”

“…Brainstem damage by CSF dissemination of malignant cell is rare, literature reporting a few cases with central hyperventilation, Wallenberg syndrome and diplopia, facial nerve palsy and unsteadiness of gait secondary of CSF dissemination of malignant tumour cells (20,47,50).…”

“…It is important to mention, in terms of diagnosis, that only 54% of all cases with leptomeningeal meningiomatous dissemination revealed malignant cells in the CSF on initial lumbar puncture, and only 8% of these cases remained negative, even after repeated examinations (51). The reason for this low specificity of the lumbar puncture remains unclear, although Fujimaki et al speculate the fact that malignant cells adhere rather than float freely in the CSF (47). Although identification of malignant cells by CSF cytology has been considered the diagnostic gold standard, these paradigms have changed nowadays due to limited sensitivity of cytology.…”

Identificação de marcadores tumorais implicados na recorrência tumoral dos meningiomas atípicos

“…Da mesma forma, meningiomas intraventriculares (MIVs) surgem de células aracnóides presentes no plexo coróide(67).Interessantemente, observamos uma proporção maior de subtipos fibrosos nos MIVs, onde a sua frequência foi de 39,7%. Vale ressaltar que os tumores fibrosos, de acordo com nossa revisão, mostraram comportamento mais agressivo do que outros subtipos de meningiomas grau I. Observou-se mais metaplasia maligna e progressão tumoral em tumores originalmente fibrosos(68,69); recorrência tumoral no local cirúrgico (70); carcinomatose no LCR (71) e outros órgãos (72) e associação a presença de hemorragia intratumoral(73)(74)(75)(76). Adicionalmente, os tumores fibrosos apresentaram maior taxa de mortalidade e recorrência tumoral que os demais subtipos de meningiomas.Embora os MIVs apresentem uma localização anatômica peculiar, com íntima relação com os seios venosos e importantes estruturas neuronais, como as vias visuais, que representam um grande desafio para os cirurgiões, o manejo neurocirúrgico tem sido a melhor opção terapêutica para este tipo de tumor(77).…”