Cerebrospinal fluid dissemination of anaplastic intraventricular meningioma: report of a case presenting with progressive brainstem dysfunction and multiple cranial nerve palsies
© The Author(s). 2016
Received: 19 June 2015
Accepted: 27 May 2016
Published: 31 May 2016
It 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.
An 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.
If 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.
KeywordsBrainstem dysfunction Multiple cranial nerve palsies Cerebrospinal fluid dissemination Anaplastic meningioma
Intraventricular meningioma is a rare neoplasm, representing only 0.5–3 % of all intracranial meningiomas . Moreover, it is extremely rare to see a metastasis of meningioma cells (malignant meningioma), via cerebrospinal fluid (CSF) dissemination, involved in the diverse central nervous system (CNS) structures, such as cerebellum, multiple cranial nerves, spinal nerve roots and the cauda equina [2–4]. Recently, we encountered a patient who developed acute progressive decline of brainstem function caused by the CSF dissemination of intraventricular malignant meningioma. Here, we present the clinical and pathological data of the patient.
Generally, neoplastic meningitis is not uncommon with solid carcinoma, e.g., breast cancer, lung cancer, and gastrointestinal cancer ; however, it is extremely rare to see the CSF dissemination of intraventricular malignant meningioma. To our knowledge, only eight such cases have been hitherto reported . In these cases, tumor mass of meningioma in the brain or spinal cord was found transformed from low-grade meningioma to malignant meningioma, eventually resulting in the CSF dissemination of malignant tumor cells. In the present patient, the solitary tumor in the lateral ventricle contained mixed pathology of benign and malignant parts originated from the common cellular profile of the meningioma. Of note, unlike the previously reported cases, neuroimaging studies could not demonstrate any tumor mass suggestive of metastasis in the present patient. This may be one reason for the delay in the clinical diagnosis despite such widespread dissemination of tumor cells. CSF dissemination of malignant tumor cells may potentially cause a wide variety of neurological symptoms, such as hydrocephalus, cerebellar dysfunction, disturbances of multiple spinal nerve roots or the cauda equina, and cranial nerve palsy . Conversely, brainstem dysfunction by CSF dissemination of malignant cells is rare; only a few reports are available in the literature in which Wallenberg syndrome or central hyperventilation was induced by CSF dissemination of malignant tumor cells [8, 9]. In the present patient, although marked and progressive decline of brainstem function was observed, an accurate diagnosis could not be made until the post-mortem examination, suggesting that it is by no means easy to make a prompt diagnosis of this type of clinical condition.
Clinically, peripheral facial nerve palsy, diplopia and unsteadiness of gait were the initial manifestations in the present patient. These symptoms, if they occur acutely, risk misleading the differential diagnoses towards more frequent clinical conditions, such as brainstem encephalitis or Fisher syndrome . Unfortunately, CSF cytology, which was performed repeatedly, did not identify any malignant cells in the present patient. Although the specificity of CSF cytology is high, the sensitivity is low, between 45 and 95 % . Reportedly, in cases of leptomeningeal metastasis, malignant cells were identified in the CSF in 54 % of cases on initial lumber puncture, and remained negative in 8 %, even after repeated examination . The reason for the low specificity of the CSF cytology in leptomeningeal metastasis remains unclear regardless of extensive seeding of the malignant cells via the CSF. We speculate that the malignant cells may have a propensity to adhere easily to the meninges rather than floating freely in the CSF.
The pathology showed that the malignant part of this tumor was positive for cytokeratin. Cytokeratin, a known marker of metastatic carcinoma, was expressed only in cells of the malignant part. This finding did not rule out the diagnosis of meningioma because cytokeratin expression was also detected in 75 % of malignant meningiomas .
Although it is certainly not easy to make a precise diagnosis of this type of clinical condition, it is clinically important to note that when seeing a patient with an intraventricular tumor in whom any neurological symptoms appeared progressively, the possibility should be kept in mind that CSF dissemination of tumor cells, after malignant transformation, might be the potential cause, even if CSF cytology is negative.
CSF, cerebrospinal fluid; CNS, central nervous system; MRI, magnetic resonance imaging; sIL2-R, Soluble interleukin 2-receptor; ACE, angiotensin-converting enzyme; ANCA, anti-neutrophil cytoplasmic antibody; AQP-4, anti-aquaporin-4; IVIg, intravenous immunoglobulins; DWI, diffusion-weighted image; FLAIR, fluid attenuated recovery; HE, hematoxylin-eosin; EMA, epithelial membrane antigen; GFAP, glial fibrillary acidic protein
We thank Dr. Kanako Ogura and Prof. Toshiharu Matsumoto, Department of Pathology at Juntendo University Nerima Hospital, for the general pathological study.
Availability of data and materials
The dataset supporting the conclusions of this article is included within the article.
MF performed data acquisition, analyzes and interpreted the data, and drafted the manuscript. MT performed the pathological analysis and revised the manuscript. AK performed molecular and genetic analysis. MK, KY, and YM performed data acquisition. NH revised the manuscript. HM contributed to conception and design, data acquisition, data analysis and interpretation, and manuscript revision. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
Consent for publication
Written informed consent for publication of this case report and any accompanying images was obtained from the patient’s family because he had passed away.
Ethics approval and consent to participate
The authors declare that ethics approval was not required for this case report.
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