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| Year : 2001 | Volume
: 49
| Issue : 4 | Page : 398-400 |
Mixed tumour of schwannoma and meningioma in a patient with neurofibromatosis-2 : a case report.
Elizabeth J, Menon G, Nair S, Radhakrishnan VV
Department of Pathology, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Trivandrum - 695011, India.
Correspondence Address:
Department of Pathology, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Trivandrum - 695011, India.
The co-existence of schwannoma and meningioma as a mixed intracranial tumour is uncommon and so far only eight cases have been published in the literature. Because of rarity, we report a unique case of mixed tumour having schwann cell and meningeal components, in a patient with neurofibromatosis type -2 (NF-2). The possible mechanisms for the occurrence of these mixed tumours are discussed.
How to cite this article:
Elizabeth J, Menon G, Nair S, Radhakrishnan V V. Mixed tumour of schwannoma and meningioma in a patient with neurofibromatosis-2 : a case report. Neurol India 2001;49:398-400 |
How to cite this URL:
Elizabeth J, Menon G, Nair S, Radhakrishnan V V. Mixed tumour of schwannoma and meningioma in a patient with neurofibromatosis-2 : a case report. Neurol India [serial online] 2001 [cited 2023 Mar 21];49:398-400. Available from: https://www.neurologyindia.com/text.asp?2001/49/4/398/1212 |
Neurofibromatosis-2 (NF-2) or central von Recklinghausen's disease is an autosomal dominant hereditary disease affecting 1 in 50,000 people.[1] These patients usually present with multiple intracranial and intraspinal neoplasms, the most common being schwannoma, glioma and meningioma. Approximately, 50% of the patients with NF-2 manifest with intracranial meningioma and more than 30% present with multiple non-vestibular schwannomas. But the co-existence of meningioma and schwannoma as two distinct histology components within the same tumour in patients with NF-2 is extremely uncommon. The review of English literature till August 1999 revealed only [eight] documented cases. We report here a mixed tumour composed by schwann cell and meningeal components in a twenty six year old male who had features of NF-2.
A 26 year old male who had deafness of right ear since birth presented with a history suggestive of raised intracranial pressure of three months duration. General physical examination showed multiple subcutaneous nodules. Biopsy from one of the subcutaneous nodules showed features of a schwannoma. He had no other neurological deficits except for sensory neural deafness in the right ear. Family history was negative for von Recklinghausen's disease. Computed tomography (CT) revealed a large hyperdense enhancing lesion in the right cerebellopontine angle with broad basal attachment to the petrous apex. Magnetic resonance imaging (MRI) revealed areas of calcification as hypointense areas while the remainder of the tumour showed intense gadolinium enhancement [Figure. 1]. The tumour was seen to extend into the supratentorial compartment. The [eighth] cranial nerve was not seen distinctly and there was a small intrameatal component of the tumour. MRI features were interpreted as schwannoma and the patient was operated through suboccipital craniotomy. Peroperatively, the lesion was hard, calcified, densely attached to the dura over petrous bone but had a good plane of cleavage. The tumour was arising from the right vestibulocochlear nerve. The other cranial nerves were not involved. A near total microsurgical excision could be achieved except for the densely calcified portion, which burrowed into the peterous apex. The post operative period was uneventful. The patient was discharged on the tenth post operative day. He was attending the post-operative follow-up clinic for one year and had no recurrence of the tumour.
The specimen was sent for routine histopathological examination. Multiple paraffin sections were studied using haematoxylin and eosin. The sections showed a tumour composed by two histological components.
Interlacing bundles of spindle cells were the predominant component; the nuclei were arranged in periodic rows (Antoni A). Imperceptibly merging with this were loosely textured sparsely cellular zones composed of stellate cells (Antoni B). Numerous discrete concentric lamellated psammoma bodies composed the second component within the tumour with cells having moderate eosinophilic cytoplasm and round nucleus arranged in whorls and was interpreted as transitional meningioma. These two components were clearly demonstrated in several microscopic fields in almost all the paraffin sections [Figure - 2]. Immunohistochemical stain revealed S-100 protein positive cells in the schwannoma component only.
Neurofibromatosis (NF) manifests in two distinct forms: NF-1 (peripheral neurofibromatosis) and NF-2 (central neurofibromatosis). NF-2 is characterised by bilateral acoustic schwannoma, meningioma, astrocytoma and extracranial schwannomas. The coexistence of intracranial tumours of diverse histological types is a criterion for the diagnosis of NF-2, if only one vestibular schwannoma is present.[2] Cushing and Eisenhardt[1] reported cases of mixed schwannoma and meningioma in their classic monograph on meningiomas. Davidoff and Martin[3] reported two familial cases of bilateral cerebellopontine angle tumour showing schwann cell and meningeal components. Geddes et al[4] reported five cerebellopontine angle tumours from four patients with NF and three biopsy specimens appeared to be histologically mixed tumours. In one patient, arachnoidal proliferation around schwannoma resulted in the formation of multiple 'micromeningiomas'. Kim et al[5] reported a mixed tumour containing schwann cell and meningeal
components in an eighteen year old female patient. In all these cases, the precise mechanism for the tumourogenesis of mixed tumours was not defined and they were essentially documented as unusual histological combinations.
However, Kim et al[5] postulated two possible mechanisms for the occurrence of mixed tumour in patients with NF-2. The first postulation was that the collision between two different components of tumours and that the two components within the tumour developed independently with a time lag difference. The second hypothesis was the bidirectional differentiation from a common progenitor cell. However, the consensus is that, occurrence of two histological components within the same tumour is a collision phenomenon rather than bidirectional differentiation because there is no neurobiological evidence for a common progenitor of schwann cells and meningothelial cells. Kepes and Kernohan[6] regarded that apparently one tumour containing areas resembling another, rather than a combination of two could explain mixed schwannoma meningioma. In our case, the two components of the tumour were not distinct on morphological grounds, but also by immunostaining characteristics. S-100 immunostain was positive in schwannomatous zones and negative in meningothelial foci. The presence of heterologous foci of meningothelial cells in NF-2 has also been attributed due to metaplasia. Geddes et al[4] considered an alternative possibility of reactive meningothelial hyperplasia adjacent to the tumour could be responsible for the occurrence of meningothelial component in these tumours. They also observed that arachnoidal proliferation appears to be more exuberant in bilateral acoustic neurofibromatosis than in sporadic acoustic schwannomas, possibly as a result of a disease associated growth factor, produced locally by the schwann cells.
| 1. | Cushing H, Eisenhardt L : In: Meningiomas, Hafner, New York. 1938; 100-114.  |
| 2. | Ian F. Pollack, John J Mulvihill : Neurofibromatosis 1 and 2. Brain Pathol 1997; 7 : 823-836. |
| 3. | Davidoff LM, Martin J : Hereditary combined neurinomas and meningiomas. J Neurosurg 1955; 12 : 375-384. |
| 4. | Geddes JF, Sutcliffe JC, King TT : Mixed cranial nerve tumours in neurofibromatosis type-2. Clin Neuropathol 1995; 14 : 310-313. |
| 5. | Kim Dg, pack SH, Chi JG et al : Mixed tumour of schwannoma and meningioma components in a patient with NF-2. Acta Neurochir (wien) 1997; 139 : 1061-1065. |
| 6. | Kepes JJ, Kernohan JW : Meningiomas : problems of histological differential diagnosis. Cancer1959; 12 : 364-370. |
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