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| Year : 2000 | Volume
: 48
| Issue : 4 | Page : 396-8 |
Primary fibrosarcoma of brain.
Vatsal DK, Sharma S, Renjen PN, Kaul S, Jha AN
Department of Neurosurgery, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi, 110044, India.
Correspondence Address:
Department of Neurosurgery, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi, 110044, India.
This is a case presentation of a young patient with an intracranial space-occupying lesion following multiple episodes of generalised tonic clonic seizures for the last 20 years. Such a long latency period between the onset of fits and the discovery of an intracranial lesion is highly unusual in malignant brain tumours. This lesion was excised completely and proved to be a primary lesion of the brain - fibrosarcoma. These rare tumours of mesenchymal origin in the central nervous system are very rare.
How to cite this article:
Vatsal D K, Sharma S, Renjen P N, Kaul S, Jha A N. Primary fibrosarcoma of brain. Neurol India 2000;48:396 |
Sarcomas involving the central nervous system can develop either in the parenchyma or in the adjacent structures[1],[2] or indeed may develop in distant organs and then metastasise to the brain. The incidence of brain sarcomas varies from 0.5 to 2.7% of intracranial neoplasms. The origin of such tumour cells is still controversial; while some authors have proposed a meningeal origin, others have suggested that the vascular endothelium is the site of the origin of these tumours.[3] lntracranial sarcomas that develop after radiation therapy are well known.[4],[5] This is a case report of a primary brain fibrosarcoma and the rarity of the disease as well as the unusual presentation has prompted us to present this case.
A 38 year old right handed hypertensive patient was admitted in the department of neurosurgery at the Indraprastha Apollo Hospital, New Delhi in November 1998 following the onset of progressive headaches and vomiting, which would increase in the morning hours over the last four weeks. This patient had a past history of multiple episodes of generalised tonic-clonic convulsions. However, a CT scan or MRI had not been carried out during this period. Examination revealed bilateral papilloedema and MRI revealed a large contrast enhancing left parieto occipital tumour with sizeable peritumoural oedema and mass effect [Figure. 1].
The patient was operated following steroid preparation and at surgery a large, soft, pinkish welldelineated tumour occupying the posterior medial parietal lobe extending in the occipital and posterior temporal lobe was found. There was no connection of this tumour to the dural margins and it was entirely intrinsic. The tumour was resected completely with the aid of intraoperative navigation. A frozen section at the time of the operation revealed a malignant tumour and it was possible to excise this completely. The patient recovered well with improvement in his headaches and vomiting. A post operative scan was carried out the next day with contrast enhancement, which confirmed a total excision of the tumour [Figure. 2]. The histological examination of the tumour demonstrated typical fibrosarcoma.
Pathological data : On gross examination the specimen consisted of several pinkish bits of tissue. Microscopically, it showed a highly cellular tumour comprising spindle shaped cells arranged in sheets, interlacing fascicles and exhibited a herring-bone pattern at places [Figure. 3]. Individual cells possessed pale eosinophilic cytoplasm and elongated nuclei with mild hyperchromasia. Brisk mitotic activity (7-8 mitotic Figures/10hpf) was observed. There was no evidence of whorling of tumour cells and psammoma bodies. Tumour cells were negative for GFAP and EMA, but showed strong positivity for vimentin [Figure. 4].
Primary intracranial sarcoma may originate from mesenchymal cells in the duramater, leptomeninges, adventitia of intraparenchymal blood vessels and stalk at choroid plexus. Most primary intracranial sarcomas are fibrosarcomas,[1] although examples of rhabdomyosarcoma and chondrosarcoma has also been reported.[2],[6],[7] Angiosarcoma is another variant which have been described in peripheral nerves and in the central nervous system.
Sarcomas are found in the brains of patients of all ages, most frequently in infants and children. Poorly differentiated tumours appear at an older age.[7] There are no characteristic clinical and radiological findings that might help to distinguish sarcoma from other neoplasms. The prognosis of patients with brain sarcoma is generally poor. The aetiology of brain sarcoma is unknown, however, several factors viz; genetic, viruses, trauma, radiation and certain chemicals may be involved in their aetiology.[8] Fibrosarcomas are common among the radiation induced neoplasms.[9] The latency period between the onset of radiation treatment and the discovery of sarcomas ranges from 5 to 12 years.
Fibrosarcomas can appear in three situations in the central nervous system, as a part of gliosarcoma, following irradiation of tumours like pituitary adenomas or rarely as a primary tumour. A primary fibrosarcoma in the central nervous system is either dura based or may arise within the brain, possibly from leptomeningeal infoldings. This case represents the latter. Histologically these tumours resemble a fibrosarcoma at any other body site. Considering the rarity of the primary fibrosarcomas of the central nervous system, it is mandatory to exclude common neoplasms like gliosarcoma and a meningioma. The former is excluded by GFAP negativity of the tumour cells and if a tumour is dural based, it may difficult to say whether the sarcoma has arisen de novo or it represents a sarcomatous change in a meningioma. A classical herring-bone pattern suggests a fibrosarcoma rather than a meningioma. EMA immuno reactivity is seen in meningiomas but not in a fibrosarcoma. In the present case the tumour was GFAP and EMA negative and exhibited a strong immuno reactivity for vimentin. All these features put together confirmed the diagnosis of primary fibrosarcoma of the brain, probably arising from the leptomeningeal infoldings.
It is difficult to explain the long latency period that was present in this case with the onset of epilepsy being 20 years prior to the discovery of the tumour. Possibilities include a malignant change occurring in a relatively benign tumour at a later stage but we feel that in this case the occurrence of a fibrosarcoma in an epileptic patient has to be kept in mind. The present case with a significant long history of 20 years is unusual and is different from others reported in the literature.
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| 5. | Gonzalez Vitale JC, Stavin RE, Mac Queen JD : Radiation induced intracranial malignant fibrous histiocytoma. Cancer 1976; 32 : 2960-2963. |
| 6. | Shuangshoti S, Phonprasert C : Primary intracranial rhabdomyosarcoma producing proptosis. J Neurosurg Psychiat 1976; 39 : 531-535. |
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| 8. | Riki Okeda : Origin of intracranial fibrosarcoma. Acta Neuropath 1980; 52 : 223-230. |
| 9. | Christensen E, Lara DE : lntracranial sarcomas. J Neuropathol Exp Neurol. 1953; 12 : 41-56. |
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