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 »  Introduction
 »  Case reports
 »  Discussion

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Year : 1999  |  Volume : 47  |  Issue : 2  |  Page : 136-8

Familial gliomas : a case report.


Division of Neurosurgery and Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, 221005, India.

Correspondence Address:
Division of Neurosurgery and Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, 221005, India.

  »  Abstract

Two non-twin brothers were found to have intracranial malignant neoplasms. The age of presentation was third and fourth decade but the onset was simultaneous, at the same time. Diagnosis in each of them was made by computed tomography and confirmed by histopathology. Elder among them had cellular ependymoma and the younger had oligodendroglioma. Both the brothers received radiotherapy post operatively and were surviving asymptomatically without any neurological deficit, leading active life as police constable, 12 months after surgical treatment.

How to cite this article:
Bhatt A R, Mohanty S, Sharma V, Shukla P K. Familial gliomas : a case report. Neurol India 1999;47:136


How to cite this URL:
Bhatt A R, Mohanty S, Sharma V, Shukla P K. Familial gliomas : a case report. Neurol India [serial online] 1999 [cited 2020 Aug 7];47:136. Available from: http://www.neurologyindia.com/text.asp?1999/47/2/136/1635




   »   Introduction Top


Genetic factors are unlikely to play a major part in the aetiology of gliomas other than those within the context of specific hereditary syndromes such as neurofibromatosis. Yet, the occasional occurrence of familial tumours demands a critical evaluation of possible genetic and environmental influences.[1]

Some authors[2],[3],[4],[5],[6],[7],[8],[9],[10] believe that a genetic factor is involved and others[11] deny involvement of any such factor. In the present cases, no other family member had brain tumour and on examination, there was no evidence of neurofibromatosis or tuberous sclerosis. There was history of consanguinity and parents were healthy. Review of literature showed a long list of reported brain tumours in twins and non-twin relatives in the western countries but surprisingly such cases from India are seldom reported.


   »   Case reports Top


Case 1 : A 25 year old man presented with headache and focal seizures followed by secondary generalisation of 3 years duration. He had spells of unconsciousness following convulsions for upto half an hour. He developed diplopia and also complained of slight weakness of left side of his body of four months duration. On examination, he was alert, oriented and haemodynamically stable. He had bilateral papilloedema, left 6th and supranuclear 7th nerve palsy along with left hemiparesis and left extensor planter.

A computed tomographic scan showed right parietal mixed attenuating hypodense mass with an enhancing nodule [Figure 1]. Two calcified spots and perilesional oedema were noted with a midline shift to left side. Right parietal craniotomy was performed. A large cystic lesion with a solid nodule measuring approx. 4cm x 3cm x 3cm was found. Cystic fluid was drained out and gross radical excision of tumour carried out. Post operative course was uneventful. He was put on anti-convulsants and steroids. His hemiparesis improved. Papilloedema and headache regressed. Histopathological examination of specimen proved an oligodendroglioma [Figure 2]. The follow up examination 12 months after surgery showed no obvious neural deficit.

Case 2 : A 32 year old man was admitted 5 days after his elder brother (case 1) was discharged from hospital. He had occasional headaches for the last 20 years. He was anxious about diplopia, which he had developed gradually over the last one year. However, there was no other positive history. On examination, he was in a good general condition. There was no sensory and motor deficit except bilateral papilloedema. CT Scan [Figure 3] revealed a hyperdense contrast enhancing, multilobulated, intraventricular mass in left lateral ventricle with obstructive hydrocephalus, more on right side. He was put on prophylactic anticonvulsants, steroid therapy and was subjected to excision biopsy of mass through a left frontal trephine craniotomy. Tumour was vascular and extended to opposite lateral ventricle. A decompressive procedure was carried out. Post-operative period passed uneventfully. Histopathological examination [Figure 4] was suggestive of cellular ependymoma. Patient's diplopia and headache improved post operatively. He was referred for radiation therapy.


   »   Discussion Top


There seems no doubt that hereditary factors play an important role in the genesis of certain tumours of the nervous system. In Von-Recklinghausen's disease the occurrence of gliomas, meningiomas and cranial nerve sheath tumours is well documented. There is a strong evidence of genetic factor in tuberous sclerosis, which may be associated with gliomas. Familial polyposis coli has been recently noted to be associated with cerebral tumours.[2] Apart from these genetically determined diseases there are a few reports suggesting that genetic factors play a part in the causation of gliomas.[2],[3],[4],[5],[6],[7],[8],[9],[10] Gliomas have been reported in the two sisters, a brother and sister and in two brothers.[9],[12] Two major statistical studies have been undertaken to try to establish whether the relations of patients with proven gliomas are more likely to suffer from the same disease than the general population.[1] Harvald and Hauge studied 1744 relatives of 169 patients with proven glioblastomas and compared them with a similar series of controls.[11] They concluded that inherited factors play no major aetiological role in glioblastomas. The authors carried out a similar study in a series of glioblastomas, medulloblastomas and meningiomas, and again concluded that hereditary factors scarcely play any part in the aetiology of these tumours. However, Van der Wiel, in a similar investigation,[13] studied 5262 relatives of 100 patients with proven gliomas. He found that 7 of these relatives had died of a verified glioma. Among the control group, there was no case of glioma. Vander Wiel concluded that the mortality from glioma was 4 times greater in the relatives of patients with proven gliomas than in the general population of the Netherlands.[13] The subject was well reviewed by Koch[14] and Kjellin et al.[3] It is difficult to assess the statistical significance of these relatively small number of cases.[1]

We report a family of 8 members with two brothers in 3rd and 4th decade presenting simultaneously with intracranial neoplasm, diagnosed by computed tomography as gliomas and verified histopathologically. Other family members were healthy. There was no history of any other malignancy in the family and there was no clinical evidence of phakomatosis.

The question of hereditary carcinomas has been studied in general cancerology and the term, `cancer family' (cancer family syndrome) has been coined. This is defined as tumour growth in non-related families widely dispersed geographically and presenting an increased occurrence of various types of cancer.[15] Although glial tumours are not uncommon and these families may be coincidental, yet several factors support genetic aetiology. Ikizler and Van Meyal investigated the blood relative of patients with glial tumours and found the incidence as 6.7%.[16] Detection of chromosomal abnormalities in cases harbouring gliomas, however, does not correlate with familial incidence.

 

 

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