Familial oligodendroglioma

Case report

Full access

✓ A case of familial oligodendroglioma, occurring in a mother and her daughter, is presented.

Abstract

✓ A case of familial oligodendroglioma, occurring in a mother and her daughter, is presented.

In contrast to astrocytomas and glioblastomas, which have occasionally been reported as occurring in members of one family, the familial appearance of oligodendrogliomas has only been described once.8 We present two patients, mother and daughter, both of whom were operated on for an oligodendroglioma.

Case Reports
Case 1

This 37-year-old mother was admitted for evaluation of grand mal epilepsy. The neurological examination was normal. A right posterofrontal focus was noted in the electroencephalogram, and right carotid angiography demonstrated an avascular space-occupying lesion. A right frontal lobectomy was performed, and the tumor was excised. Histopathological examination revealed an oligodendroglioma with a homogeneous parenchyma and microcysts (Fig. 1). There were no mitoses or neovascularization. Nine years after the operation the tumor recurred, and the patient was submitted to a second operation. The tumor again proved to be an oligodendroglioma with essentially the same features as shown by the previous specimen.

Fig. 1.
Fig. 1.

Case 1. Photomicrograph of the tumor showing a typical ohgodendroglioma with a honeycomb structure. Hematoxylin & saffron, × 262.

Case 2

The 22-year-old daughter of the patient in Case 1 came under medical attention because of a grand mal seizure. A right parietal hypodense lesion was visualized on computerized tomography (CT) scanning. The patient was treated expectantly because of a lack of mass effect, a normal neurological examination, and control of her seizures with adequate antiepileptic therapy. Three years later, however, a slight left hemiparesis developed during pregnancy, and the epilepsy became resistant to therapy. Angiography and CT scanning confirmed progression of the tumor. A large biopsy of the tumor was performed. An oligodendroglioma with a metachromatic parenchyma, numerous microcysts, and a homogeneous isomorphous cellularity was diagnosed on microscopic study (Fig. 2). The tumor showed no mitoses or pathological hypervascularization.

Fig. 2.
Fig. 2.

Case 2. Photomicrograph of the biopsy specimen showing an oligodendroglioma with the same features as in Case 1, but with a more apparent microcystic component. Hematoxylin & saffron, × 105.

Discussion

The familial occurrence of gliomas is a well known phenomenon in cases of von Recklinghausen's disease, tuberous sclerosis,10 and Turcot's syndrome,13,14 but has also been observed with no association to diseases. These were almost always astrocytomas or glioblastomas.1–4,6,9,11,12,16,17 We have found only one report of familial oligodendrogliomas: in 1962, Parkinson and Hall8 described two brothers, each of whom had a right frontal oligodendroglioma.

Studies have been carried out to determine if some increased predisposition to develop a glioma exists in close relatives of patients with astrocytoma and glioblastoma. Van der Wiel15 investigated the families of 100 glioma patients and found that the mortality resulting from glioma was four times higher in comparison with a control group. Metzel and Mohadjer7 found a prevalence of gliomas in the families of glioma patients 10 times higher than in the general population. However, in contrast to these results, Hauge and Harvold5 were unable to collect any evidence for a genetic determination of the ocurrence of gliomas in their study. They found eight cases of intracranial tumors among 2020 relatives of 174 probands with astrocytomas and five cases of intracranial tumors among 1813 relatives of 179 probands with glioblastomas, as opposed to 10 cases of intracranial tumors among 2288 relatives of 249 control individuals. No oligodendrogliomas were present in the 535 probands with brain tumors, one oligodendroglioma was found in the eight secondary cases within the group of 174 astrocytoma probands, and two were diagnosed in the 10 secondary cases within the control material.

These figures emphasize the rarity of oligodendrogliomas compared with astrocytomas and glioblastomas. In view of this rarity, the demonstration of a familial occurrence in two brothers by Parkinson and Hall,8 as well as in a mother and her daughter by us, is of some significance. Of course, we cannot speculate on a genetic mechanism, but we may conclude that in certain families there may be an increased predisposition in close relatives to develop oligodendrogliomas. Further case reports will be of great value to show if this is true.

Acknowledgment

The authors thank Mr. Gery Draps of The Upjohn Co., Belgium, for generous help with the literature search.

References

Article Information

Address reprint requests to: Christian De La Porte, M.D., Dienst voor Neurochirurgie, Akademisch Ziekenhuis der Universiteit, Wilrijkstraat 10, B-2520 Antwerpen-Edegem, Belgium.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Case 1. Photomicrograph of the tumor showing a typical ohgodendroglioma with a honeycomb structure. Hematoxylin & saffron, × 262.

  • View in gallery

    Case 2. Photomicrograph of the biopsy specimen showing an oligodendroglioma with the same features as in Case 1, but with a more apparent microcystic component. Hematoxylin & saffron, × 105.

References

1.

Chadduck WMNetsky MG: Familial gliomas: report of four families, with chromosome studies. Neurosurgery 10:4454491982Neurosurgery 10:

2.

Challa VRGoodman HODavis CH Jr: Familial brain tumors: studies of two families and review of recent literature. Neurosurgery 12:18231983Neurosurgery 12:

3.

de Tribolet NDeruaz JPZander E: Familial gliomas. Neurochirurgia (Stuttg) 22:2252281979Neurochirurgia (Stuttg) 22:

4.

Fairburn BUrich H: Malignant gliomas occurring in identical twins. J Neurol Neurosurg Psychiatry 34:7187221971J Neurol Neurosurg Psychiatry 34:

5.

Hauge MHarvald B: Genetics in intracranial tumours Acta Genet 7:5735911957Acta Genet 7:

6.

Isamat FMiranda AMBartumeus Fet al: Genetic implications of familial brain tumors. J Neurosure 41:5735751974J Neurosure 41:

7.

Metzel EMohadjer M: Familial incidence of brain tumorsFusek IKunc J (eds): Present Limits of Neurosurgery. Prague/Avicenum: Czechoslovak Medical Press19721718Present Limits of Neurosurgery.

8.

Parkinson DHall CW: Oligodendrogliomas. Simultaneous appearance in frontal lobes of siblings. J Neurosurg 19:4244261962J Neurosurg 19:

9.

Pendergrass TWFraumeni JF JrFagan EL: Brain tumors in sibs, one with the Turner syndrome. J Pediatr 85:8751974 (Letter)J Pediatr 85:

10.

Russell DSRubinstein LJ: Pathology of Tumours of the Nervous Systemed 4. London: Edward Arnold 1977 pp 48

11.

Schianchi PKraus-Ruppert R: Familial brain tumor: rhombencephalon-astrocytoma grade I in father and son. Acta Neuropathol 52:1531551980Acta Neuropathol 52:

12.

Symonds C: Disease of mind and disorder of brain. Br Med J 2:151960Symonds C: Disease of mind and disorder of brain. Br Med J 2:

13.

Todd DWChristoferson LALeech RWet al: A family affected with intestinal polyposis and gliomas. Ann Neurol 10:3903921981Ann Neurol 10:

14.

Turcot JDespres JPSt Pierre F: Malignant tumors of the central nervous system associated with familial polyposis of the colon: report of two cases. Dis Colon Rectum 2:4654681959Dis Colon Rectum 2:

15.

Van der Wiel HJ: Inheritance of Glioma. Amsterdam: Elsevier1960251Van der Wiel HJ: Inheritance of Glioma.

16.

Von Motz IPBots GTAMEndtz LJ: Astrocytoma in three sisters. Neurology 27:103810411977Neurology 27:

17.

Wald SLLiwnicz BHTruman TAet al: Familial primary nervous system neoplasms in three generations. Neurosurgery 11:12151982Neurosurgery 11:

TrendMD

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 1 1 1
Full Text Views 68 68 35
PDF Downloads 51 51 35
EPUB Downloads 0 0 0

PubMed

Google Scholar