Surgical management of diffuse low-grade gliomas associated with other intracranial diseases

Abstract

The association of diffuse, low-grade glioma (DLGG) with other intracranial pathologies is a rare condition, raising the question of what constitutes its most effective therapeutic management. It is not known whether this is a simple coincidence or whether there is a higher significant risk involved with the co-existence of DLGG and another disease. We report the first consecutive series of nine patients who underwent surgical resection for such a glioma. We reviewed DLGGs removed between 1998 and 2013 that were associated with another intracranial pathology. For all cases, we collected and analyzed information regarding clinico-radiological features, surgical procedures, and clinical outcomes. Nine consecutive patients (four men, five women, mean age: 38.8 years) presented with a conjunction of DLGG and another disease: two cases of vestibular schwannoma, two pituitary adenomas, two meningiomas, one lymphoma, one arteriovenous malformation, and one case of multiple sclerosis. The DLGG was diagnosed because of seizures in four patients and incidentally in the other five patients. The average delay between the diagnosis of the glioma and its resection was 40.8 months (range 1-84 months). The mean follow-up after surgery was 43 months (6-120 months). Gross-total or subtotal resection was achieved in all cases. There were no cases of mortality or permanent morbidity associated with surgery. The Karnofsky Performance Scale score was 90 or 100 in all cases. The associated pathology was treated surgically in three cases, medically in four cases, and tracked under observation in two cases. These intracranial diseases, especially meningiomas and pituitary adenomas, might have a significant higher risk to be associated with DLGGs in comparison with their incidence and prevalence in the general population. Active management of this rare, dual pathology allows patients to enjoy a normal and prolonged quality of life. We therefore suggest considering early and maximal surgical resection as the first therapeutic option for DLGGs combined with another intracranial disease, as is done in the case of isolated DLGGs.