Brainstem gliomas in adults: prognostic factors and classification

Abstract

In contrast to childhood brainstem gliomas, adult brainstem gliomas are rare and poorly understood. The charts of 48 adults suffering from brainstem glioma were reviewed in order to determine prognostic factors, evaluate the effect of treatment and propose a classification of these tumours. Mean age at onset was 34 years (range 16-70 years). The main presenting symptoms were gait disturbance (61%), headache (44%), weakness of the limbs (42%) and diplopia (40%). Four patterns were identified on MRI, representing non-enhancing, diffusely infiltrative tumours (50%), contrast-enhancing localized masses (31%), isolated tectal tumours (8%) and other patterns (11%). Treatment consisted of partial resection (8%), radiotherapy (94%) and chemotherapy (56%). Overall median survival was 5.4 years. On univariate analysis, the following favourable prognostic factors were identified (P< 0.01): age of onset <40 years, duration of symptoms before diagnosis >3 months, Karnofski performance status >70, low-grade histology, absence of contrast enhancement and 'necrosis' on MRI. On multivariate analysis, the duration of symptoms, the appearance of 'necrosis' on MRI and the histological grade of the tumour remained significant and independent prognostic factors (P< 0.05). Eighty-five percent of the tumours could be classified into one of the following three groups on the basis of clinical, radiological and histological features. (i) Diffuse intrinsic low-grade gliomas (46%) usually occurred in young adults with a long clinical history before diagnosis and a diffusely enlarged brainstem on MRI that did not show contrast enhancement. These patients were improved by radiotherapy in 62% of cases and had a long survival time (median 7.3 years). Anaplastic transformation (appearance of contrast enhancement, 27%) and relentless growth without other changes (23%) were the main causes of death. (ii) Malignant gliomas (31%) occurred in elderly patients with a short clinical history. Contrast enhancement and necrosis were the rule on MRI. These tumours were highly resistant to treatment and the patients had a median survival time of 11.2 months. (iii) Focal tectal gliomas (8%) occurred in young patients and were often revealed by isolated hydrocephalus. The course was indolent and the projected median survival period exceeded 10 years. In conclusion, adult brainstem gliomas are different from the childhood forms and resemble supratentorial gliomas in adults. Low-grade tumours have a clinicoradiological pattern that is so characteristic that the need for a potentially harmful biopsy is debatable. The optimum timing of treatment for supratentorial low-grade tumours remains unclear. In high-grade gliomas, the prognosis remains extremely poor despite aggressive treatment with radiotherapy and chemotherapy.