Brainstem glioblastoma in a patient with secondary progressive multiple sclerosis.

Abstract

Co-occurrence of glioblastomas (GBM) and multiple sclerosis (MS) in the same patient is very uncommon. Less than 50 cases in which MS and gliomas coexisted have been reported [1]. In several cases, development of glioma occurred years after the diagnosis of MS. Generally, the appearance of symptoms and signs of tumor in patients with MS is initially attributed to disease relapses in the relapsing–remitting, or to chronic worsening in the progressive forms; therefore, neuroradiological studies are not always performed with a subsequent delay in correct diagnosis. Even when neuroradiological examinations are performed it may sometimes be difficult to distinguish a tumoral from a demyelinating ‘‘tumor-like’’ lesion and histological confirmation is often required. A 60-year-old man with MS was admitted to our unit in April 2012 complaining marked worsening of right hemiparesis and dysarthria over the preceding 3 weeks. His disease had presented a relapsing–remitting course from 1994 (when diagnosis was formulated in agreement with clinical, cerebrospinal fluid and radiological data) to 2001, when secondary progression occurred (ataxia and spastic paraparesis, followed from 2006 by mild cognitive impairment), with superimposed relapses; the last relapse had occurred in 2006 with right optic nerve involvement. Azathioprine therapy had been started in 2004. Brain MRI showed typical features of long-standing demyelinating disease with thinning of the corpus callosum and widespread T2-weighted hyperintensities in the periventricular zones and in the brainstem; irregular enhancement on T1 post-gadolinium scan was detected, involving the lateral part of the left cerebral peduncle, pons and left superior and inferior cerebellar peduncles. Cerebrospinal fluid examination was repeated, revealing a protein concentration of 81 mg/dL and normal cell count, with negative culture; oligoclonal bands were detected at isoelectrofocusing. Treatment with high-dose intravenous methylprednisolone was performed. However, over the next 6 weeks his clinical conditions showed a rapidly progressing deterioration and the patient complained of tetraparesis, dysphagia and dysarthria. Repeat brain MRI (Fig. 1a–d) showed an increase in the extension of the brainstem lesion, with thick enhancement surrounding a partly cystic-like cavity, and involvement of the left middle cerebellar peduncle. The main diagnostic hypothesis was a high-grade glioma; tumor-like plaque was deemed unlikely due to lack of ‘‘open ring sign’’ in enhancement, and brain abscess unlikely due to the thickness and irregularity of contrast enhancement. MS plaques usually are localized in the periventricular white matter, optic nerves and chiasm, cerebellum, brainstem and spinal cord. On MRI examination, these plaques may show contrast enhancement and only in few cases they show mass effect, raising the possibility of an intracranial neoplasm. Size, localization, mass effect, perilesional oedema and progressive volume increase despite steroidal therapy can be red flags in the differential diagnosis of MS [2]. & Andrea Salmaggi a.salmaggi@ospedale.lecco.it