Presentation
5 months pregnant with seizures, abnormal behaviour, decreased level of consciousness, aphasia, inability to walk, and sphincter incontinence.
Patient Data
Plain MRI brain (IV contrast (gadolinium) was not given due to our limited experience regarding its safe use in pregnancy).
The scan reveals a focal ill-defined heterogeneous mass lesion measuring 3 x 4 x 7 cm in the anterior corpus callosum with extension on either side into the white matter of both frontal lobes (butterfly lesion). The mass appears hypointense on T1 and hyperintense on T2 and FLAIR images.
Multiple tiny haemorrhagic foci are visible in the mass on SWI. The mass exhibits mild diffusion restriction. Moderate vasogenic oedema is observed around the lesion in both frontal lobes, with extension into the anterior limbs of both internal capsules and into the external capsule-claustrum regions.
Mass effect is evident over the frontal horns of both lateral ventricles; however, no midline shift or hydrocephalus is detected. The remaining brain parenchyma appears normal. Based on these MRI features, potential diagnoses include high-grade glioma, primary CNS lymphoma, tumefactive demyelination, or an atypical infection like toxoplasmosis.
Selected images from contrast-enhanced CT brain show an enhancing intra-axial mass in the anterior corpus callosum, crossing the midline and involving both frontal lobes, likely representing a malignant neoplastic process, such as lymphoma or GBM.
Case Discussion
Further history revealed that she had this speech problem for a couple of years, which started worsening in the last 3-4 weeks, leading to complete aphasia at the time of presentation. There was also a history of hypothyroidism due to Hashimoto's (lymphocytic) thyroiditis.
A stereotactic brain biopsy was done, and a diagnosis of glioblastoma, IDH wild-type (negative IDH-1 and IDH-2 mutations), was made. MGMT promoter methylation was present.
Due to the highly malignant nature and location of the tumour, no surgery was offered. The patient was managed conservatively with steroids, tracheostomy, and mechanical ventilation to support foetal maturation; however, her general condition further deteriorated, and an emergency C-section was done (around 25 weeks of gestation) due to foetal distress & severe oligohydramnios. Three weeks later (within two months of the initial presentation), the patient died due to cardiopulmonary arrest.
Glioblastoma, a high-grade astrocytoma (WHO grade IV), is an aggressive primary tumour of the central nervous system. In approximately 3% of cases of GBM, the tumour extends inter-hemispherically across the midline through the corpus callosum, simulating the wings of a butterfly. Frontal butterfly gliomas are more common than posterior butterfly gliomas (like this case).
Butterfly GBM is a fatal diagnosis with a reported survival rate of a few weeks to months. Complete excision is usually not possible due to the infiltrative nature and bilateral extension of the tumour. Differential diagnosis includes primary CNS lymphoma, tumefactive demyelination, cerebral toxoplasmosis and rarely cerebral metastases.
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