Presentation
5 months pregnant with seizures, abnormal behavior, decreased level of consciousness, aphasia, inability to walk and sphinter 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 shows a focal ill-defined heterogeneous mass lesion measuring 3 x 4 x 7 cm in the anterior corpus callosum with extension, on either side of the corpus callosum into the white matter of both frontal lobes (Butterfly lesion). The mass is hypointense on T1 & hyperintense on T2 & FLAIR images. Multiple tiny hemorrhagic foci are seen in the mass on SWI. The mass shows mild diffusion restriction. Moderate vasogenic edema is noted 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 noted over the frontal horns of both lateral ventricles; however, no midline shift or hydrocephalus is seen. Remaining brain parenchyma is normal. Based on these MRI features, a few possibilities are high-grade glioma, primary CNS lymphoma, tumefactive demyelination, or an atypical infection like toxoplasmosis.
Selected images from contrast-enhanced CT brain showing 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 tumor, no surgery was offered. The patient was managed conservatively with steroids, tracheostomy, and mechanical ventilation to support fetal maturation; however, her general condition further deteriorated and an emergency C-section was done (around 25 weeks of gestation) due to fetal distress & severe oligohydramnios. 3 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 tumor of the central nervous system. In approximately 3% cases of GBM, the tumor 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 tumor. Differential diagnosis includes primary CNS lymphoma, tumefactive demyelination, cerebral toxoplasmosis & rarely cerebral metastases.
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