Giant Adamantinomatous Craniopharyngioma in a child with paresis: A Case Report

A 21-month-old male baby was brought with a history of left-sided weakness. He is unable to grasp objects in his left hand and had difficulty walking. He has normal social, language, and cognitive development. Normal perinatal history without similar episodes in family members was noted.

MRI shows the heterogeneous solid cystic lobulated suprasellar lesion, which shows the marked mass effect with compression of optic chiasma and adjacent cerebral cortex (Figure 1). The cystic component shows the T1 and T2 high signal due to protein, cholesterol, or blood products (Figure 1A,1B,1D, and 1E). Post-contrast T1 weighted image shows heterogeneous enhancement of the solid component (Figure 1C).

 The mass is causing the compression of the foramen of Monroe with mild dilatation of the left lateral ventricle (Figure 1A and 1D). The cystic component is not suppressed in the FLAIR image (Figure 1F).

 The mass is causing anterior displacement of the M1 segment of the right middle cerebral artery as well as the A1 segment of the bilateral anterior cerebral artery. The distal basilar artery and bilateral P1 segment of the posterior cerebral artery are displaced posteriorly by the mass. No significant luminal narrowing is seen (Figure 1E).

Craniopharyngioma is a rare, low-grade malignancy arising from the craniopharyngeal duct.  Histologically it is of two types, adamantinomatous and papillary types, with varying age distribution. Adamantinomatous type shows bimodal peak age distribution (5-15 years and 45-60 years, while papillary type occurs predominately in adult age(50-60years) [1].

 These two variants also differ genetically based on the expression of beta-catenin. Beta-catenin is coded by the CTNN1 gene whose mutation activates the WNT pathway leading to the histological features of adamantinomatous craniopharyngioma(ACP). While papillary craniopharyngioma(PCP) is associated with BRAF gene mutation with activation of mitogen-activated protein kinase signaling pathway [2].

The diagnosis of childhood craniopharyngioma is generally made late with nonspecific features of headache and nausea. Other clinical manifestations include endocrine deficit and visual impairments based on the compression effect of mass to the hypothalamic-pituitary axis and optic chiasma. Less common presenting symptoms include motor disorders like Hemi- or monoparesis, seizures, psychiatric symptoms like emotional liability and hallucinations, and precocious puberty. Hemiparesis accounts for 7% in ACP, 1% in PCP, and 3% in total. Hemiparesis may be caused due to compression of the cerebral cortex by the mass [3]. Our case presented with left-sided hemiparesis which is a rare presentation.

 Imaging is essential in the assessment of craniopharyngioma concerning the location and relation with the adjacent vital structures, which helps in planning the best course of treatment. Pivotal information about the tumor, its consistency, and adjacent neurovascular structures are best evaluated by MRI. The cystic component shows iso to high signal intensity to brain parenchyma due to protein, cholesterol, or blood products with mixed signal intensity in T2 weighted images. Solid portions show T1 iso or low and T2 variable signal intensity with enhancement after contrast injection. It shows no restriction in Diffusion images. Magnetic resonance angiography (MRA) is helpful to see its relationship with critical vascular structures. Although additive role, CT illustrates the calcification in mass as well as skull base changes like enlarged Sella and its erosion [4]. The plain x-ray also helps to look for the changes in sella [5].

Surgery with or without external beam radiotherapy is the most widely used approach. A mass attached or infiltrated to vital structures increased the chance of residual tumor and obviates the need for external radiotherapy [6]. A multidisciplinary approach including surgeons, endocrinologists, neuro-anesthesiologist, and neurocritical care specialists plays an important role to provide the safest and all-inclusive treatment with minimal postoperative morbidity [4].