Patient With Craniopharyngioma

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A craniopharyngioma is a benign slow growing tumor most commonly occurring in the suprasellar region of the brain near the pituitary gland and optic nerves. The overall incidence of this tumor is about 1.2% and 4.6% with a peak incidence in children aged 5-14 years and in adults between 65 and 74 years of age (1, 2). The incidence does not vary by gender or race (2). These tumors account for 2% to 5% of all primary intracranial neoplasms (3).

Clinical Presentation. The most common presenting clinical features of craniopharyngiomas include headaches and visual field defects as well as nausea and vomiting (3, 4). Usually the visual disturbance manifests itself as a bitemporal hemianopsia as was the case with our patient. However, patients can also present with a homonymous hemianopia (5). The endocrine function may be suppressed and therefore manifest hypopituitarism, hypothyroidism, diabetes insipidus and or impotence (3, 4,5). Our presenting patient was positive for erectile dysfunction. On the other hand, there can also be an exaggeration of the endocrine system that may present as precocious puberty in children and obesity in adults (5).
Differential Diagnosis. The most common differential diagnosis considered when evaluating CT or MR images of craniopharyngiomas are (6):

Pituitary adenomas
Chiasmatic gliomas
Rathke’s pouch cyst
Epidermoid tumor

Pituitary adenomas tend to be bulkier and more likely to expand the sella, whereas craniopharyngiomas tend to expand upwardly leaving the bony sella unexpanded. The optic pathway gliomas differ from craniopharyngiomas in that they rarely involve the sella and are not usually calcified. Rathke’s cleft cysts can sometimes be challenging to differentiate from cranipharyngiomas, but these cysts are usually not solid or calcified. Epidermoid tumors rarely appear in the suprasellar area, but are still considered a possibility keeping in mind that enhancement of the peripheral rim is less common when compared to cranipharyngiomas (6).

Etiology. Craniopharyngiomas are not glial or neuronal lesions. They are epithelial neoplasms that can be both solid and cystic in nature (7). There are two theories as to how these tumors arise, the embryogenetic theory and metaplastic theory. The embryogenetic theory states that the Rathke’s pouch, which gives rise to the anterior pituitary gland during the fourth week of gestation, and the remnants of the craniopharygeal duct (the neck of the Rathke pouch) may be the site of origin for craniopharyngiomas. The metaplastic theory states residual squamous epithelial cells of the ectoderm of the embryo may undergo metaplasia and develop into a craniopharyngioma (5, 8).

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