Adamantinomatous craniopharyngioma

Adamantinomatous craniopharyngioma (ACP) is a rare, benign brain tumor that is typically diagnosed in children and adolescents. Despite its benign nature, ACP can cause significant morbidity due to its location near critical structures in the brain.

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Adamantinomatous craniopharyngioma - high mag

Etiology

The exact cause of ACP is unknown. However, it is believed to arise from remnants of the Rathke's pouch, an embryonic precursor to the pituitary gland. Some studies have suggested a link between mutations in the CTNNB1 gene and the development of ACP, but further research is needed to confirm this association.

Clinical Presentation

Patients with ACP often present with symptoms related to increased intracranial pressure, such as headache, nausea, and vomiting. Other symptoms can include visual disturbances, hormonal imbalances, and growth retardation in children.

Diagnosis

The diagnosis of ACP is typically made based on imaging studies, such as magnetic resonance imaging (MRI) or computed tomography (CT) scans. These tests can reveal a characteristic appearance of the tumor, often described as a "soap bubble" or "honeycomb" pattern. Definitive diagnosis requires a biopsy of the tumor.

Treatment

Treatment for ACP typically involves surgery to remove as much of the tumor as possible. However, complete removal can be challenging due to the tumor's location and its tendency to adhere to surrounding structures. Radiation therapy may be used as an adjunct to surgery, particularly in cases where complete resection is not possible. Hormone replacement therapy may also be necessary for patients with pituitary dysfunction.

Prognosis

The prognosis for patients with ACP is generally good, with a high rate of long-term survival. However, these patients often require lifelong follow-up and treatment for endocrine disorders and other long-term effects of the tumor and its treatment.