Craniopharyngioma: Causes, Pathology, Clinical manifestations, Diagnosis and Treatment
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Practice Essentials
Craniopharyngiomas are dysontogenic tumors with benign histology and malignant behavior. These lesions have a tendency to invade surrounding structures and to recur after a seemingly total resection. Craniopharyngiomas most frequently arise in the pituitary stalk and project into the hypothalamus. They extend horizontally along the path of least resistance in various directions.
Signs and symptoms
The time interval between the onset of symptoms and diagnosis usually ranges from 1 to 2 years.
The most common presenting symptoms are headache (55-86%), endocrine dysfunction (66-90%), and visual disturbances (37-68%). Headache is slowly progressive, dull, continuous, and positional; it becomes severe in most patients when endocrine symptoms become obvious.
Diagnosis
The diagnostic evaluation of craniopharyngioma includes high-definition brain imaging. Brain MRI with and without contrast is the gold standard. The use of computed tomography (CT) scan is optional and can show the common calcifications that can be seen in these tumors. However, it is important to note that a CT is not specific enough as a standalone diagnostic test.
Management
Essentially, two main management options are available for craniopharyngiomas: (1) attempt a gross total resection or (2) perform a planned subtotal resection followed by radiotherapy or some other adjuvant therapy.
Agents/modalities used in the treatment of craniopharyngioma include (1) radiation therapy applied as external fractionated radiation, stereotactic radiation, or brachytherapy (intracavitary irradiation) and (2) bleomycin for local intracystic chemotherapy.
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