Craniopharyngioma

Craniopharyngiomas originate from squamous rest cells in the remnant of Rathke's pouch located between the adenohypophysis and the neurohypophysis. These neoplasms may be entirely intrasellar (25%), solely extrasellar, or found in both areas. Commonly, these tumors present with nonendocrine symptoms, but anterior pituitary failure is frequent. GH deficiency and hypogonadotropic hypogonadism are the usual findings, but with diabetes insipidus (DI) occurring more commonly than in tumors of the anterior pituitary (34,35).

Surgery is the treatment of choice, transsphenoidally where possible; however, because most tumors have an extrasellar component, recurrence following surgery alone is common (36). The majority of patients require radiotherapy after surgery for craniopharyngioma. The consequences of treatment vary with the extent and location of the tumor and whether radical resection is undertaken. As complete an excision as possible, but with avoidance of damage to the carotid vessels, optic apparatus, and hypothalamus, offers the best long-term prognosis (37). DI and anterior pituitary failure are common after surgery, and these deficiencies may be permanent (38). Careful evaluation of ACTH and TSH status and water balance should be performed before discharge after any surgical procedure.

In those patients with recurrent tumors, further surgery may be necessary with marsupialization of cystic components, internal or external shunting, and occasionally cystic insertion of radionuclide. Clearly, long-term follow-up is central to the management of these patients, and most will require full pituitary hormone replacement, including desmopressin and growth hormone (see the following subheading).

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