Adult survivors of childhood craniopharyngiomas, the second most common type of childhood brain tumor, face many challenges, including multiple life-threatening metabolic abnormalities. Serious metabolic deficits can result from injury to the pituitary gland or hypothalamus.

Effective therapy must balance the threat of death from the tumor with concern about damage to normal tissue from aggressive therapy—especially in the developing brain of pediatric patients. Nurses are in a unique position to educate survivors about metabolic risks, encourage compliance with prescribed therapy, and contribute to quality of life.

PATIENT OVERVIEW

TS is a 23-year-old white male who was diagnosed in June 1996, at 11 years of age, with a craniopharyngioma. He underwent a craniotomy/subtotal tumor resection, Ommaya reservoir insertion, and 5,580 cGy external beam radiation therapy to the suprasellar area of the brain. All therapy was completed in December 1996. Subsequently, TS was monitored at frequent intervals for possible tumor recurrence and complications resulting from his tumor and treatment.

Shortly after the diagnosis, TS developed growth hormone and thyroid hormone deficiencies, adrenocorticosteroid insufficiency, and hypogonadism. These multiple endocrine abnormalities were treated with hormone replacement therapy, including stress dosing of corticosteroids.
Within 4 years, TS developed obesity (current body mass index, 40.2), along with hyperinsulinism and dyslipidemia (cholesterol, 248 mg/dL; triglycerides, 304 mg/dL; LDL, 143 mg/dL; and HDL, 44 mg/dL). Although TS experienced some visual field deficits, his visual acuity, hearing, and academic performance were normal.

He graduated from high school, attended college for 2 years, and is employed as a dispatcher
in a trucking firm. He is intermittently compliant with needed hormone replacement therapy (levothyroxine, steroids, and testosterone) and continues to struggle with morbid obesity.

DISCUSSION

This case illustrates important endocrine sequelae that can be seen in patients with brain tumors, the most common malignancy in childhood. Craniopharyngiomas, histologically benign tumors, account for about 10% of all pediatric brain tumors.[1] They occur in the center of the brain near vital structures such as the optic nerve, hypothalamus, or pituitary and tend to adhere to surrounding delicate brain tissue. Treatment includes surgical resection, radiation therapy, or both[2,3] and results in an overall survival of 80% to 90%.[4,5]

Damage to the pituitary and hypothalamus can occur from tumor extension or compression, during surgical resection or secondary to radiation therapy. The consequences of such damage are devastating. Figure 1 illustrates the many pituitary hormones and their diverse functions on target organs. These hormones promote body growth, protein synthesis, and fertility. They regulate glucose metabolism, cortisol/thyroid hormone production, and urine output.

Figure 1: Pituitary hormones and their target organs—ACTH, adrenocorticotropic hormone; GH, growth hormone; MSH, melanocyte-stimulating hormone; TSH, thyroid-stimulating hormone; FSH, follicle-stimulating hormone; LH, luteinizing hormone; ADH, antidiuretic hormone.