Answer and Discussion of Quiz Set: U-001

1.    Which of the following statements is true?

A.  About 20% of all pituitary adenoma occurs in children.

B.  The majority of tumors involving the sella in pediatric patients are craniopharyngioma.

C.  Papillary craniopharyngioma is equally common in adult, adolescence, and children.

D.  Endocrine dysfunction is uncommon in craniopharyngioma occuring in children.

E.  Pituitary gigantism is not part of McCune Albright syndrome.

Answer and Discussion: The answer is (A). The majority (80-90%) of tumor in the sella of pediatric patients are craniopharyngiomas. Pituitary adenoma is rare in children and infants. Only about 3.5-8.5% are diagnosed in patients under 20 years of age. Papillary craniophryngioma is limited to adult and is not seen in pediatric cases. Endocrine dysfunction is seen in about 80% of pediatric patients with craniopharyngiomas at the time of diagnosis with growth hormone deficiency the most common. Although craniopharyngiomas are often large at the time of diagnosis, hyperprolactinemia secondary to compression or disruption of the pituitary stalk is present only in about 20% of the cases. Diabetes insipidus occurs in about only 9 to 17% of patients but it is frequently an initial presentation. McCune Albright syndrome is characterized by polyostotic fibrous dysplasia, café-au-lait pigmented lesions, endocrine abnormalities (precocious puberty, thyrotoxicosis, pituitary gigantism, and Cushings syndrome) and rarely by other tumors.

Further Reading:

Margaret F. Keil, Constantine A. Stratakis. Pituitary Tumors in Childhood: an update in their diagnosis, treatment and molecular genetics. Expert Rev Neurother. 2008 April; 8(4): 563–574.