Congenital craniopharyngioma and hypercalcemia induced by parathyroid hormone-related protein.
OBJECTIVE
To report a case of congenital craniopharyngioma and parathyroid hormone-related protein (PTHrP)-associated humoral hypercalcemia.


METHODS
Details of this unusual case are reviewed, from detection of fetal hydrocephalus and a brain tumor, through cesarean delivery at 36 weeks of gestation, to subsequent laboratory studies, management, and confirmation of the diagnosis.


RESULTS
Although PTHrP has been well documented as a cause of humoral hypercalcemia of malignancy (HHM) in adult patients with cancer, HHM is uncommon in children. In addition, HHM has rarely been ascribed to nonmalignant tumors. To the best of our knowledge, we report the first case of a neonate with congenital craniopharyngioma and refractory hypercalcemia (peak ionized calcium level of 1.92 mmol/L; normal, 1.05 to 1.3) attributed to an elevated PTHrP value of 8.6 pmol/L (normal, less than 4.7). Intact parathyroid hormone was appropriately undetectable (less than 10 pg/mL; normal, 15 to 65). Despite calcitonin treatment, the hypercalcemia persisted. Although pamidronate infusion stabilized the serum calcium level, the baby did not survive.


CONCLUSIONS
The diagnosis of craniopharyngioma was confirmed at autopsy, and immunohistochemical studies substantiated that the craniopharyngioma produced PTHrP.
Brown
Jessica L
JL
Department of Pediatrics, Division of Pediatric Endocrinology, University of Florida, Gainesville, Florida 32610, USA.
Burton
Douglas W
DW
Deftos
Leonard J
LJ
Smith
Amy A
AA
Pincus
David W
DW
Haller
Michael J
MJ
D000328:Adult; D002118:Calcium / Q000097:blood; D003397:Craniopharyngioma / Q000151:congenital* / Q000378:metabolism* / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006934:Hypercalcemia / Q000209:etiology* / Q000473:pathology; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D044162:Parathyroid Hormone-Related Protein / Q000378:metabolism*; D010911:Pituitary Neoplasms / Q000151:congenital* / Q000378:metabolism* / Q000473:pathology; D011247:Pregnancy; D011296:Prenatal Diagnosis
D044162:Parathyroid Hormone-Related Protein; D002118:Calcium