A 15-year-old boy had hypercalcemia in association with malignant retroperi
toneal paraganglioma. He had suppressed circulating levels of intact parath
yroid hormone, whereas parathyroid hormone-related protein (PTHrP) immunore
activity was elevated in plasma. Both the serum 25-hydroxyvitamin D and 1,2
5-dihydroxyvitamin D levels were normal. Preoperatively the patient require
d control of hypercalcemia with intravenous pamidronate therapy. His circul
ating calcium and PTHrP concentrations became normal after a successful sur
gical resection of the primary retroperitoneal tumor. To our knowledge, thi
s is the first reported case of elevated PtHrP levels in a patient with par
aganglioma which resolved postoperatively.