A 15-year-old boy had hypercalcemia in association with malignant retroperitoneal paraganglioma. He had suppressed circulating levels of intact parathyroid hormone, whereas parathyroid hormone-related protein (PTHrP) immunoreactivity was elevated in plasma. Both the serum 25-hydroxyvitamin D and 1,25-dihydroxyvitamin D levels were normal. Preoperatively the patient required control of hypercalcemia with intravenous pamidronate therapy. His circulating calcium and PTHrP concentrations became normal after a successful surgical resection of the primary retroperitoneal tumor. To our knowledge, this is the first reported case of elevated PtHrP levels in a patient with paraganglioma which resolved postoperatively.

Keywords:
Humoral hypercalcemia, Paraganglioma, Parathyroid hormone-related protein, Malignancy
1.
Ali IM, Graham C, Sanalla B, Bell D: Carotid body tumor associated with hyperparathyroidism. Ann Vasc Surg 1994;8:595–598.
2.
White MC, Hickson BR: Multiple paragangliomata secreting catecholamines and calcitonin with intermittent hypercalcaemia. J R Soc Med 1979;72:532–538.
3.
Passwell J, Boichis H, Lotan D, David R, Theodor R, Cohen BE, Many M: The metabolic effects of excess noradrenaline secretion from a pheochromocytoma. Am J Dis Child 1977;131:1011–1014.
4.
Stewart AF, Hoecker JL, Mallette LE, Segre GV, Amatruda TT Jr, Vignery A: Hypercalcemia in pheochromocytoma. Evidence for a novel mechanism. Ann Intern Med 1985;102:776–779.
5.
Burtis WJ, Wu T, Bunch C, Wysolmerski JJ, Insogna KL, Weir EC, Broadus AE, Stewart AF: Identification of a novel 17,000-dalton parathyroid hormone-like adenylate cyclase-stimulating protein from a tumor associated with humoral hypercalcemia of malignancy. J Biol Chem 1987;262:7151–7156.
6.
Moseley JM, Kubota M, Diefenbach-Jagger H, Wettenhall RE, Kemp BE, Suva LJ, Rodda CP, Ebeling PR, Hudson PJ, Zajac JD, et al: Parathyroid hormone-related protein purified from a human lung cancer cell line. Proc Natl Acad Sci USA 1987;84:5048–5052.
7.
Strewler GJ, Stern PH, Jacobs JW, Eveloff J, Klein RF, Leung SC, Rosenblatt M, Nissenson RA: Parathyroid hormonelike protein from human renal carcinoma cells. Structural and functional homology with parathyroid hormone. J Clin Invest 1987;80:1803–1807.
8.
Kimura S, Nishimura Y, Yamaguchi K, Nagasaki K, Shimada K, Uchida H: A case of pheochromocytoma producing parathyroid-related protein and presenting with hypercalcemia. J Clin Endocrinol Metab 1990;70:1559–1563.
9.
Bridgewater JA, Ratcliffe WA, Bundred NJ, Owens CWI: Malignant phaeochromocytoma and hypercalcaemia. Postgrad Med J 1993;69:77–79.
10.
Suva LJ, Winslow GA, Wettenhall REH, Hammonds RG, Moseley JM, Diefenbach-Jagger H, Rodda CP, Kemp BE, Rodriguez H, Chen EY, et al: A parathyroid hormone-related protein implicated in malignant hypercalcemia: cloning and expression. Science 1987;237:893–896.
11.
Juppner H, Abou-Samra AB, Freeman M, Kong XF, Schipani E, Richards J, Kolakowski LF Jr, Hock J, Potts JT Jr, Kronenberg MM, et al: A G protein-linked receptor for parathyroid hormone and parathyroid hormone-related peptide. Science 1991;254:1024–1026.
12.
Budayr AA, Nissenson RA, Klein RF, Pun KK, Clark OH, Diep D, Arnaud CD, Strewler GJ: Increased levels of a parathyroid hormone-like protein in malignancy-associated hypercalcemia. Ann Intern Med 1989;111:807–812.
13.
Burtis WJ, Brady TG, Orloff JJ, Ersbak JB, Warrell RP Jr, Olson BR, Wu TL, Mitnick BE, Broadus AE, Stewart AF: Immunochemical characterization of circulating parathyroid hormone-related protein in patients with humoral hypercalcemia of cancer. N Engl J Med 1990;322:1106–1112.
14.
Grill V, Ho P, Body JJ, Johanson N, Lee SC, Kukreja SC, Moseley JM, Martin TJ: Parathyroid hormone-related protein: Elevated levels in both humoral hypercalcemia of malignancy and hypercalcemia complicating metastatic breast cancer. J Clin Endocrinol Metab 1991;73:1309–1315.
15.
Mune T, Katakami H, Kato Y, Yasuda K, Matsukara S, Miura K: Production and secretion of parathyroid hormone-related protein in pheochromocytoma: Participation of an α-adrenergic mechanism. J Clin Endocrinol Metab 1993;76:757–762.
16.
Stewart AF, Horst R, Deftos LJ, Cadman EC, Lang R, Broadus AE: Biochemical evaluation of patients with cancer-associated hypercalcemia. Evidence for humoral and nonhumoral groups. N Engl J Med 1980;303:1377–1383.
17.
Schilling T, Pecherstorfer M, Blind E, Leidig G, Ziegler R, Raue F: Parathyroid hormone-related protein (PTHrP) does not regulate 1,25-dihydroxyvitamin D serum levels in hypercalcemia of malignancy. J Clin Endocrinol Metab 1993;76:801–803.
18.
Schweitzer DH, Hamdy NAT, Frolich M, Zwinderman AH, Papapoulos SE: Malignancy-associated hypercalcemia: resolution of controversies over vitamin D metabolism by a pathophysiological approach to the syndrome. Clin Endocrinol 1994;41:251–256.
19.
Wimalawansa SJ: Hypercalcaemia in malignancy: aetiology, pathophysiology, and its clinical management. Rev Endocr Rel Cancer 1993;45:5–24.
20.
Gurney H, Grill V, Martin TJ: Parathyroid hormone-related protein and response to pamidronate in tumor-induced hypercalcaemia. Lancet 1993;341:1611–1613.
21.
Wimalawansa SJ: Significance of plasma PTH-rp in patients with hypercalcemia of malignancy treated with biphosphonate. Cancer 1994;73:2223–2230.
22.
Walls J, Ratcliffe WA, Howell A, Bundred NJ: Response to intravenous biphosphonate therapy in hypercalcemic patients with and without bone metastases: The role of parathyroid hormone-related protein. Br J Cancer 1994;70:169–172.
23.
Pecherstorfer M, Schilling T, Blind E, Zimmer-Roth I, Baumgartner G, Ziegler R, Raue F: Parathyroid hormone-related protein and life expectancy in hypercalcemic cancer patients. J Clin Endocrinol Metab 1994;78:1268–1270.
1998
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