Abstract
Acromegaly and hyperprolactinemia have been described in association with polyostotic fibrous dysplasia; the pathogenetic mechanisms involved in the development of the endocrinopathies is unknown. We report a 26-year-old man with polyostotic fibrous dysplasia and hypersecretion of GH and PRL. Plasma GH, PRL, and insulin-like growth factor-l (IGF-I) were elevated. Glucose-non-suppressible plasma GH concentrations, GH responsiveness to TRH and GHRH, and GH suppression after a testdose of somatostatin, octreotide, and bromocriptine were found. Plasma GHRH levels were within the normal range (< 25 ng/l). Computed tomography of the sella turcica and visual fields were normal. [IIIln-DTPA-D-Phe’]-octreotide scintigraphy were used to localize a possible tumor; no radioactivity was visualized at the site of the hypothalamus, the pituitary or elsewhere in the body but a considerable accumulation of radioactivity was found in the os frontalis. Therapy with octreotide by continuos sc infusion partially suppressed GH and IGF-I (and normalized PRL). The results suggest that hypersecretion of GH in our patient is not due to a GH-secreting pituitary tumor, eutopic or ectopic hypersecretion of GHRH or autonomous somatotroph function. The origin of the disease in this patient might be an abnormal hypothalamic regulation of somatotrophs and/or an alteration in the transmembrane signalling systems.
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References
Van Merkesteyn J.P.R. Fibrous Dysplasia. Doctoral Thesis. Amsterdam, April 1988.
Tolis G. Secretory tumors of the pituitary gland. In: Blach P.M., Zervas N.T., Redgway E., Martini J.B. (Eds.), Progress in Endocrine Research and Therapy. Raven Press, New York, 1984, p. 145.
Thorner M.O., Peryman R.L., Cronin M.J., Rogol A.D., Draznin M., Johanson A., Vale W., Horvath E., Kovacs K. Somatotroph hyperplasia. Successful treatment of acromegaly by removal of a pancreatic islet tumor secreting a growth hormone-releasing factor. J. Clin. Invest. 70: 965, 1982.
Lightner E.S., Penny R., Fraiser S.D. Growth hormone excess and sexual precocity in polyostotic fibrous dysplasia (McCune-Albright syndrome): Evidence for abnormal hypothalamic function. J. Pediatr. 87: 922, 1975.
Lipson A., Hsu T.H. The Albright syndrome associated with acromegaly: Report of a case and review of the literature. Johns Hopkins Med. J. 149: 10, 1981.
Geffner M.E., Nagel R.A., Dietrich R.B., Kaplan S.A. Treatment of acromegaly with a somatostatin analog in a patient with McCune-Albright syndrome. J. Pediatr. 111: 740, 1987.
Cuttler L, Jackson J.A., Saeed uz-Zafar M., Levitsky L.L., Mellinger R.C., Frohman L.A. Hypersecretion of growth hormone and prolactin in McCune Albright syndrome. J. Clin Endocrinol Metab. 68: 1148, 1989.
Scurry M.T., Bicknell J.M., Fajans S.S., Arbor A. Polyostotic fibrous dysplasia and acromegaly. Arch. Intern. Med. 114: 40, 1964.
Joishy S.K., Morrow LB. McCune-Abright syndrome associated with a functioning pituitary chromofobe adenoma. J. Pediatr. 89: 73, 1976.
Kovacs K., Horvath E., Thorner M.O., Rogol A.D. Mammosomatotroph hyperplasia associated with acromegaly and hyperprolactinemia in a patient with the McCune-Albright syndrome. Vichows Arch. (Pathol. Anat.) 403: 77, 1984.
Mauras N., Blizzard R.M. The McCune-Albright syndrome. Acta Endocrinol. (Copenh.) 113 (suppl.): 207, 1986.
Lee P.A., Van Dop C, Migeon J. McCune-Albright syndrome. Long-term follow-up. JAMA 256: 2980, 1986.
Hall R., Warrick Ch. Hypersecretion of hypothalamic releasing hormones: a possible explanation of the endocrine manifestations of polyostotic fibrous dysplasia (Albright’s Syndrome). Lancet 1: 1313, 1972.
Melmed S. Acromegaly. N. Engl. J. Med. 322: 966, 1990.
DiGeorge A.M. Albright syndrome: is it coming of age? J. Pediatr. 87: 1087, 1975.
Vallar L, Spada A., Giannattasio G. Altered Gs and adenylate cyclase activity in human GH-secreting pituitary adenomas. Nature 330: 566, 1987.
Landis C.A., Masters S.B., Spada A., Pace A.M., Bourne H.R., Vallar L. GTPase inhibiting mutations activate the chain of Gs and stimulate adenylyl cyclase in human pituitary tumours. Nature 340: 692, 1989.
Krenning E.P., Bakker W.H., Kooij P.P.M., Breeman W.A.P., Oei H.Y., de Jong M., Reubi J.C., Visser T.J., Bruns C, Kwekkeboom D.J., Reijs A.E.M., Van Hagen P.M., Koper J.W., Lamberts S.W.J. Somatostatin receptor scintigraphy with IIIln-DTPA-D-Phe’]-octreotide in man: metabolism, dositometry and comparison with (123|-Tyr3)-octreotide. J. Nucl. Med. 1992, in press.
Senior B., Robboy S.J. Case records of the Massachusetts General Hospital (case 4-1975). N. Engl. J. Med. 292: 199, 1975.
Casey P.J., Gilman A.G. G protein involvement in receptor effector coupling. J. Biol. Chem. 263: 2577, 1988.
Spada A., Vallar L, Giannattasio G. Presence of an adenylate cyclase dually regulated by somatostatin and human pancreatic growth hormone (GH)-releasing factor in GH-secreting cells. Endocrinology 115: 1203, 1984.
Weistein L.S., Shenker A., Gejman P.V., Merino M.J., Friedman E., Speigel A.M. Activating mutations of the stimulatory G protein in the McCune-Albright syndrome. N. Engl. J. Med. 325: 1688, 1991.
Reubi J.C., Landolt A.M. High density of somatostatin receptors in pituitary tumors from acromegalic patients. J. Clin. Endocrinol. Metab. 59: 1148, 1984.
Reubi J.C., Maurer R., Koper J.W., Lamberts S.W.J. Distribution and biochemical characterization of somatostatin receptors in tumors of the human central nervous system. Cancer Res. 47: 5758, 1987.
Reubi J.C., Maure R., Klijn J.G.M., Stefanko S.Z., Foekens J.A., Blaauw G., Blankenstein M.A., Lamberts S.W.J. High incidence of somatostatin receptors in human meningiomas: biochemical characterization. J. Clin. Endocrinol. Metab. 63: 433, 1986.
Reubi J.C., Maurer R, Von Werder K., Torhorst J., Klijn J.G.M., Lamberts S.W.J. Somatostatin receptors in human endocrine tumors. Cancer Res. 47: 551, 1987.
Reubi J.C., Hacki W.H., Lamberts S.W.J. Hormone-producing gastrointestinal tumors contain a high density of somatostatin receptors. J. Clin. Endocrinol. Metab. 65: 1127, 1987.
Runzi M.W., Jaspers C, Windeck R., Benker G., Mehdorn H.M., Reinhardt V., Reinwein D. Successful treatment of meningioma with octreotide. Lancet 13: 1074, 1989.
Lamberts S.W.J., Reubi J.C., Uiterlinden P., Zuiderwijk J., VD Werff P., Van Hal P. Studies on the mechanism of action of the inhibitory effect of the somatostatin analog SMS 201–995 on the growth of the prolactin/adrenocorticotropin-secretin pituitary tumor. Endocrinology 118: 2188, 1986.
Lamberts S.W.J., Krenning E.P. Reubi J.C. The role of somatostatin and its analogs in the diagnosis and treatment of tumors. Endocr. Rev. 12: 450, 1991.
Merola B., Colao A., Rossi R., Spaziante R., Manco A., Oliver C, Lombardi G. Bilateral and simultaneous inferior petrosal sinus sampling in the early diagnosis of an ACTH-producing pituitary microadenoma and its detection by magnetic resonance one year later. Horm. Res. 37: 64, 1992.
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Garcia, M.B., Koppeschaar, H.P.F., Lips, C.J.M. et al. Acromegaly and hyperprolactinemia in a patient with polyostotic fibrous dysplasia: Dynamic endocrine studies and treatment with the somatostatin analogue octreotide. J Endocrinol Invest 17, 59–65 (1994). https://doi.org/10.1007/BF03344964
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DOI: https://doi.org/10.1007/BF03344964