Riassunto
La sindrome di Cushing può essere ACTH-dipendente o ACTH-indipendente ed ha una incidenza compresa fra 0,7 e 2,4/1.000.000 per anno. Il quadro clinico generalmente presenta manifestazioni “tipiche”, ma esse possono essere presenti con una frequenza e in combinazioni così variabili, tali da rendere la diagnosi difficoltosa. Esistono, tuttavia, quadri cosiddetti “atipici” talora correlati alle particolari età in cui si manifestano, ma, il più delle volte legati alle specifiche cause patogenetiche sottostanti. In questo articolo si intende richiamare l’attenzione sia del medico pratico, sia dello specialista, su alcuni quadri clinici particolari e su alcuni aspetti patogenetici peculiari (sia ACTH-dipendenti, sia ACTH-indipendenti), che sottendono la possibile presenza di una sindrome di Cushing.
Bibliografia
Desai N, Kapoor A, Singh BK, Liu J. Bilateral adrenal adenomas and persistent leukocytosis: a unique case of Cushing’s syndrome. Am J Med 119: e3, 2006.
Gursoy A, Dogruk Unal A, Ayturk S, Karakus S, Nur Izol A, Bascil Tutuncu N, Guvener Demirag N. Polycythemia as the first manifestation of Cushing’s disease. J Endocrinol Invest 29: 742, 2006.
Magiakou MA, Chrousos GP. Cushing’s syndrome in children and adolescents: current diagnostic and therapeutic strategies. J Endocrinol Invest 25: 181, 2002.
Storr HL, Isidori AM, Monson JP, Besser GM, Grossman AB, Savage MO. Prepubertal Cushing’s disease is more common in males, but there is no increase in severity at diagnosis. J Clin Endocrinol Metab 89: 3818, 2004.
Stratakis CA, Kirschner LS, Carney JA. Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation. J Clin Endocrinol Metab 86: 4041, 2001.
Lindsay JR, Nieman LK. The hypothalamic-pituitary-adrenal axis in pregnancy: challenges in disease detection and treatment. Endocr Rev 26: 775, 2005.
Walker AB, Leese GP, Vora JP. Diagnostic difficulties in periodic Cushing’s syndrome. Postgrad Med J 73: 426, 1997.
Lacroix A, Baldacchino V, Bordeau I, Hamet P, Tremblay J. Cushing’s syndrome variants secondary to aberrant hormone receptors. Trends Endocrinol Metab 15: 375, 2004.
Dall’Asta C, Ballarè E, Mantovani G, Ambrosi B, Spada A, Barbetta L, Colombo P, Travaglini P, Loli P, Beck-Peccoz P. Assessing the presence of abnormal regulation of cortisol secretion by membrane hormone receptors: in vivo and in vitro studies in patients with functioning and non-functioning adrenaladenoma. Horm Metab Res 36: 578, 2004.
Barzon L, Sonino N, Fallo F, Palù G, Boscaro M. Prevalence and natural history of adrenal incidentalomas. Eur J Endocrinol 149: 273, 2003.
Tauchmanovà L, Rossi R, Biondi B, Pulcrano M, Nuzzo V, Calmieri EA, Fazio S, Lombardi G. Patients with subclinical Cushing’s syndrome due to adrenal adenoma have increased cardiovascular risk. J Clin Endocrinol Metab 87: 4872, 2002.
Chiodini I, Guglielmi G, Battista C, Carnevale V, Torlontano M, Cammisa M, Trischitta V, Scillitani A. Spinal volumetric bone mineral density and vertebral fractures in female patients with adrenal incidentalomas: the effects of subclinical hypercortisolism and gonadal status. J Clin Endocrinol Metab 89: 2237, 2004.
Chiodini I, Torlontano M, Scillitani A, Arosio M, Bacci S, Di Lembo S, Epaminonda P, Augello G, Enrini R, Ambrosi B, Adda G, Trischitta V. Association of subclinical hypercortisolism with type 2 diabetes mellitus: a case-control study in hospitalized patients. Eur J Endocrinol 153: 837, 2005.
Terzolo M, Bovio S, Pia A, Conton PA, Reimondo G, Dall’Asta C, Bemporad D, Angeli A, Opocher G, Mannelli M, Ambrosi B, Mantero F. Midnight serum cortisol as a marker of increased cardiovascular risk in patients with a clinically inapparent adrenal adenoma. Eur J Endocrinol 153: 307, 2005.
Midorikawa S, Sanada H, Hashimoto S, Suzuki T, Watanabe T. The improvement of insulin resistance in patients with adrenal incidentaloma by surgical resection. Clin Endocrinol 54: 797, 2001.
Barzon L, Fallo F, Sonino N, Boscaro M. Development of overt Cushing’s syndrome in patients with adrenal incidentaloma. Eur J Endocrinol 146: 61, 2002.
Komiya I, Takasu N, Aizawa T, Yamada T, Koizumi Y, Hashizume K, Ishihara M, Hiramatsu K, Ichikawa K, Katakura M, Kobayashi M, Yamauchi K, Yanagisawa K, Naka M, Miyamoto T. Black (or brown) adrenal cortical adenoma: its characteristic features on computed tomography and endocrine data. J Clin Endocrinol Metab 61: 711, 1985.
Ambrosi B, Colombo P, Faglia G. Cushing’s syndrome due to a black adenoma of the adrenal gland: lack of tumour visualization by radiocholesterol scintigraphy. Eur J Nucl Med 21: 1367, 1994.
Ambrosi B, Colombo P, Bochicchio D, Bassetti M, Masini B, Faglia G. The silent corticotropinoma: is clinical diagnosis possibile? J Endocrinol Invest 15: 443, 1992.
Tan EU, Ho MSJ, Rajasoorya CR. Metamorphosis of a non-functioning pituitary adenoma to Cushing’s disease. Pituitary 3: 117, 2000.
Woo YS, Isidori AM, Wat WZ, Kaltsas GA, Afshar F, Sabin I, Jenkins PJ, Monson JP, Besser GM, Grossman AB. Clinical and biochemical characteristics of adrenocorticotropin-secreting macroadenomas. J Clin Endocrinol Metab 90: 4963, 2005.
Cazabat L, Ragazzon B, Groussin L, Bertherat J. PRKAR1A mutations in primary pigmented nodular adrenocortical disesae. Pituitary 9: 211, 2006.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Corbetta, S., Ermetici, F. & Ambrosi, B. Sindrome di Cushing: forme atipiche. L’Endocrinologo 9, 3–13 (2008). https://doi.org/10.1007/BF03344598
Published:
Issue Date:
DOI: https://doi.org/10.1007/BF03344598