Severe Ectopic Cushing’s Syndrome Due to ACTH-Secreting Pheochromocytoma
- 1 Second Division of Internal Medicine and Hypertension Unit, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy
- 2 Diabetologic Unit, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy
- 3 Second Division of Internal Medicine and Hypertension Unit, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy
- 4 Diabetologic Unit, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy
- 5 Second Division of Internal Medicine and Hypertension Unit, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy
- 6 Diabetologic Unit, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy
- 7 Radiology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy
- 8 Pathology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy
Abstract
We report a new case of ectopic Cushing’s syndrome caused by an ACTH-producing pheochromocytoma. A 55-year- old woman presented with a history of severe proximal muscle weakness, polyuria, progressive virilization, anxiety, dyspnea on exercise, difficult to treat hypertension, and type 2 diabetes mellitus since 4 months. The laboratory data demonstrated ACTH-dependent hypercortisolism. The abdominal computed tomography scan showed a 30 mm well- defined mass in the left adrenal gland suggestive for pheochromocytoma. The adrenal veins were sampled, with intra procedural cortisol measurement, to dosing selective ACTH and cathecolamines. The results established clearly the left adrenal gland as the source of ACTH overproduction. A left sided adrenalectomy was performed with subsequent reso lution of Cushing’s syndrome. The patient was discharged in good clinical condition.
- J. Lindholm, S. Juul, J. O. Jorgensen, J. Astrup, P. Bjerre, U. Feldt-Rasmussen, C. Hagen, J. Jorgensen, M. Kosteljanetz, L. Kristensen, et al., “Incidence and Late Prognosis of Cushing’s Syndrome: A Population-Based Study,” The Journal of Clinical Endocrinology & Metabolism, Vol. 86, No. 1, 2001, pp. 117-123. doi:10.1210/jc.86.1.117
- J. Newell-Price, X. Bertagna, A. B. Grossman and L. K. Nieman, “Cushing’s Syndrome,” Lancet, Vol. 367, No. 9522, 2006, pp. 1605-1617. doi:10.1016/S0140-6736(06)68699-6
- D. J. Torpy, N. Mullen, I. Ilias, et al., “Association of Hypertension and Hypokalemia with Cushing’s Syndrome Caused by Ectopic ACTH Secretion: A Series of 58 Cases,” Annals of the New York Academy of Sciences, Vol. 970, No. 1, 2002, pp. 134-144. doi:10.1111/j.1749-6632.2002.tb04419.x
- J. P. Aniszewski, W. F. Young Jr., G. B. Thompson, C. S. Grant and J. A. van Heerden, “Cushing Syndrome Due to Ectopic Adrenocorticotropic Hormone Secretion,” World Journal of Surgery, Vol. 25, No. 7, 2001, pp. 934-940. doi:10.1007/s00268-001-0032-5
- M. F. Nijhoff, O. M. Derkers, L. J. Vleming, J. W. A. Smit, J. A. Romijn and A. M. Pereira, “ACTH-Producing Pheocromocytoma: Clinical Considerations and Concise Review of the Literature,” European Journal of Internal Medicine, Vol. 20, No. 7, 2009, pp. 682-685. doi:10.1016/j.ejim.2009.08.002
- M. Mannelli, J. W. M. Lenders, K. Pacak, G. Parenti and G. Eisenhofer, “Subclinical Phaeochromocytoma,” Best Practice and Researche Clinical Endocrinology and Metabolism, Vol. 26, No. 4, 2012, pp. 507-515.
- P. M. Stewart, “Mineralocorticoid Hypertension,” Lancet, Vol. 353, No. 9161, 1999, pp. 1341-1347. doi:10.1016/S0140-6736(98)06102-9