Category: Pituitary Disorders/Neuroendocrinology
Objective : To present a case of cyclic Cushing's syndrome (CSC) in a 60-year-old patient
Methods : The clinical and paraclinical characteristics of the patient are presented
Results : Woman, 60 years old, HTA for 15 years and T2DM 3 years ago which partially controls with metformin. She reports that she starts illness with facial erythema spread to the bilateral malar region and presented full moon facies, a increase in the abdominal perimeter with abdominal red striae> 1cm, dorsal hump, ecchymosis in the lower extremities, and emaciation of the extremities. . Analytical: UFC: 290ug/24h (VN: 21-85) and ACTH: 55pg/ml (0-46). His hospitalization was decided for study. Loss of the circadian rhythm of cortisol was found; serum cortisol post Dexa2: 16.6ug/dl; Serum Cortisol post Dexa8: 1.48 ug/dl. Pituitary MRI: no adenoma. Given the results, it was proposed to perform IPSS, which was not accepted by the patient, initiating treatment with cabergoline and ketoconazole, which was suspended due to an adverse event. A year later she presented serum cortisol Dexa2: <1ug/dl, leaving discharge with a diagnosis of Cushing's syndrome to be considered cyclic. At follow-up, three years later, a new MRI revealed a 7x3mm microadenoma in the right hypophysis, undergoing IPSS after confirmation of hypercortisolism (baseline serum cortisol: 33.8ug/dl, serum cortisol Dexa2> 1.8ug/dl), IPSS with vasopressin demonstrated a ACTH central hypersecretion without being able to define laterality. The patient underwent transsphenoidal adenomectomy, finding a 6 mm tumor in the right hemihypophysis, which resulted: a solid-pattern pituitary adenoma with more than 90% ACTH-positive cells. Their controls at 3 and 6 months show normal values of serum and urinary cortisol, without signs of hypopituitarism and gradual decrease in clinical characteristics.
Discussion : A true SCC is accepted as such if it shows two peaks and a valley of cortisol production. It is usually caused by a Cushing's disease but it can also be caused by an ectopic tumor that secretes ACTH or an adrenal adenoma. This condition should be suspected in patients who present signs and symptoms of Cushing but with discrepant biochemical findings, patients who do not have an adequate response to dexamethasone suppression tests.
Conclusion : The diagnosis of SCC requires a high index of suspicion.
HOSPITAL NACIONAL EDGARDO REBAGLIATI - UNIVERSIDAD NACIONAL MAYOR DE SAN MARCOS. LIMA - PERU
Lima, Lima, Peru
PERUVIAN ENDOCRINOLOGIST, UNIVERSITY TEACHER, ACTIVE MEMBER OF AACE, AREAS OF INTEREST NEUROENDOCRINOLOGY, THYROID, ENDOCRINOLOGY OF REPRODUCTION AND BONE MINERAL METABOLISM