UNUSUAL EVOLUTION OF CUSHING`S DISEASE: AN UNSUSPECTED SPONTANEOUS REMISSION

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SUN 163-194-Pituitary Disorders & Case Reports
Basic/Clinical
Sunday, June 16, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SUN-181
Karina Miragaya*, Marcia Gabriela Jimenez, Ana Paula Lisdero, Miguel Walter Vasquez Cayoja, Cecilia Goldaracena, Maria Cecilia Paskiewicz and Javier Mauricio Farias
Sanatorio GŁemes, Buenos Aires, Argentina
 INTRODUCTION: Only sporadic cases of spontaneous remission of Cushing´s disease have been reported in the literature(1)(2). Pituitary apoplexy due to hemorrhage or infarction of a corticotroph adenoma has been described as the possible causes. This phenomenon mainly occurs in macroadenomas, and the clinical presentation can varies from silent infarction of adenoma without hormonal deficiencies, to a life-threatening clinical syndrome with neurological signs and symptoms of hypoadrenalism(3).The objective is to describe the case of a patient with Cushing´s disease who survived to pituitary apoplexy that leads to an untreated adrenal insufficiency, with subsequent spontaneous recover of the pituitary adrenal axis.

 CASE REPORT: A 40-year-old woman was referred to our institution due to oligomenorrhea, in April, 2010. On physical examination, she presented with typical features of Cushing’s syndrome, including moon fascies, truncal obesity with supraclavicular fat pads, hirsutism and mild hypertension. Blood tests showed urinary free cortisol level (UFC) of 456µg/24 hs [normal range (NR): 20-90)], late night salivary cortisol: 0.76 µg/dl (NR: 0.08-0.36) and cortisol post 1-mg-dexametasone test of: 2.6 µg/dl. Due to an ACTH level of 70 pg/ml (NR: 5-60), a pituitary MRI  was requested but medical follow up was discontinued by the patient. In March, 2012, the patient returned to medical care. She didn’t have any of the typical features of Cushing’s syndrome that were observed two years ago. She referred that between March and August last year suffered from severe headache, fatigue, dizziness, vomit and diarrhea. During this period she lost 30 Kg. Biochemical tests revealed serum cortisol level of 8 µg/dl, UFC of 15 µg/24 hs  and late night salivary cortisol <0.08 µg/dl. She had not received any corticosteroid replacement during this period. The MRI of the pituitary gland performed in 2010 showed a 7-mm lesion on middle of the gland with low signal intensity on a coronal T1 weighted image after gadolinium injection. A spontaneous remission of Cushing´s disease after presumed pituitary apoplexy with hypoadrenalism was suspected.  A new MRI showed disappearance of the microadenoma, with partial arachnoidocele and lateralization of the pituitary stalk. The patient was reassessed at regular intervals during the last year. Endocrine tests showed recovery of pituitary-adrenal axis and preservation of other pituitary functions.

CONCLUSION: Spontaneous remission of Cushing`s disease is considered rare. Nevertheless the knowledge of this possible outcome is important in order to prevent its complications. Recurrence of Cushing`s disease may occur up to several years post remission. Therefore careful patient follow-up is required.

(1) Pignatta AB and Bruno OD et al.,  Pituitary 2004;7(1):45-9. (2) Le Nestour E et al., Eur J Endocrinol. 1994 May;130(5):469-71 (3) Ishibashi M et al., Arch Intern Med. 1993 Jan 25; 153: 251-255

Nothing to Disclose: KM, MGJ, APL, MWV, CG, MCP, JMF

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