Background: Cushing disease defined as hypercortisolism due to pituitary adrenocorticotrophic hormone (ACTH) secreting adenoma is a very rare disease, especially in childhood and adolescence. The purpose of this report is to follow up Cushing disease patient who presented with osteoporosis, rapid weight gain, decreased growth rate, and relapsing pituitary adenoma after transsphenoidal adenomectomy (TSA).
Case: A 17-years-old boy visited our hospital for evaluation of vertebral compression fracture and obesity. His height was 149.5 cm (−4.1 SDS), body weight was 63.6 kg (−0.01 SDS), and his BMI was 38.5 kg/m2 (3.0 SDS). He looked a moon shaped face, a buffalo hump, multiple abdominal striae, truncal obesity, relatively thin limb, and hypertrichosis. The volume of testis was 8 ml, and Tanner stage of pubic hair was 3. The Z-score of bone densiometry was −4.3. The hormone findings showed high 24-h urinary free cortisol (UFC) and loss of cortisols diurnal rhythm. Also, cortisol was not suppressed by low dose of dexamethasone, but was suppressed by high dose of dexamethasone. For the evaluation of the laterality of the ACTH secretion, the bilateral inferior petrosal sinus sampling (BIPSS) after injection of corticotropin-releasing hormone (CRH) was done, resulting in no difference of the peak ACTH levels between left and right inferior petrosal sinus sampling. However, the MRI finding showed only left pituitary microadenoma sized 8 mm. Left hemihypophysectomy was performed by the TSA and the histological finding was compatible with pituitary adenoma, and the immunohistochemistry was positive in ACTH. Transiently clinical improvement was observed, but the follow-up pituitary MRI finding showed newly developed right pituitary microadenoma six months after the left hemihypophysectomy. We are planning to perform the right hemihypophysectomy.
Conclusion: This case report describes the relapsing Cushing disease patient presenting osteoporosis, rapid weight gain, and growth failure. Thus, the need for careful clinical and radiographic follow-up in surgically cured patient should be emphasized in search for potential progression of relapsing Cushing disease.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology