ESPE Abstracts (2014) 82 P-D-3-2-640

ESPE2014 Poster Category 3 Adrenals & HP Axis (1) (12 abstracts)

Therapeutic Troubles of Cushing’s Disease in Adolescence: Report of a Case

Manuela Deiana a , Marco Losa b , Adolfo Trettene a , Alessandra Scolari a & Alessandro Salvatoni a

aPediatric Clinic, Insubria University, Varese, Italy; bNeurosurgery Clinic, IRCCS, San Raffaele Hospital, Milan, Italy

Background: Cushing’s disease, due to ACTH-secreting pituitary adenomas, is rare in the pediatric age (0.2–0.5/ million people per year), although accounts for 75–80% of Cushing syndrome in childhood compared to 49–71% in adulthood. We report clinical presentation, diagnostic workup and treatment of a new case with major therapeutics problems.

Case report: A 15-year-old girl was referred to our clinic because of secondary amenorrhea, acne, hirsutism, and rapid weight gain (15 kg in few months). The physical examination revealed height 157 cm, weight 69.6 kg, BMI 28 kg/m2, blood pressure 140/95 mmHg, ‘full-moon face’, central obesity, wide purple striae on thighs, abdomen and arms and terminal hair on the upper abdomen, sacral region, cheeks and chin. Endocrine findings showed loss of ACTH-cortisol circadian rhythm (67 pg/ml–194 μg/ml 0800 h, 109 pg/ml–255 μg/ml at 1600 h), very high levels of midnight salivary cortisol (25.86 μg/ml) and urinary free cortisol (357 μg/24 h). High dose (8+8 mg/24 h), but not low dose (1 mg), dexametasone suppression test suppressed both serum and urinary cortisol and serum ACTH. MRI showed a 0.45 cm adenoma at the left side the anterior pituitary gland. Transsphenoidal adenomectomy was unsuccessful in reducing the levels of ACTH and cortisol and histological examination did not show the presence of ACTH-secreting cells. Three months later a bilateral inferior petrosal sinus sampling for ACTH, indicated lateralization of ACTH secretion to the right side the pituitary gland. Subsequent treatment options may be a new attempt of adenomectomy, a partial hypophysectomy or adrenalectomy. The latter option may cause Nelson syndrome.

Conclusion: Cushing’s disease in the pediatric age range may be a difficult therapeutic challenge. Trassphenoidal surgery is now considered first-line therapy, but selective microadenomectomy can be technically very difficult and an appreciable rate of failure exists.

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