ECE2019 ePoster Presentations Pituitary and Neuroendocrinology (37 abstracts)
Management challenge of child hood Cushing disease
Meriem Adel 1 , Manel Jemel 1, , Hajer Kandara 1, , Mahdi Kalthoum 1 , Sonia Nagi 2, & Ines Kammoun 1,
1National Institute of Nutrition and Food Technology Department of Endocrinology, Tunis, Tunisia; 2Manar University Tunis, Tunis, Tunisia; 3National Institute of Neurology Department of Neuroradiology, Tunis, Tunisia.
Introduction: Cushing’s syndrome (CS) is rare in children. The most common cause of CS in children is exogenous or iatrogenic CS. The most common cause of endogenous CS is Cushing disease (CD). Pediatric CD is almost always caused by a pituitary microadenoma. Here we present a rare case of (CD) related to pituitary macroadenoma.
Clinical observation: An 11 year old boy presented with complaints of excessive appetite and progressive weight gain. He had chushingoid face, skin thinning and purplish striae. He has no growth retardation. Laboratory investigations revealed an over production of cortisol (2089 pg/ml) and non-suppressive value after low-dose dexamethasone suppression test (1912 pg/ml). ACTH was 262 pg/ml. Pituitary magnetic resonance imaging (MRI) revealed an extensive macroadenoma sized 20×18×13 mm. The patient underwent a trans-sphenoidal resection. The postoperative MRI showed a subtotal tumor resection. One year later, in the follow-up, the investigations exhibit a relapse of Cushing disease and the recurrence of the macroadenoma on the pituitary MRI. The patient underwent a second operation. However 4 months later, the patient presented behavioural changes and persistent cushing disease. A third surgery was indicated followed by a radiotherapy.
Conclusion: Our case highlights the challenge of Cushing disease in children with macroprolactinoma. In several studies remission rates is poor after first transsphenoidal operation. Moreover, outcome was dismal with a second transsphenoidal operation and postoperative radiotherapy.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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