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Endocrine Abstracts (2020) 70 AEP2 | DOI: 10.1530/endoabs.70.AEP2

ECE2020 Audio ePoster Presentations Adrenal and Cardiovascular Endocrinology (121 abstracts)

Ovarian adrenal rest tumor in congenital adrenal hyperplasia: Is medical treatment the first line option?

Ronit Koren 1,2 , Shlomit Koren 2,3,4 , Alla Khashper 2,5 , Carlos Benbassat 4 , Marina Pekar-Zlotin 2,6 & Zvi Vaknin 2,6

1Shamirmedical center, Department of Internal Medicine A, Zerifin, Israel; 2Tel-Aviv University, Sackler faculty of medicine, Tel Aviv, Israel; 3Shamir medical center, Diabetes unit, Zerifin, Israel; 4Shamir medical center, Endocrine Institute, Zerifin, Israel; 5Shamir medical center, Diagnostic Imaging, Zerifin, Israel; 6Shamir medical center, Obstetrics and Gynecology, Zerifin, Israel

Introduction: Ovarian adrenal rest tumors (OARTs), in contrast with testicular adrenal rest tumors, are very rare. Up to date, 13 cases were reported in the literature; all treated surgically.

Aim: We describe a case of a young female with uncontrolled classical congenital adrenal hyperplasia (CCAH), presenting with bilateral OARTs, successfully treated with steroid replacement.

Methods: Data on clinical history and biochemical work-upwas obtained from medical records.

Case presentation: A 20 years old women presented with severe abdominal pain, vomiting, diarrhea,and fever. She was known to have 21OH-CCAH. As a result of poor compliance, 6 months before her admission hirsutism worsened and amenorrhea, hyperpigmentation,and weakness developed. ACTH levels were 278 < pmol/l and 17OHP 91.3 nmol/l. She was admitted for parenteral antibiotics and high dose hydrocortisone treatment. CT revealed bilateral juxta-ovarian masses (6.2 × 3.6 × 7.4 cm left and 5 × 2.2 × 3.2 cm right) that on MRI were iso-intense in T1 and hypo-intense in T2, with early enhancement and rapid washout. Trans-abdominal US found the same masses with no ovarian torsion. One week of high dose hydrocortisone resulted in significant clinical and laboratory improvement,and a multidisciplinary teem decided to continue conservative management. The patient was discharged with 2 mg dexamethasone per day. One-month later US revealed shrinkage of the masses and dexamethasone dose was decreased to 1 mg/day. At three months from discharge, she has resumed regular menses,and a repeated MRI (Figure 1) revealed the para-ovarian masseshaveshrunk to 2.7 × 2.4 × 5 cm on the left and 1.6 × 1.4 × 2.3 cm on the right. Dexamethasone levels were decreased to 0.25 mg/day. One year after the diagnosis, the paraovarian masses shrunk to 2.8 × 1.9 × 4.3 on the left (down from 4.6 × 3.3 × 7 cm) and 2.1 × 0.9 × 1.2 on the right (down from 2.5 × 1.6 × 3.9) with less contrast enhancement in comparison to previous test possibly due to fibrotic changes of the tissue

Conclusion: OARTs are rare tumors with a poorly known natural history, and surgery has been the first option in the few reported cases. We demonstrate here that medical treatment is a good alternative, leading to significant tumor shrinkage over a short period.

Volume 70

22nd European Congress of Endocrinology

05 Sep 2020 - 09 Sep 2020

European Society of Endocrinology 

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