Ectopic adrenocortical carcinoma located in the ovary
- 1 October 2016
- journal article
- case report
- Published by Oxford University Press (OUP) in Acta Endocrinologica
- Vol. 175 (4), K17-K23
- https://doi.org/10.1530/eje-16-0224
Abstract
Aim: Ovarian corticosteroid-producing tumors are exquisitely rare. Our aim was to describe the first case observed in our practice. Case history: A 34-year-old female was referred for Cushing’s syndrome (CS) occurring in the postpartum period. Clinical examination showed severe CS with diabetes mellitus, hypertension, and a large mass in the right lower abdomen. Biochemistry demonstrated corticotropin (ACTH)-independent CS (cortisol=1900ng/mL (n=50–250), ACTH5, associated with peritoneal and lymph node metastases. Immunohistochemical staining was positive for inhibin-α, melan-A, and SF1, demonstrating tissue of adrenal origin. After surgery, plasma glucose level spontaneously returned to normal. However, the patient died on the second post-surgical day due to catastrophic pulmonary embolism. Conclusion: In this reported case, clinical, hormonal, histological, and immunohistochemical findings confirmed a cortisol and sex hormone-producing ovarian tumor with peritoneal and lymph node metastases, a very rare but important condition to recognize.Keywords
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