A rare source of cushing’s syndrome: ovary

Cushing’s syndrome (CS) is a rare illness that leads to visceral adiposity, muscle wasting, insulin resistance, osteoporosis, diabetes, blood clots, high blood pressure, dyslipidemia and frequent infections. Subsequently, affected individuals have increased mortality rates.

The most common cause of the syndrome is a pituitary tumor, otherwise called cushing disease. Although rare, other organs also have the potential of producing excessive cortisol. The article describes a unique case of CS where the source is a right ovarian mass. The selective ovarian and adrenal venous sampling was important in confirming the diagnosis, and eventually driving the therapy.


Also see:

Cushing syndrome related posts

Cortisol related posts

Cushing Syndrome Prevalence.jpg


Case Report

March 2018


Cushing syndrome is rarely caused by the secretion of cortisol from ovarian tumors. In clinical decision-making, it is important to determine whether the ovarian tumor is capable of secreting cortisol. Selective ovarian and adrenal venous sampling is scarcely reported in the localization of ACTH-independent ectopic Cushing syndrome.

Case Description:

We present a case of 40-year-old Chinese woman who had weight gain, hirsutism, hypertension, and menstrual disorder over 6 months. Her physical examination and biochemical assessment revealed ACTH-independent Cushing syndrome.

Adrenal computed tomography scan indicated no abnormality. A mass of 5.7 cm × 4.2 cm × 3.4 cm was discovered by pelvic ultrasonography. Somatostatin receptor scintigraphy revealed no abnormal radioactivity intake. Combined ovarian and adrenal venous sampling together with a cortisol assay were conducted.

Results revealed cortisol concentration of the right-side ovarian vein, left-side ovarian vein, and peripheral vein of 269, 29, and 35 μg/dL, respectively, suggesting a right-side ovarian origin.

A right-side salpingo-oophorectomy was performed and the pathological diagnosis revealed ovarian steroid cell tumor, not otherwise specified. The cortisol level was substantially lower after the patient underwent surgery and symptoms of Cushing syndrome disappeared. At 3-year follow-up, the patient remained disease free, and no tumor was observed on pelvic ultrasonogram.


Combined ovarian and adrenal venous sampling is valuable in the localization of adrenocorticotropic hormone–independent ectopic Cushing syndrome.