Prompt diagnosis, treatment key to minimizing pregnancy problems

Study followed 5 women in China with Cushing’s caused by adrenal tumors

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by Andrea Lobo |

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A pregnant woman holds her belly as she strolls.

A timely diagnosis of Cushing’s syndrome during pregnancy, along with appropriate individualized treatment, is critical for minimizing complications for the mother and the child, according to a case series study that followed five pregnant women with Cushing’s caused by adrenal tumors at a hospital in China.

“By implementing a multidisciplinary team … [Cushing’s syndrome] in pregnant women can be effectively managed, leading to improved outcomes,” the researchers wrote in “Cushing’s Syndrome in Pregnancy Secondary to Adrenocortical Adenoma: A Case Series and Review,” which was published in Endocrinology, Diabetes & Metabolism.

Cushing’s syndrome is a group of disorders marked by high levels of the stress hormone cortisol, but that rarely occurs during pregnancy. When it does, it’s considered a high-risk condition and if it isn’t promptly diagnosed and treated it’s associated with an increased risk of complications for both the mother and fetus.

In most cases where Cushing’s arises during pregnancy, it’s caused by adrenal adenomas, benign tumors in the adrenal glands, which sit on top of the kidneys and help produce cortisol. Cushing’s disease, which is caused by tumors in the pituitary gland, rarely occurs in pregnant women.

However, diagnosing “[Cushing’s syndrome] during pregnancy is challenging due to overlapping features with normal pregnancy,” such as a rounded moon face, high blood pressure, or stretch marks in the abdomen and legs, said the researchers, who explored the clinical presentation and best treatment options for five pregnant women with Cushing’s syndrome caused by adrenocortical adenomas, in China from August 2016 to June 2023.

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Overlap between Cushing’s, pregnancy symptoms

All the women started having symptoms during pregnancy, between 17 and 36 weeks of gestation. The most common symptoms included severe high blood pressure, a moon face, weight gain, unusual fat buildup between the shoulder blades, called a buffalo hump, and purple stretch marks in the abdomen and thighs. All the women were shown by laboratory tests to have low blood potassium levels, or hypokalemia. Four had diabetes — two were diagnosed during pregnancy, called gestational diabetes.

Because overlapping symptoms can mean missing being able to recognize Cushing’s syndrome in pregnant women, the researchers noted the presence of both hypokalemia and high blood pressure may serve as a red flag that signals the condition.

All the women had elevated blood cortisol levels, which had lost their normal daily variations. Although blood and urine cortisol levels can be elevated during pregnancy, if cortisol levels in the urine exceed three times the reference range, Cushing’s should be suspected, the researchers said.

The women also had lower than normal levels of adrenocorticotrophic hormone (ACTH), which prompts the adrenal glands to produce cortisol. These results were consistent with the presence of an ACTH-independent form of Cushing’s and led researchers to perform CT or MRI scans of the adrenal glands.

All the patients had tumors in one of the adrenal glands and underwent laparoscopic adrenalectomy, a minimally invasive surgical procedure, to remove them. Two women had the procedure during their second trimester, while three had surgery after their babies were born.

The diagnosis of adrenocortical adenoma was confirmed by analyzing the removed tumors. All the women required hormone replacement therapy with hydrocortisone to avoid adrenal insufficiency, which occurs when the adrenal glands are unable to produce enough of certain hormones.

“Treatment should be individualized due to a lack of explicit optimum therapeutic approaches. Laparoscopic adrenalectomy may be an optimal choice, along with multidisciplinary management including hormone replacement therapy,” the researchers wrote.

All the babies were born healthy, between weeks 31 and 37 weeks, except in the case of one woman who terminated her pregnancy at 17 weeks due to her condition worsening. Cortisol and/or ACTH levels returned to normal in the patients whose data was available, and their blood pressure was controlled.