Adrenal gland cancer causes Cushing’s in pregnant woman

Rare malignancy carries a 'poor prognosis,' case report note

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by Andrea Lobo |

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A 28-year-old pregnant woman was diagnosed with Cushing’s caused by advanced adrenal gland cancer, a rare malignancy with a poor prognosis, scientists reported.

Her case highlights the difficulties in diagnosing and treating such tumors, particularly during pregnancy, when hormonal changes can camouflage its symptoms.

“It is reported that benign and malignant adrenocortical tumors represent 70% of all cases of CS [Cushing’s syndrome] diagnosed during pregnancy,” the scientists wrote, adding that they “represent a small percentage of adrenal tumors.”

The case report, “Adrenocortical Carcinoma with Cushing’s Syndrome and Hyperandrogenism in a 28 year old Pregnant Female,” was published in the journal AACE Clinical Case Reports.

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Woman was about 15 weeks into pregnancy when admitted to the hospital

Cushing’s syndrome is characterized by excessive levels of the stress hormone cortisol, produced by the adrenal glands located atop the kidneys. Cushing’s disease, a form of this syndrome, is caused by a tumor in the brain’s pituitary gland, which releases high amounts of adrenocorticotropic hormone (ACTH) — a hormone that signals the adrenal glands to produce cortisol.

The woman was 15 weeks and six days pregnant when she was admitted to a hospital in or near Atlanta due to high blood pressure, first noted at 12 weeks into her pregnancy. At that time, high protein levels also were found in the urine, but they lowered with dietary changes.

She reported that intermittent sharp pains in her left flank for the past two years had worsened with pregnancy, and she began experiencing shortness of breath, palpitations, and facial hair growth. Laboratory analysis showed high blood levels of cortisol and lactate dehydrogenase, a marker of tissue damage.

An MRI scan found a mass in her left adrenal gland, along with several lung nodules. Biopsies from both organs came back consistent with a diagnosis of metastatic adrenocortical carcinoma, a rare form of adrenal gland cancer that had spread to other organs. Surgical removal of the adrenal tumor was not considered due to the lung lesions.

The woman’s cortisol levels did not vary during the day, which was considered unusual. Of note, cortisol levels are known to increase gradually in pregnant women up to the time of delivery.

She also had high levels of steroids, which are produced in the adrenal glands as precursors of sexual hormones, and testosterone. Her progesterone levels also were higher than would be expected at her stage of pregnancy.

The patient was ultimately diagnosed with stage IV adrenocortical carcinoma, Cushing’s syndrome, and hyperandrogenism, or excessive levels of androgens, the sex hormones related to the development of male characteristics.

Initially, she declined chemotherapy treatment to continue her pregnancy, but she had a spontaneous abortion at 17 weeks gestation.

A chemotherapy regimen then was planned, along with metyrapone — a cortisol-lowering medication, marketed under the brand name Metopirone — to treat Cushing’s. At her request, she was discharged after 26 days in the hospital.

A few days later, she was readmitted due to shortness of breath and swelling. After fertility preservation, she started a lighter chemotherapy treatment with mitotane alone, plus metyrapone for Cushing’s and a medication for hyperandrogenism. She did not support the mitotane, and its use was stopped.

A repeat CT scan, however, found an enlargement of the adrenal tumor, with kidney and diaphragm invasion. A chemotherapy regimen was started along with dexamethasone, a glucocorticoid medication, due to potential adrenal insufficiency — a condition in which the adrenal glands fail to produce enough of certain hormones.

Treatment stopped at the start of a second chemotherapy cycle, and the woman moved to hospice care. She died a short time later.

“Although patients with ACC [adrenocortical carcinoma] have a poor prognosis, faster diagnosis could increase chances of surgical [removal] and improve overall outcomes from this rare cancer,” the researchers wrote.

“Clinicians should remember to evaluate for ACC in the setting of [Cushing’s syndrome], particularly when hyperandrogenism is co-existing,” they added.