Adrenocortical carcinoma masquerading as Cushing’s disease

Posted on April 1, 2017 by MaryO

BMJ Case Reports 2017; doi:10.1136/bcr-2016-217519

Summary

Cushing’s syndrome (CS) can be classified as adrenocorticotropic hormone (ACTH)-dependent or ACTH-independent depending on the ACTH levels.

However, 30% of the patients with CS have ACTH levels in the ‘grey zone’ (5–20 pg/mL), thereby posing a challenge in establishing the aetiological diagnosis. In a patient with full-blown features of Cushing’s syndrome with equivocal ACTH levels, and a pituitary microadenoma on contrast-enhanced MRI sella, can falsely lead to a diagnosis of Cushing’s disease. Pituitary microadenoma, if <6 mm in size, may be an incidental finding (incidentaloma) in this scenario and can be present in ∼3–27% of the healthy population. Therefore, in a patient with CS with equivocal ACTH levels and a pituitary microadenoma, multiple samplings for ACTH and adrenal imaging should be performed to exclude ACTH-independent CS and if required, bilateral inferior petrosal sinus sampling to determine the source of ACTH excess.

Find the entire article here: http://casereports.bmj.com/content/2017/bcr-2016-217519.full

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A Faster Way to Diagnose Cushing’s Syndrome

Posted on April 1, 2017 by MaryO

Diagnosing Cushing’s syndrome can take 24 hours of complicated and repeated analysis of blood and urine, brain imaging, and tissue samples from sinuses. But that may soon be in the past: National Institutes of Health (NIH) researchers have found that measuring cortisol levels in hair samples can do the same job faster.

Patients with Cushing’s syndrome have a high level of cortisol, perhaps from a tumor of the pituitary or adrenal glands, or as a side effect from medications. In the study, 36 participants—30 with Cushing’s syndrome, six without—provided hair samples divided into three equal segments. The researchers found that the segments closest to the scalp had the most cortisol (96.6 ± 267.7 pg/mg for Cushing’s syndrome patients versus 14.1 ± 9.2 pg/mg in control patients). Those segments’ cortisol content correlated most closely with the majority of the initial biochemical tests, including in blood taken at night (when cortisol levels normally drop).

The study was small; Cushing’s syndrome is rare, and it’s hard to recruit large numbers of patients. Still, the researchers believe it is the largest of its kind to compare hair cortisol levels to diagnostic tests in Cushing’s patients. “Our results are encouraging,” said Mihail Zilbermint, MD, the study’s senior author and an endocrinologist at NIH’s Eunice Kennedy Shriver National Institute of Child Health and Human Development. “We are hopeful that hair analysis may ultimately prove useful as a less-invasive screening test for Cushing’s syndrome or in helping to confirm the diagnosis.” The authors suggest the test is also a convenient alternative with the “unique ability” for retrospective evaluation of hypercortisolemia over months.

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From https://www.ptcommunity.com/journal/article/full/2017/4/271/research-briefs-april-2017

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Postsurgical treatment often necessary in persistent, recurrent Cushing’s disease

Posted on March 30, 2017 by MaryO

Nearly half of adults with Cushing’s disease that persists or recurs after surgical treatment require second and sometimes third therapeutic interventions, including pituitary surgical reintervention, radiotherapy, pharmacotherapy or bilateral adrenalectomy, study data from Mexico show.

Moisés Mercado, MD, FRCPC, of the ABC Hospital Neurological and Cancer Centers in Mexico City, and colleagues evaluated 84 adults (median age, 34 years; 77 women) with Cushing’s disease to determine the long-term efficacy of secondary interventions for persistent and recurrent Cushing’s disease. Median follow-up was 6.3 years.

Overall, 81 participants were primarily treated with transsphenoidal surgery. More than half experienced long-lasting remission (61.7%); disease remained active in 16%, who were diagnosed with persistent Cushing’s disease; and 22% experienced relapse after remission and were diagnosed with recurrent Cushing’s disease.

After the initial procedure, 18 participants required pituitary surgical reintervention, including 10 with recurrent and eight with persistent disease. Radiation therapy was administered to 14 participants, including two as primary therapy and 12 after failed pituitary surgery. Pharmacologic treatment with ketoconazole was prescribed for 15 participants at one point during the course of disease. Bilateral adrenalectomy was performed in 12 participants.

Pituitary surgical reintervention was the most commonly used secondary treatment (22.2%), followed by pharmacologic therapy with ketoconazole (16%), radiotherapy (14.8%) and bilateral adrenalectomy (14.8%). More than half of participants experienced early remissions after a second operation (66.6%) and radiotherapy (58.3%), whereas long-lasting remission was reached in only 33.3% of participants who underwent a second surgery and 41.6% of participants who underwent radiotherapy. Half of participants who underwent bilateral adrenalectomy were diagnosed with Nelson’s syndrome.

Overall, 88% of participants achieved remission, and disease was biochemically controlled with pharmacologic treatment in 9.5% of participants after their initial, secondary and third-line treatments.

“The efficacy of treatment alternatives for recurrent or persistent [Cushing’s disease] vary among patients, and often, more than one of these interventions is required in order to achieve a long-lasting remission,” the researchers wrote. – by Amber Cox

Disclosure: The researchers report no relevant financial disclosures.

From http://www.healio.com/endocrinology/adrenal/news/in-the-journals/%7B5519b312-5912-4c65-b2ed-2ece3f68e83f%7D/postsurgical-treatment-often-necessary-in-persistent-recurrent-cushings-disease

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Science Teacher Receives Support After Cushing’s Disease Diagnosis

Posted on March 26, 2017 by MaryO

I find it amazing that it’s newsworthy in this day and age for anyone receiving support after a diagnosis.  Of course, a diagnosed person should be getting support as a matter of course.  If she had cancer, everyone would be all over this.

For Kara Murrow, the most rewarding moments as a teacher come when students learn about animals in the classroom. So it’s difficult for the Bonham Elementary fifth-grade science and social studies teacher to be away from school while she prepares for surgery.

“I enjoy it, and I know my kids enjoy the class and enjoy science because of it,” Murrow said. “With the science club I do after school once a week, the kids get upset when it gets canceled because of meetings. Not having it now is upsetting, too.”

Murrow was diagnosed this month with Cushing’s disease, a condition that develops when a tumor on the pituitary gland causes it to secrete too much adrenocorticotropic hormone. Murrow, who moved to West Texas from Arizona three years ago, said she has received support from Midland ISD employees and others in the local community.

Murrow’s mother, Louise Gonzalez, also appreciates Midlanders’ concerns for her daughter.

“People in Midland have been wonderful, considering how new we are to the area,” Gonzalez said. “The school district sent out the GoFundMe page and there’s been an outpouring of support for that. People at my church always ask me.”

Murrow’s family is collecting donations from the website GoFundMe to cover the costs of medical and travel expenses. Murrow and her husband, Kai, recently spent money on hospital stays connected to their 4-year-old son’s food sensitivities.

“They’ve been paying off those bills and doing OK until this came,” Gonzalez said. “Plus, she’s been going to the doctor about this. Because Cushing’s is so rare, doctors don’t recognize it.”

Murrow was diagnosed with the disease after medical professionals discovered a tumor on her pituitary gland. For six years, she experienced symptoms — including weight gain, dizziness and headaches — but said doctors couldn’t determine the cause. Murrow was thankful when she received an answer.

“It was a huge relief to finally have a diagnosis and know that I wasn’t crazy or making things up,” Murrow said. “It’s weird to be excited about a brain tumor. It’s a relief to know what was happening and that I have a solution.”

Murrow traveled this week to Barrow Neurological Institute in Phoenix, where she’s scheduled to undergo surgery to remove the tumor. Though Murrow said recovery lasts several months, she hopes to return to the classroom next school year.

Jaime White, fourth-grade language arts and social studies teacher at Bonham, said both staff and students miss her presence. She said Murrow expresses concern for her students during her time away.

“She’s worried about how kids will do on the STAAR [State of Texas Assessments of Academic Readiness],” White said. “She doesn’t want them to think she abandoned them. The disease has to take center stage.”

At school, White said she noticed her colleague’s dedication toward helping her students understand science.

“She’s hands-on,” White said. “When it comes to science, she’s always making sure the kids are doing some sort of experiment. She wants to make sure the kids grasp it.”

Murrow teaches students about animals through dissections and presentations. Before she became a teacher nine years ago, she coordinated outreach programs at an Arizona zoo.

When she came to MISD, Murrow saw an opportunity to generate enthusiasm about science. She launched an invite-only science club for fifth-graders who show interest in the subject.

“I started it because there wasn’t really anything,” Murrow said. “They have tutorials for reading and math. There’s not a lot kids can do with science after school. They get science in the younger grades, but the focus is on reading and math. Science is something kids really enjoy.”

Though Murrow is disappointed about not being able to facilitate the club, she recognizes the importance of her upcoming surgery. She’s happy her mother, husband and two children will be in Phoenix for support.

“I hope that it will bring about a sense of relief to all the symptoms I’ve been dealing with and provide a chance for myself and my family to continue along with a full life,” Murrow said.

From http://www.mrt.com/news/local/article/Science-teacher-receives-support-after-11026581.php

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Suspecting Pituitary Disorders: “What’s Next?”

Posted on March 22, 2017 by MaryO

In this “3 in 3” video, Ji Hyun (CJ) Chun, PA-C, BC-ADM, covers 3 types of pituitary disorders and describes useful workups for use by primary care clinicians.

Clinician Reviews’ “3 in 3” video series delivers 3 take-home points in 3 minutes—or less.

View the video here

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