Rare Nasal Cancer May Have Caused Cushing’s Syndrome

A very rare case of Cushing’s syndrome developing as a result of a large and also rare cancer of the nasal sinuses gives insights into how to screen and treat such an anomaly, of which fewer than 25 cases have been reported in literature.

Paraneoplastic esthesioneuroblastoma (ENB), a very rare type of nasal tumor, may sometimes produce excess adrenocorticotrophic hormone (ACTH), leading to symptoms of Cushing’s syndrome, according to a recent case report that describes a case of ACTH-secreting ENB. The report aims to demonstrate the importance of recognizing its pathophysiology and treatment.

The case report, “A Case of Cushing’s Syndrome due to Ectopic Adrenocorticotropic Hormone Secretion from Esthesioneuroblastoma with Long Term Follow-Up after Resection,” was published in the journal Case Reports in Endocrinology.

It describes a 52-year-old Caucasian male who had a history of high blood pressure, severe weakness, abnormal production of urine, extreme thirstiness, and confusion.

He was scheduled to undergo surgery for a 7-centimeter skull base mass; the surgery was postponed due to severe high serum potassium concentrations and abnormally high pH levels. His plasma ACTH levels also were elevated and Cushing’s syndrome was suspected. Since imaging of the chest, abdomen, and pelvis did not show any ectopic (abnormal) sources of ACTH, the ENB was suspected to be the source.

Surgery was performed to remove the tumor, which was later found to be secreting ACTH. Consequently, following the procedure, his ACTH levels dropped to normal (below detection limit) and he did not need medication to normalize serum potassium levels. He then underwent subsequent chemoradiation and has shown no sign of recurrence 30 months after the operation, which is considered to be one of the longest follow-up periods for such a case.

Researchers declared it “a case of olfactory neuroblastoma with ectopic ACTH secretion that was treated with resection and adjuvant chemoradiation.”

“Given the paucity of this diagnosis, little is known about how best to treat these patients and how best to screen for complications such as adrenal insufficiency and follow-up,” they wrote. “Our case adds more data for better understanding of this disease.”

From https://cushingsdiseasenews.com/2018/04/03/rare-nasal-cancer-caused-cushings-syndrome-case-report-says/

Doctors Use Microwave Therapy on Cushing’s Patient Too Weak to Have Surgery

Microwave therapy improved the Cushing’s syndrome of a woman whose lungs had almost failed, allowing her to have the adrenal surgery needed to control her disease, a case study showed.

Lung infections had led to her near-respiratory failure.

Cushing’s syndrome stems from the pituitary gland producing excessive amounts of adrenocorticotropic hormone. Too much of the hormone leads to the adrenal glands generating excessive amounts of another hormone, cortisol — and that overproduction results in Cushing’s. The disease’s symptoms include increasing obesity, skin problems, muscle weakness, bone loss, fatigue, cognitive difficulties, and an inability to control emotions.

Doctors often remove patients’ adrenal glands to prevent cortisol production. But in this case, the patient was not in good enough condition to have the surgery. So doctors used microwave technology to reduce her cortisol levels to the point where surgeons could operate.

The case study, published in BMJ Case Reports, was titled “Ectopic ACTH syndrome complicated by multiple opportunistic infections treated with percutaneous ablation of the adrenal glands.

Excessive pituitary gland production of adrenocorticotropic hormone is the cause of 80 percent of Cushing’s cases.

In 5 to 10 percent of cases, a tumor in another part of the body also produces the hormone, leading to excessive amounts of it in the body. When a tumor is generating the hormone, the condition is called ectopic ACTH syndrome. The patient in the case study had ACTH syndrome.

The 63-year-old woman had complained to her family doctor about weight gain, headache, weakness, and flushing. When laboratory tests led to her being diagnosed with ectopic ACTH-dependent Cushing’s syndrome, she was admitted to a hospital’s internal medicine department.

Doctors planned surgery to remove her adrenal glands, but two days before the operation was scheduled, respiratory failure sent her to the hospital’s Intensive Care Unit. There, physicians treated her for two infections in her lungs, plus infections in her blood and urinary tract. She experienced serious medical complications while in the Intensive Care Unit.

After a month, she was in good enough condition to leave intensive chair but too frail for surgery. Instead, doctors used microwaves to destroy as much of her adrenal glands as they could.

Within two weeks, her condition was better. She had been unable to leave her hospital bed while in intensive care. After the microwave treatment, she engaged in physiotherapy that led to her being able to use a two-wheeled walker to go short distances. She could also make short excursions outside the hospital with her family.

Six months later she returned to the hospital for surgical removal of her adrenal glands.

There were no complications from the operation, and doctors discharged her two days later. Her cortisol levels have been at acceptable levels since then.

“Our experience demonstrates that percutaneous ablation is a viable alternative in patients with ectopic ACTH syndrome in whom medical therapy has failed and surgical adrenalectomy is not feasible,” the researchers wrote. “Further research comparing the efficacy and complication rates between percutaneous ablation [microwave therapy] and surgical adrenalectomy is needed.” In addition, “research is needed to determine the optimal method of percutaneous intervention,” the team wrote.

From https://cushingsdiseasenews.com/2017/12/01/case-study-shows-microwave-therapy-helped-cushings-patient-who-was-too-frail-for-surgery/

Primary Adrenal Insufficiency (PAI)

 Al-Jurayyan NA
Background: Primary adrenal insufficiency (PAI) in children is an uncommon, but potentially fatal. The current symptoms include weakness, fatigue, anorexia, abdominal pain, weight loss, orthostatic hypotension, salt craving and characterized by hyperpigmentation.
Material and Methods: This is a retrospective, hospital based-study, conducted at King Khalid University Hospital (KKUH), during the period January 1989 and December 2014. Review of medical record of patient diagnosed with primary adrenal insufficiency. The diagnosis was based on medical history, physical examination and low levels of glucocorticoids and raised adrenocorticotropic hormone (ACTH). Appropriate laboratory and radiological investigations were also reviewed.
Results: During the period under review, January 1989 and December 2014, a total of 125 patients with the diagnosis of primary adrenal insufficiency were seen. Inherited disorders like congenital adrenal hyperplasia and hypoplasia were common, 85.5%. However, variable autoimmune mediated etiologic diagnosis accounted for, 13%, were also seen. The appropriate various laboratory and radiological investigations should be planned.
Conclusion: Although, congenital adrenal hyperplasia was the commonest etiology, however, congenital adrenal hypoplasia should not be over looked. The diagnosis of PAI can be challenging in some patients, and therefore appropriate serological and radiological investigations should be done.

Interview with Deborah March 30, 2016

Deborah has many symptoms but is not yet diagnosed.

interview

Deborah will be our guest in an interview on BlogTalk Radio  Wednesday, March 30 at 6:00 PM eastern.  The Call-In number for questions or comments is (845) 241-9850.

The archived interview will be available after 7:00 PM Eastern through iTunes Podcasts (Cushie Chats) or BlogTalkRadio.  While you’re waiting, there are currently 89 other past interviews to listen to!

~~~~~~

Deborah’s Bio:

Hello all,

I do not know where to begin. For many years I have been struggling with these symptoms. I have proximal weakness, intolerance to stress, blood pressure fluctuations, hyperpigmentation, reactive hypoglycemia, sweating, severe dehydration, very bad confusion, vision, memory problems, physical body changes (hump, bruises), carb intolerance, and inability to exercise.

My endocrinologist did a workup for Cushing’s disease and the midnight saliva test was high. She brushed it off as “stress”. I am seeing a doctor now that says I have POTS and Dysautonomia. My doctor says I have inappropriate adrenaline rushes.

My body is falling apart because I haven’t found a doctor who will take my symptoms and test results serious. I would like to talk to others who are having trouble getting diagnosed and also to those who have gotten diagnosed who have a good doctor.

God Bless and Thank You,
Deborah

HOME | Sitemap | Adrenal Crisis! | Abbreviations | Glossary | Forums | Donate | Bios | Add Your Bio | Add Your Doctor | MemberMap | CushieWiki

Irreversible Effects of Previous Cortisol Excess on Cognitive Functions in Cushing’s Disease | Laika’s MedLibLog

Irreversible Effects of Previous Cortisol Excess on Cognitive Functions in Cushing’s Disease | Laika’s MedLibLog.

If we listen to Cushing patients, who are “cured” and have traded Cushing’s disease for Addison’s disease, we notice that they feel better after their high levels of cortisol have normalized, but not fully cured (see two examples of ex-Cushing patients with longlasting if not irreversible health) problems in my previous post here. [added 2010-04-17)

To realize how this affects daily life, I recommend to read the photo-blog 365 days with Cushing by Robin (also author of Survive the Journey). Quite a few of her posts deal with the continuous weakness (tag muscle atrophy), tiredness (tag fatigue), problems with (short-term) memory (see tag memory)  or both (like here and here).

Scientifically the question is to which extent ex-Cushing patients score worse than other healthy individuals or chronically ill people and, if so, whether this can be attributed to the previous high levels of glucocorticoids….

via Irreversible Effects of Previous Cortisol Excess on Cognitive Functions in Cushing’s Disease | Laika’s MedLibLog.

%d bloggers like this: