Patient Develops Cyclic Cushing’s Syndrome Due to Lung Neuroendocrine Tumor

Tumors located outside the pituitary gland that produce the adrenocorticotropic hormone (ACTH) may cause, on rare occasions, cyclic Cushing’s syndrome — when cortisol levels show substantial fluctuations over time.

That finding, based on the case of a patient with ACTH-secreting lung cancer,  is found in the study, “Cyclic Cushing’s syndrome caused by neuroendocrine tumor: a case report,” which was published in Endocrine Journal.

Cushing’s syndrome is characterized by too much cortisol, either due to adrenal tumors that produce cortisol in excess, or because too much ACTH in circulation — resulting from ACTH-producing tumors — act on the adrenal glands to synthesize cortisol.

Cyclic Cushing’s syndrome (CCS) is a rare type of Cushing’s in which cortisol production is not steadily increased. Instead, it cyclically fluctuates, from periods with excessive cortisol production interspersed with periods of normal levels.

The fluctuations in cortisol levels over time pose difficulties for a definite diagnosis. Moreover, the precise mechanism underlying the periodic peaks of cortisol peaks are unknown.

Investigators now reported the case of a 37-year-old man admitted to the hospital due to repeated attacks of dizziness, weakness, and high cortisol levels for two weeks.

Repeated tests measuring the levels of cortisol in the blood and a 24-hour urine free cortisol (24 hUFC) assay confirmed a cyclic fluctuation of cortisol, with levels peaking three times and dropping twice (the standard rule for diagnosing CSC).

Upon hospitalization, he further developed high blood pressure and weight gain.

The patient underwent computed tomography (CT) scans, which revealed the presence of an ACTH-secreting tumor in the lungs, the likely cause of the patient’s Cushing’s symptoms. These type of tumors are called neuroendocrine tumors because they are able to release hormones into the blood in response to signals from the nervous system.

Additional scans detected tumors in the adrenal and pituitary glands, but further analysis revealed they were non-functioning tumors, i.e., as their name indicates, they didn’t release excessive ACTH. The thyroid gland also was positive for a tumor.

The patient underwent resection surgery to remove the tumor located in the lungs and nearby lymph nodes. After the surgery, the levels of cortisol in the blood and urine returned to normal, confirming the tumor as the source of the CSC.

The patient also received surgery to remove his thyroid tumor.

An analysis of the patient’s genomic DNA revealed a novel mutation in the PDE11A gene, which is linked to a rare form of ACTH-independent Cushing’s syndrome called primary pigmented nodular adrenocortical disease (PPNAD) type 2.

Whether the patient developed PPNAD, however, and the contribution of a potential PPNAD diagnosis to the CCS, requires further investigation. “To explore pathogenicity of the genetic mutation, we will still plan for a follow-up visit to this patient,” researchers wrote.

From https://cushingsdiseasenews.com/2019/01/24/patient-develops-cyclic-cushings-syndrome-due-to-lung-neuroendocrine-tumor/

Paediatric cyclical Cushing’s disease due to corticotroph cell hyperplasia

Cushing’s disease is very rare in the paediatric population. Although uncommon, corticotroph hyperplasia causing Cushing’s syndrome has been described in the adult population, but appears to be extremely rare in children.

Likewise, cyclical cortisol hypersecretion, while accounting for 15 % of adult cases of Cushing’s disease, has only rarely been described in the paediatric population. Here, we describe a very rare case of a 13-year old boy with cyclical cortisol hypersecretion secondary to corticotroph cell hyperplasia. The case is that of a 13-year old boy, presenting with a long history of symptoms and signs suggestive of hypercortisolism, who was found to have cyclical ACTH-dependent hypercortisolism following dynamic pituitary testing and serial late-night salivary cortisol measurements.

The patient underwent endoscopic transsphenoidal resection of the pituitary. Early surgical remission was confirmed by undetectable post-operative morning plasma cortisol levels.

Histology and immunocytochemistry of the resected pituitary tissue showed extensive corticotroph cell hyperplasia.

Conclusion: This report describes a rare case of cyclical Cushing’s disease secondary to corticotroph hyperplasia in a paediatric patient. This highlights the challenging and varied nature of Cushing’s disease and its diagnosis, and the need to keep a differential diagnosis in mind during the diagnostic process.

Author: E. Noctor S. Gupta T. Brown M. Farrell M. Javadpour C. Costigan A. Agha
Credits/Source: BMC Endocrine Disorders 2015, 15:27

From http://7thspace.com/headlines/510543/paediatric_cyclical_cushings_disease_due_to_corticotroph_cell_hyperplasia.html

Interview May 13 with Michelle B (MichelleB), Cyclic Cushing’s Patient

Hello all, I’m Michelle mother of 3 beautiful children, I work part-time, 33yrs young, non-smoker, non-drinker, overall health is good for the most part…..Where do I even begin.

I just recently received the diagnosis of cyclic Cushing’s. I’m not really sure how long I have actually had Cushing’s because I have had a diagnosis of PCOS since I was 17 yrs. old ( I’m now the ripe young age of 33). However looking back through labs with my endocrinologist who I see every 6 months, my ACTH levels have been elevated for a bit over 1 yr. It was not until recently January of 2015- things were going terribly wrong.

Starting in January I started to feel genuinely unwell, on a regular basis. I cant really explain all my symptoms there were so many different sensations and feelings that were seemingly different daily. However the red flag was I was having blood pressure spikes from really high, to very low back to back. I never had any blood pressure issues so this was a concern that led me to see a cardiologist. Upon tons of testing the cardio MD felt that something was telling my otherwise very healthy heart to do this and I should see a endocrinologist. (thank goodness for him) I contacted my endo and let him know…. the testing began.

I did every test: the midnightcortisol saliva test, dex suppression, 24 hr urine test, CRH stimulation testing. And I did them more than once. Each time it was a different response either, inconclusive, normal high, or high. I was then referred to the head of the Cleveland clinics pituitary department Dr. Kennedy. He said he is having a hard time believing when he looks at me that its Cushing’s. However all my labs say it is. I will say I do fit the mold of PCOS to a tee- which symptoms of that do coincide with Cushing’s but he still said we have to be sure its Cushing’s. To add to the mix I did have a normal MRI as well.

Dr. Kennedy started me on a 2 week midnight cortisol saliva test- Upon completion we noted levels of cortisol all over the place, some Normal, normal on high range, high, and really high. He confirmed with all the other tests this is Cushing’s. Now we are trying to figure out what is next…. and where is this damn little tumor at. he feels that it is most likely in the pituitary from my test results, but we still are not ruling out else where. He is thinking that the next step would be exploratory neurosurgery or the IPSS. I’m not sure what to think of all this, except I want to hope for the best like everyone- and just be cured!!

On a side note during all of this I also had episodes of severe pain in my chest and nausea. I went to see a GI who did an upper endo scope. They found I had eosinpphilic esophagitis. I also have never had any GI problems until now; and they came on suddenly. Im also having pain in my pancreas area- not sure if any of the two are related at all to Cushing’s. But once again I was fine until recently with all these issues at once it seems.

wish me luck on further testing, treatment, and ultimately a CURE!!

interview

Michelle was our guest in an interview on BlogTalk Radio  Wednesday, May 13, 2015.

The archived interview is available now through iTunes Podcasts (Cushie Chats) or BlogTalkRadio. There are currently 83 other past interviews for your listening pleasure!

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#1 ~ Cushing’s Myths and Facts

Myth: “Cushing’s is RARE”, “No one has Cushing’s!”, “It is literally impossible for you to have Cushing’s Disease!”

myth-busted

Fact: We have all been guilty of referring to Cushing’s as a “Rare” disease. I*, myself, say this all the time. In fact, the statistics state that only about 2 in every million people are afflicted with this disease. However, these are documented cases.

In reality, Cushing’s is not as rare as we once thought. The fact is that Cushing’s is just rarely diagnosed! Non experts tend to not test accurately and adequately for Cushing’s.

With an inappropriate protocol for testing, the prevalence of accurate diagnoses decreases. Cushing’s experts DO understand how extensive and difficult the diagnostic process is, so they tend to be more deliberate and thorough when exploring possible Cushing’s in their patients. Cushing’s patients who cycle also have to be more persistent in asking for adequate testing so that they are appropriately diagnosed.

The following video is an accurate portrayal of what many patients experience when trying to get help for their symptoms:

Please review the following links:
http://home.comcast.net/~staticnrg/Cushings/LimitationsSC_UFC_dex_mildCS.pdf
http://survivethejourney.blogspot.com/2008/11/new-research-has-shown-cushings.html

* Dr. Karen Ternier Thames

Use late-night salivary cortisol to catch recurrent Cushing’s


AT ICE/ENDO 2014


CHICAGO – Late-night salivary cortisol exceeded normal limits in 10 women with recurrent Cushing’s disease a mean of 3.5 years after transsphenoidal surgery, but their urinary free cortisol remained in normal limits, according to a retrospective review from the Medical College of Wisconsin, Milwaukee.

That adds strength to the notion that late-night salivary cortisol (LNSC) catches recurrent Cushing’s that’s missed by urinary free cortisol, even though UFC remains a standard screening approach in some places.

The study is tiny and retrospective, but at the joint meeting of the International Congress of Endocrinology and the Endocrine Society, lead investigator Dr. Ty Carroll explained why the findings still matter, and also why two LNSC measurements are better than one.

Video: http://www.clinicalendocrinologynews.com/home/article/video-use-late-night-salivary-cortisol-to-catch-recurrent-cushings/d7fad98e9289f9402034e73455b7560c.html

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