Pituicytoma is a tumor extremely rare in childhood, with only 4 cases reported in literature. It is thought to arise from the specialized glial elements called “pituicytes.” The association of pituicytoma and Cushing’s disease (CD) has been described only once so far, in an adult patient.
A 7-year-old girl was referred for clinical signs of hypercortisolism, and a diagnosis of CD was made. MRI revealed 2 pathologic areas in the pituitary gland. The patient underwent surgery, with microscopic transsphenoidal approach, and a well-circumscribed area of pathologic tissue was identified and removed. Surprisingly, histologic and immunohistochemical study provided unequivocal evidence of pituicytoma. No pituitary adenoma could be identified.
For persistent hypercortisolism, the patient necessitated transsphenoidal endoscopic reintervention and 2 other lesions were removed. By immunohistological examination, these lesions were confirmed to be corticotropin-secreting adenoma. Unfortunately, there was no postoperative decrease in corticotropin and cortisol levels, and the patient underwent bilateral laparoscopic adrenalectomy.
Considering that we report a second case of association of pituicytoma and corticotropin-secreting adenoma, that CD is infrequent, and pituicytoma is extremely rare in childhood, the coexistence of these 2 tumors should not be considered a mere coincidence. To date, there is no conclusive evidence about the origin of these different subtypes of pituitary tumors. This case supports the hypothesis that these tumors share a common progenitor cell, which could be the folliculostellate cell.
To lessen the risk for comorbidity and death, the Endocrine Society’s newly published guidelines on the treatment of Cushing’s syndrome focus on surgical resection of the causal tumor with the goal of normalizing cortisol levels. Furthermore, there is increased emphasis on individualizing treatment options when choosing a second-line treatment.
In July 2015, the Endocrine Society published treatment guidelines to assist endocrinologists in appropriately initiating treatment or referring patients with Cushing’s syndrome to treatment. A task force of experts compiled evidence from systematic reviews and graded the strength of the recommendations.
“We hope that it will lead to improved treatment of comorbidities both before and after definitive treatment of the syndrome, and to increased individualization of patient treatment,” said chair of the task force Lynnette Nieman, MD, who is chief of the Endocrinology Consultation Service at the National Institutes of Health Clinical Center.
“There are two new drugs that were approved in 2012, and so I think that is what prompted the review. Still, medications are not the first line of treatment, but we have some new therapeutic options, and I think the idea was to help people understand where to use them,” Julie Sharpless, MD, assistant professor and director of the UNC Multidisciplinary Pituitary Adenoma Program, told Endocrinology Advisor.
“The primary treatment is surgical resection of the causal tumor(s). If that cannot be done (because the tumor is occult or metastatic) or is not successful, then the choice of secondary treatment should be individualized to the patient. The comorbidities of Cushing’s syndrome, for example hypertension and diabetes, should be treated separately as well,” Nieman said.
For example, the guidelines recommend surgical removal of the causative lesion, with the exception of cases which are unlikely to cause a drop in glucocorticoids or in patients who are not surgical candidates.
Likewise, in patients with benign unilateral adrenal adenoma, adrenalectomy by an experienced surgeon has a high rate of cure in children and adults. Because of the poor prognosis associated with adrenal carcinoma, the guidelines highlight the need for complete resection and possibly medical treatment to stabilize cortisol levels.
Other first-line treatment options include recommending surgical resection of ectopic ACTH-secreting tumors; referring to an experienced pituitary surgeon for transsphenoidal selective adenomectomy; treatments to block hormone receptors in bilateral micronodular adrenal hyperplasia; and surgical removal in bilateral adrenal disorders.
The elevated mortality rate seen in patients with Cushing’s syndrome is due to infection, venous thrombosis and cardiovascular disease (CVD). Appropriately lowering cortisol levels improves hypertension, insulin resistance, dyslipidemia and obesity in patients with Cushing’s syndrome. Therefore, the guidelines highlight the need for restoring cortisol levels and treating the associated comorbidities.
Nevertheless, the task force specifically recommends against treatment without an established diagnosis or when there are no signs of Cushing’s syndrome and hypothalamic-pituitary-adrenal laboratory studies are borderline.
In patients who are not surgical candidates or in cases of noncurative resection, the decision on whether to consider second-line treatment options such as medical therapy, radiation, bilateral adrenalectomy or repeat transsphenoidal surgery should be based on several factors. For instance, the guidelines recommend taking into consideration location and size of the tumor, patient desires, goals of treatment and level of biochemical control.
The guidelines note medical therapy should be based on cost, efficacy and individualization of treatment. Endocrinologists can approach medical therapy with a goal of establishing normal cortisol levels or reducing cortisol levels to very low levels and replacing to achieve desired levels.
Remission in Cushing’s syndrome is associated with notable improvement; however, long-term follow-up is recommended for osteoporosis, CVD and psychiatric conditions.
After treatment, patients may experience reductions in weight, blood pressure, lipids and glucose levels that may allow reduction or discontinuation of medications. Even so, patients with a history of Cushing’s syndrome tend to have higher rates of hypertension, hyperlipidemia and diabetes. Likewise, rates of myocardial infarction are higher in this population, further emphasizing the need for treatment and management of diabetes and hypertension.
Sharpless highlighted that Cushing’s syndrome is rare.
“There are multiple studies that have shown that patients do better when they are treated in a specialty center where people see a lot of cases of this. So in that sense, treatment is not usually going to fall to the general practitioner,” she said.
She continued that the guidelines are helpful and provide guidance to endocrinologist who “can’t readily refer their patient to a pituitary center.”
Sharpless went on to describe the multidisciplinary care involved in Cushing’s syndrome including endocrinologists, neurosurgeons, radiologists, counselors and radiation oncologist.
“When the care is complicated, you want to ensure all of your providers have reviewed your case together and figured out the best plan.”
The guidelines were co-sponsored by the European Society of Endocrinology. Nieman received salary support for her work on the manuscript from the Intramural Research Program of the Eunice Kennedy Shiver Institute of Child Health and Human Development. Members of the task force reported multiple disclosures.
Reference
Nieman LK et al. J Clin Endocrinol Metab. 2015;100(8):2807-2831.
Cushing’s syndrome can affect fertility in both men and women.
Women
The high levels of cortisol in Cushing’s syndrome disrupt a woman’s ovaries. Her menstrual periods may stop completely or become irregular. As a result, women with Cushing’s syndrome almost always have difficulty becoming pregnant.5,6,7For those who do become pregnant, the risk of miscarriage is high.5,6,7
In rare cases, usually when a woman’s Cushing’s syndrome is caused by a benign adrenal tumor, pregnancy can occur, but it brings high risk for the mother and fetus.5,6,7
After a woman is treated for Cushing’s syndrome, her ovaries often recover from the effects of too much cortisol. Her regular menstrual cycles will return, and she can become pregnant.8
In some women, regular periods do not return after they are treated for Cushing’s syndrome. This occurs if surgery removes the part of the pituitary gland involved in reproduction.4 An infertility specialist can prescribe hormone therapy to bring back regular periods, ovulation, and fertility.8
Men
A man diagnosed with Cushing’s syndrome may have a decline in sperm production and could have reduced fertility.9 He also might experience a lowered sex drive as well as impotence (pronounced IM-puh-tuhns). In addition, some medications used to treat Cushing’s syndrome can reduce fertility.10 However, fertility usually recovers after Cushing’s syndrome is cured and treatment has stopped.9
Does Cushing’s syndrome affect pregnancy?
Cushing’s syndrome can cause serious and potentially life-threatening effects for the mother and the fetus during pregnancy.11,12 For example, Cushing’s syndrome raises a woman’s risk of developing pregnancy-related high blood pressure (called preeclampsia, pronounced pree-i-KLAMP-see-uh, or eclampsia) and/or pregnancy diabetes, which also is called gestational (pronounced je-STEY-shuhn-ul) diabetes). Infection and slow healing of any wounds are more likely, as is heart failure. When the syndrome is caused by a tumor, it will be surgically removed as early as possible to reduce any threat.13
Biddie, S. C., Conway-Campbell, B. L, & Lightman, S. L. (2012). Dynamic regulation of glucocorticoid signalling in health and disease. Rheumatology, 51(3), 4034-4112. Retrieved May 19, 2012, from PMID: 3281495.
Pickard, J., Jochen, A. L., Sadur, C. N., & Hofeldt, F. D. (1990). Cushing’s syndrome in pregnancy. Obstetrical & Gynecological Survey, 45(2), 87-93.PMID 2405312.
Lindsay, J. R., Jonklaas, J., Oldfield, E. H., & Nieman, L. K. (2005). Cushing’s syndrome during pregnancy: Personal experience and review of the literature. Journal of Clinical Endocrinology and Metabolism, 90(5), 3077.PMID 15705919.
Stewart, P. M., & Krone, N. P. (2011). The adrenal cortex. In Kronenberg, H. M., Shlomo, M., Polonsky, K. S., Larsen P. R. (Eds.). Williams textbook of endocrinology (12th ed.). (chap. 15). Philadelphia, PA: Saunders Elsevier.
Buescher, M. A. (1996). Cushing’s syndrome in pregnancy. Endocrinologist, 6, 357-361.
Ezzat, S., Asa, S. L., Couldwell, W. T., Barr, C. E., Dodge, W. E., Vance M. L., et al. (2004). The prevalence of pituitary adenomas: A systematic review.Cancer, 101(3), 613-619. PMID 15274075.
In case you haven’t guessed it, my cause seems to be Cushing’s Awareness. I never really decided to devote a good portion of my life to Cushing’s, it just fell into my lap, so to speak – or my laptop.
I had been going along, raising my son, keeping the home-fires burning, trying to forget all about Cushing’s. My surgery had been a success, I was in remission, some of the symptoms were still with me but they were more of an annoyance than anything.
I started being a little active online, especially on AOL. At this time, I started going through real-menopause, not the fake one I had gone through with Cushing’s. Surprisingly, AOL had a group for Cushing’s people but it wasn’t very active.
What was active, though, was a group called Power Surge (as in I’m not having a hot flash, I’m having a Power Surge). I became more and more active in that group, helping out where I could, posting a few links here and there.
Around this time I decided to go back to college to get a degree in computer programming but I also wanted a basic website for my piano studio. I filled out a form on Power Surge to request a quote for building one. I was very surprised when Power Surge founder/webmaster Alice (AKA Dearest) called me. I was so nervous. I’m not a good phone person under the best of circumstances and here she was, calling me!
I had to go to my computer class but I said I’d call when I got back. Alice showed me how to do some basic web stuff and I was off. As these things go, the O’Connor Music Studio page grew and grew… And so did the friendship between Alice and me. Alice turned out to be the sister I never had, most likely better than any sister I could have had.
In July of 2000, Alice and I were wondering why there weren’t many support groups online (OR off!) for Cushing’s. This thought percolated through my mind for a few hours and I realized that maybe this was my calling. Maybe I should be the one to start a network of support for other “Cushies” to help them empower themselves.
I wanted to educate others about the awful disease that took doctors years of my life to diagnose and treat – even after I gave them the information to diagnose me. I didn’t want anyone else to suffer for years like I did. I wanted doctors to pay more attention to Cushing’s disease.
The first website (http://www.cushings-help.com) went “live” July 21, 2000. It was just a single page of information. The message boards began September 30, 2000 with a simple message board which then led to a larger one, and a larger. Today, in 2012, we have over 8 thousand members. Some “rare disease”!
This was on the intro page of Cushing’s Help until 2013…
I would like to give abundant thanks Alice Lotto Stamm, founder of Power Surge, premier site for midlife women, for giving me the idea to start this site, encouraging me to learn HTML and web design, giving us the use of our first spiffy chatroom, as well as giving me the confidence that I could do this. Alice has helped so many women with Power Surge. I hope that I can emulate her to a smaller degree with this site.
Thanks so much for all your help and support, Alice!
In August 2013 my friend died. In typical fashion, I started another website…
I look around the house and see things that remind me of Alice. Gifts, print outs, silly stuff, memories, the entire AOL message boards on floppy disks…
It’s Sunday again, so this is another semi-religious post so feel free to skip it 🙂
I’m sure that many would think that this is a semi-odd choice for all-time favorite hymn.
My dad was a Congregational (now United Church of Christ) minister so I was pretty regular in church attendance in my younger years.
Some Sunday evenings, he would preach on a circuit and I’d go with him to some of these tiny churches. The people there, mostly older folks, liked the old hymns best – Fanny Crosby and so on.
So, some of my “favorite hymns” are those that I sang when I was out with my Dad. Fond memories from long ago.
In 1986 I was finally diagnosed with Cushing’s after struggling with doctors and trying to get them to test for about 5 years. I was going to go into the NIH (National Institutes of Health) in Bethesda, MD for final testing and then-experimental pituitary surgery.
I was terrified and sure that I wouldn’t survive the surgery.
Somehow, I found a 3-tape set of Readers Digest Hymns and Songs of Inspiration and ordered that. The set came just before I went to NIH and I had it with me.
At NIH I set up a daily “routine” of sorts and listening to these tapes was a very important part of my day and helped me get through the ordeal of more testing, surgery, post-op and more.
When I had my kidney cancer surgery, the tapes were long broken, but I had replaced all the songs – this time on my iPod.
Abide With Me was on this tape set and it remains a favorite to this day. Whenever we have an opportunity in church to pick a favorite, my hand always shoots up and I request page 700. When someone in one of my handbell groups moves away, we always sign a hymnbook and give it to them. I sign page 700.
I think that many people would probably think that this hymn is depressing. Maybe it is but to me it signifies times in my life when I thought I might die and I was so comforted by the sentiments here.
This hymn is often associated with funeral services and has given hope and comfort to so many over the years – me included.
If you abide in Me, and My words abide in you, you will ask what you desire, and it shall be done for you.
~John 15:7
Abide With Me
Words: Henry F. Lyte, 1847.
Music: Eventide, William H. Monk, 1861. Mrs. Monk described the setting:
This tune was written at a time of great sorrow—when together we watched, as we did daily, the glories of the setting sun. As the last golden ray faded, he took some paper and penciled that tune which has gone all over the earth.
Lyte was inspired to write this hymn as he was dying of tuberculosis; he finished it the Sunday he gave his farewell sermon in the parish he served so many years. The next day, he left for Italy to regain his health. He didn’t make it, though—he died in Nice, France, three weeks after writing these words. Here is an excerpt from his farewell sermon:
O brethren, I stand here among you today, as alive from the dead, if I may hope to impress it upon you, and induce you to prepare for that solemn hour which must come to all, by a timely acquaintance with the death of Christ.
For over a century, the bells of his church at All Saints in Lower Brixham, Devonshire, have rung out “Abide with Me” daily. The hymn was sung at the wedding of King George VI, at the wedding of his daughter, the future Queen Elizabeth II, and at the funeral of Nobel peace prize winner Mother Teresa of Calcutta in1997.
Abide with me; fast falls the eventide;
The darkness deepens; Lord with me abide.
When other helpers fail and comforts flee,
Help of the helpless, O abide with me.
Swift to its close ebbs out life’s little day;
Earth’s joys grow dim; its glories pass away;
Change and decay in all around I see;
O Thou who changest not, abide with me.
Not a brief glance I beg, a passing word;
But as Thou dwell’st with Thy disciples, Lord,
Familiar, condescending, patient, free.
Come not to sojourn, but abide with me.
Come not in terrors, as the King of kings,
But kind and good, with healing in Thy wings,
Tears for all woes, a heart for every plea—
Come, Friend of sinners, and thus bide with me.
Thou on my head in early youth didst smile;
And, though rebellious and perverse meanwhile,
Thou hast not left me, oft as I left Thee,
On to the close, O Lord, abide with me.
I need Thy presence every passing hour.
What but Thy grace can foil the tempter’s power?
Who, like Thyself, my guide and stay can be?
Through cloud and sunshine, Lord, abide with me.
I fear no foe, with Thee at hand to bless;
Ills have no weight, and tears no bitterness.
Where is death’s sting? Where, grave, thy victory?
I triumph still, if Thou abide with me.
Hold Thou Thy cross before my closing eyes;
Shine through the gloom and point me to the skies.
Heaven’s morning breaks, and earth’s vain shadows flee;
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