Interview with a Doctor on Trans-Sphenoidal surgery

Posted on August 3, 2016 by MaryO

Dr. Julius July: Neurosurgeon at the Neuroscience Center of Siloam Hospitals Lippo Village Karawaci 

A SIMPLE AND QUICK WAY TO REMOVE TUMORS VIA SURGERY THROUGH THE NOSTRIL

The mention of the word “surgery” evokes images of lengthy and elaborate procedures that involve delicate acts of cutting, abrading or suturing different parts of the body to treat an injury or disease.

This widely-held perception has led some to develop an irrational fear of surgery–especially if an operation involves a critical organ, such as the heart, or in the case of trans-sphenoidal surgery, a procedure used to remove tumors from the hormone-regulating pituitary gland located at the base of the brain.

Though the procedure has been around in different forms for the past three decades, individuals who may be in dire need of it might fear or avoid it.

To demystify this specific method of surgery, J+ spoke with Julius July, a neurosurgeon at the Neuroscience Center of Siloam Hospitals Lippo Village Karawaci. He has performed hundreds of trans-sphenoidal operations on patients throughout the country since 2008. Below is our interview, edited for length and clarity.

Tell us more about trans-sphenoidal surgery.

The goal is to extract benign tumors of the pituitary gland that are called pituitary adenoma. The pituitary gland controls different secretions of hormones. If there is a tumor and it grows large, one of the consequences could be that a patient goes blind. It can also lead to symptoms manifesting in other parts of the body due to excess hormone production, depending on the type of hormone affected by the tumor.

What does a neurosurgeon do during the procedure?

As neurosurgeons we use an endoscope with a camera attached to it and insert the instrument through the nostril. We go through the right nostril and through the sinus to reach the tumor and remove it. Once that is done, we add a coagulant to prevent bleeding. The operation takes only an hour to 90 minutes to perform and is minimally invasive. People come in and expect the surgery to last five or six hours. They hear “surgery” and fearfully assume that. But modern trans-sphenoidal surgery is simple, only lasting one to two hours.

What’s the prognosis after surgery?

In 80 percent of cases, all it takes is one surgery to remove a tumor. However, some need repeated intervention, while others require radiation. Some tumors want to invade their surroundings. In these cases, the surrounding area is a blood vessel. We can’t totally remove that type of tumor. But such cases are rare. If a patient needs more than two operations, we usually recommend radiation, because who wants to have a lot of operations?

What are the symptoms of pituitary adenoma?

Symptoms depend on whether a tumor affects hormone production or the optic nerve. The principal complaints are related to a patient’s field of vision becoming narrower. If there is a tumor in the pituitary gland area, the eye can’t see too widely. The tumors would press on the optic nerve, which leads to the periphery of your vision getting blurry.

If the tumor affects hormone production, the symptoms depend on the specific type of hormone that the tumor has affected. Different hormones have different roles. Excess prolactin hormones can lead to women–or even men–producing breast milk. If a woman who isn’t pregnant is producing breast milk, they need to be checked. The basic ingredient of milk is calcium. Without treatment, the woman will have porous bone problems. It also leads to reduced libido. If men have an excess of these prolactin hormones, they cannot get erections and will become impotent.

How does these problem develop in the first place?

Mutations lead to the creation of these benign tumors. Some things make mutations easier, such as smoking or exposure to radiation or specific chemicals. It could be anything. You could have eaten tofu and it had formalin or some meatballs with borax. Preventing it obviously requires a healthy lifestyle, but that’s easier said than done.

It’s not just one thing that causes these tumors.

Who does this pituitary tumor affect?

It affects both genders equally, more or less. The risk of pituitary adenoma compared to all other types of brain tumors is 15 percent. Children are also affected, though the condition is statistically much more likely to afflict adults. Of my patients, two in 70 would be children.

How is it diagnosed?

The doctor will check your hormones after a blood test and identify the problem. For example, if the condition affects growth hormones, a person can grow to two meters or more in height, which leads to gigantism. Alternatively, a condition could lead to horizontal growth–a bigger tongue, bigger fingers and changing shoes each month. The tongue can become so big that it causes breathing problems. Growth hormone overproduction is like a factory with the machine working overtime. As a result, a person’s life span can get cut in half. The heart works overtime, they keep growing and they die prematurely.

How many operations do you perform a year?

I’ve been doing these operations since 2008. I handle 60 to 70 such surgeries a year.

Any notable success stories to share?

One patient from Central Java came in blind. I examined him and said that there was no way we could save his vision by removing his tumor. He was crying. He had been blind for a week. But if no action was taken, the tumor would keep growing and would lead him to becoming crippled. At the end, he decided that he still wanted the operation. Surprisingly though, after the operation, he was able to see. Three months later, he was driving and reading newspapers. It was a fascinating case.

From http://www.thejakartapost.com/news/2016/07/30/well-being-trans-sphenoidal-surgery.html

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Pituitary tissue grown from human stem cells releases hormones in rats

Posted on June 15, 2016 by MaryO

Researchers have successfully used human stem cells to generate functional pituitary tissue that secretes hormones important for the body’s stress response as well as for its growth and reproductive functions. When transplanted into rats with hypopituitarism–a disease linked to dwarfism and premature aging in humans–the lab-grown pituitary cells promoted normal hormone release. The study, which lays the foundation for future preclinical work, appears June 14 in Stem Cell Reports, a publication of the International Society for Stem Cell Researchers.

“The current treatment options for patients suffering from hypopituitarism, a dysfunction of the pituitary gland, are far from optimal,” says first study author Bastian Zimmer of the Sloan Kettering Institute for Cancer Research. “Cell replacement could offer a more permanent therapeutic option with pluripotent stem cell-derived hormone-producing cells that functionally integrate and respond to positive and negative feedback from the body. Achieving such a long-term goal may lead to a potential cure, not only a treatment, for those patients.”

The pituitary gland is the master regulator of hormone production in the body, releasing hormones that play a key role in bone and tissue growth, metabolism, reproductive functions, and the stress response. Hypopituitarism can be caused by tumors, genetic defects, brain trauma, immune and infectious diseases, or radiation therapy. The consequences of pituitary dysfunction are wide ranging and particularly serious in children, who can suffer severe learning disabilities, growth and skeletal problems, as well as effects on puberty and sexual function.

Currently, patients with hypopituitarism must take expensive, lifelong hormone replacement therapies that poorly mimic the body’s complex patterns of hormone secretion that fluctuates with circadian rhythms and responds to feedback from other organs. By contrast, cell replacement therapies hold promise for permanently restoring natural patterns of hormone secretion while avoiding the need for costly, lifelong treatments.

Recently, scientists developed a procedure for generating pituitary cells from human pluripotent stem cells–an unlimited cell source for regenerative medicine–using organoid cultures that mimic the 3D organization of the developing pituitary gland. However, this approach is inefficient and complicated, relies on ill-defined cellular signals, lacks reproducibility, and is not scalable or suitable for clinical-grade cell manufacturing.

To address these limitations, Zimmer and senior study author Lorenz Studer of the Sloan Kettering Institute for Cancer Research developed a simple, efficient, and robust stem cell-based strategy for reliably producing a large number of diverse, functional pituitary cell types suitable for therapeutic use. Instead of mimicking the complex 3D organization of the developing pituitary gland, this approach relies on the precisely timed exposure of human pluripotent stem cells to a few specific cellular signals that are known to play an important role during embryonic development.

Exposure to these proteins triggered the stem cells to turn into different types of functional pituitary cells that released hormones important for bone and tissue growth (i.e., growth hormone), the stress response (i.e., adrenocorticotropic hormone), and reproductive functions (i.e., prolactin, follicle-stimulating hormone, and luteinizing hormone). Moreover, these stem cell-derived cells released different amounts of hormone in response to known feedback signals generated by other organs in the body.

To test the therapeutic potential of this approach, the researchers transplanted the stem cell-derived pituitary cells under the skin of rats whose pituitary gland had been surgical removed. The cell grafts not only secreted adrenocorticotropic hormone, prolactin, and follicle-stimulating hormone, but they also triggered appropriate hormonal responses in the kidneys.

The researchers were also able to control the relative composition of different hormonal cell types simply by exposing human pluripotent stem cells to different ratios of two proteins: fibroblast growth factor 8 and bone morphogenetic protein 2. This finding suggests their approach could be tailored to generate specific cell types for patients with different types of hypopituitarism. “For the broad application of stem cell-derived pituitary cells in the future, cell replacement therapy may need to be customized to the specific needs of a given patient population,” Zimmer says.

In future studies, the researchers plan to further improve the protocol to generate pure populations of various hormone-releasing cell types, enabling the production of grafts that are tailored to the needs of individual patients. They will also test this approach on more clinically relevant animal models that have pituitary damage caused by radiation therapy and receive grafts in or near the pituitary gland rather than under the skin. This research could have important implications for cancer survivors, given that hypopituitarism is one of the main causes of poor quality of life after brain radiation therapy.

“Our findings represent a first step in treating hypopituitarism, but that does not mean the disease will be cured permanently within the near future,” Zimmer says. “However, our work illustrates the promise of human pluripotent stem cells as it presents a direct path toward realizing the promise of regenerative medicine for certain hormonal disorders.”

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The researchers were supported by the New York State Stem Cell Science and the Starr Foundation. The work was further supported in part by the National Institutes of Health and the National Cancer Institute.

Stem Cell Reports, Zimmer et al.: “Derivation of diverse hormone-releasing pituitary cells from human pluripotent stem cells” http://www.cell.com/stem-cell-reports/fulltext/S2213-6711(16)30060-1

Stem Cell Reports, published by Cell Press for the International Society for Stem Cell Research (@ISSCR), is a monthly open-access forum communicating basic discoveries in stem cell research, in addition to translational and clinical studies. The journal focuses on shorter, single-point manuscripts that report original research with conceptual or practical advances that are of broad interest to stem cell biologists and clinicians. Visit http://www.cell.com/stem-cell-reports. To receive Cell Press media alerts, please contact press@cell.com.

Disclaimer: AAAS and EurekAlert! are not responsible for the accuracy of news releases posted to EurekAlert! by contributing institutions or for the use of any information through the EurekAlert system.

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Familial isolated pituitary adenoma (AIP study)

Posted on February 29, 2016 by MaryO

Professor Márta Korbonits is the Chief Investigator for the NIHR Clinical Research Network supported familial pituitary adenomas study (AIP) which is investigating the cause, the clinical characteristics and family screening of this relatively recently established disease group.

Please tell us about the condition in layman’s terms?
Pituitary adenomas are benign tumours of the master gland of the body, the pituitary gland. It is found at the base of the brain. The most commonly identified adenoma type causing familial disease makes excess amounts of growth hormone, and if this starts in childhood the patient have accelerated growth leading them to become much taller than their peers. This condition is known as gigantism.

How rare is this condition?
Pituitary adenomas cause disease in 1 in a 1000 person of the general population. About five to seven percent of these cases are familial pituitary adenomas.

How it is normally diagnosed?
There are different types of pituitary adenomas causing quite varied diseases. Gigantism and its adult counterpart acromegaly is usually diagnosed due to rapid growth, headaches, joint pains, sweating, high blood pressure and visual problems. Pituitary adenomas grow slowly and it usually takes 2-10 years before they get diagnosed. The diagnosis finally is made by blood tests measuring hormones, such as growth hormone, and doing an MRI scan of the pituitary area.

What is the study aiming to find out?
The fact that pituitary adenomas can occur in families relatively commonly was not recognised until recently. Our study introduced testing for gene alterations in the AIP (Aryl Hydrocarbon Receptor Interacting Protein) gene in the UK, and identified until now 38 families with 160 gene carriers via screening. We also aim to identify the disease-causing genes in our other families as well.

How will it benefit patients?
The screening and early treatment of patients can have a huge benefit to patients as earlier treatment will lead to less complications and better chance to recovery. We hope we can stop the abnormal growth spurts therefore avoiding gigantism. Patients that are screened will find out if they carry the AIP gene and whether they are likely to pass on the gene to their families. For most patients, knowing they have a gene abnormality also helps them to understand and accept their condition.

How will it change practice?
As knowledge of the condition becomes more understood, genetic testing of patients to screen for AIP changes should be more commonplace. Patients can be treated knowing they have this condition, and family members who are carriers of the gene can benefit from MRI scans to monitor their pituitary gland and annual hormone tests.

How did the NIHR CRN support the study?
The familial pituitary adenoma study is on the NIHR CRN Portfolio. The study’s association with NIHR has allowed the widespread assessment of the patients, has incentivised referrals from clinicians and raised awareness of both our study and the familial pituitary adenoma condition itself.

For more information contact NIHR CRN Communications Officer, Damian Wilcock on 020 3328 6705  or email damian.wilcock@nihr.ac.uk

From https://www.crn.nihr.ac.uk/blog/case_study/national-rare-disease-day-2016-familial-isolated-pituitary-adenoma-aip-study/

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Interview with Fabiana October 21

Posted on September 29, 2015 by MaryO

Fabiana had transsphenoidal surgery (pituitary) July 30th 2004.  She had a recurrence after seven years of being Cushing’s free.  A second pituitary surgery on 10/26/2011 was unsuccessful.

Another Golden Oldie, this bio was last updated 9/12/2015

interview

Fabiana will be our guest in an interview on BlogTalk Radio  Wednesday, October 21 at 6:00 PM eastern.  The Call-In number for questions or comments is (657) 383-0416.

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 88 other past interviews to listen to!

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Well it has taken me a year to write this bio…and just to give some hope to those of you just going thru this process…I have to say that after surgery I have not felt better! I am back to who i always knew I was….the depression and anxiety is gone and I am living life like a 24 year old should!

I guess it all started when i was sixteen (hindsight is 20-20 i guess). My periods stopped i was tired all the time and the depression started. We all kind of just chalked it up to being sixteen. But my mom insisted something was not right. we talked with my gyno…who said nothing was wrong, I had a fungus on my head (my hair was getting really thin) and sometimes girls who had normal periods (in my case three years of normal periods) just go awry.

My mom wasnt hearing that and demanded a script for an endo. I went….he did blood work…and metioned cushings. But nothing came back definitive…so they put me on birthcontol and gave me some hormones and the chushings was never mentioned again because that all seemed to work.

As time went on my depression got worse, the shape of my body started to change-my face and stomach was the most noticeable- and my energy level kept going down. I kept going back to the doctors asking to be tested for mono..or something. I went to a psycologist….but i knew there was no reason for my depression. Two of them told me “i had very good insight” and that I didnt need them. I started getting more anxiety..especially about going out socially.

High school ended and my typical optimistic personality started to decline. I put on a good act to my friends but my family was seeing me break down all the time. I went away for college (all the while gaining weight). My sophmore year I had a break down..I called my family crying that i needed help. I couldnt beat my depression. I didnt drink in college because i knew that would mean instant weight gain, i barely went out…i exercised everyday..hard….i joined weight watchers…i stuck with it. I was at 103 lbs….that crept up to 110…that crept up to 117…each time my weight goal would be “ohh if i could just get back to 108..112…115” with each weight gain my original weight goal would get higher and higher.

Internally i felt like I was constantly under a black cloud..i knew there was no reason why i shoudl feel this way..i was doing great in school, i had a supportive family, an amazing boyfriend and great friends…why was i depressed? I was becoming emotionally draining to the people closest to me…I would go home a lot on the weekends…i was diagnosed with PMDS….like severe PMS..and was given an antidepresant…i hated it it made me feel like a zombie…i stopped taking it and just made it apoint to work on fighting the depression….and the weight gain.

When i was done college i was about 120 lbs. My face was getting rounder and rounder..i was noticing more hair on my face and arms…and a hump between my shoulder blades and the bottom of my neck. My mom saw a tv show about Polycystic ovarian syndrome and felt that maybe that was what was going on with me…i went to my PCP with this and she said it was possible and that i should to talk to my gyno….I am 4’8 and at the time weighing close to 125..i talked to my gyno and she said I was not heavy..that i was just “itailan” ..i told her my periods were getting abnormal again even w/the birthcontrol and that i was so tired all the time and my arms and legs ached. I also told her that i was bruising very easily…and that the weight gain would not stop despite my exercising and following the atikins diet very strickly for over 6 weeks. My boyfriend and I decided to try the diet together..he lost 35 llbs in 6 weeks..i lost NOTHING! I went back to my PCP who ordered an ultra sound of my ovaries…..NOTHING.(i kept thinking i was going crazy and that it was all in my head)….she also decided to do some blood work…and as i was walking out the door she said..”you know what..i am going to give you this 24hr urine test too. Just so that we cover everything”. I just kept thinking please let something come back ….please dont let this be all my fault…please dont let this be all in my head…..please dont let me be crazy. When i got the test results back it turned out that the 24hr urine test was the one test i needed to get on the right track to finding what was wrong. My cortisol level was 3x’s the normal.

I went to an endo…by the time i got to the endocronoligist i was up to 130…i could not work a full day without needing a full day of sleep and my body was aching beyond description. I was crying all the time…in my room…and was becoming more and more of a recluse…i would only hang out with my boyfriend in our houses. I looked my symptoms up on the internet and saw cushings…that was it! I went to the endo and told him..i think it is cushings….he said he had only saw it one other time and that he wanted to do more tests. I got CAT scans, x-rays, MRI’s….my adrenals my pituitary my lungs….he did a CRH stimulation test which was getting blood work done every fifteen minutes for 90minutes….it took weeks to get that test scheduled..no one had ever heard of it and therefore did not know how to do it…..finally after 3 months of tests my dr. felt he had enough evidence to diagnos me with cushings disease (tumor on my pituitary) I was diagnosed in March of 2004. By this time i was about 137 lbs i had to work part time (i am an occupational therapist for children..i do home visits….i could not make it thru a whole day)

In April i had to change to office work…i could not lift the children and i could barely get up off the floor. I have to say i was one of the lucky people who worked for people who were very supportive and accomidating…my boss was very willing to work with me and willing to hold my job for me.

July 30th 2004 i finally had transphenodial surgery to remove my tumor (they went thru my lip and nose because they felt my nose was too small). It is now over 1 year later….i am down to 108 lbs, i have so much energy…no depression….and i dont mind looking at myself in the mirror…i am enjoying my friends and my boyfriend…(who stayed with me thru it all) And my family. I feel healthy mentally, emptionally, and physically. And i just got back into my size 2 jeans!!!

It was a crappy time…(as i am sure you all can atest to) but i learned a lot…..most importantly i was bombarded by good wishes and prayers….friends requested masses for me…a nun in brazil prayed for me…people who i never thought i touched their lives…took the time to wish me well…send an email..or call….I got to experience the wonderful loving nature of human beings and i was lucky to be supported by my family (my mom, dad, and two younger brothers) and my boyfriend throughout this entire tough journey.

This experience taught me to realize the strength i have as well as to appreciate the good and the bad in life. I was on hydrocortizone for about 8 months…i was lucky that my tumor was in its own little sack so my pituitary gland was not touched. In the end in took about 7 years to diagnose me..i think that if the dr. at 16 would have pursued the cushings idea nothing would have been found because it took so long for my symptoms to really peak…needless to say i love my PCP and my endo ..and that i changed gyno’s…

I just want to let anyone out there going thru this disease to know..you are not alone….and to take each day is stride…when you need help ask for it….and that this road can lead to a happy ending. God Bless!

ps- it is ok to feel bad about what you are going thru…it is a tough thing to endure…and when the docotors tell you there is noting wrong…..follow your gut…and you keep searching for the doctor that will listen… If there is anyone in the philadelphis of south jersey area who needs someone to talk to please feel free to email me…fapadula@hotmail.com…i will help you out the best i can!

Update November 6, 2011

Well- here is an update, after seven years of being Cushings free it has returned.

With in those seven years I married my college boyfriend and we now have a son- Nicholas who will be 2 in Decemeber. It has been a blessed and wonderful seven years. However right around when my son was turning 1 I started to notice symptoms again. Increase facial hair, the whole “roundness” of my body, buffalo hump. I decided I was going to work out hard, eat right, and see – I didnt just want to jump to any conclusions. I stuck to it- and nothing…..my hair started thinning again and the acne was coming back and then the missed periods…..so I went to my PCP- told them i needed the 24hr urine and wouldnt you know…..427 cortisol level (on that 0-50 scale)……here we go again.

So back to endo- now at Penn Pituitary Center…..it was another journey b/c the tumor wasnt definative on MRI, and it seems to be cycling…..but I was diagnosed with Cushings again- with the option of 2nd pit surgery or BLA…….after some months of trying to make a decision I went with the 50/50 chance of the second pituitary surgery on 10/26/2011.

It didnt work- my levels never came down in the hospital and I went home w/ out of range cortisol levels and no need for medication……BLURG……Sooooo on to the next step…..after I recover from this surgery I will most likely have the BLA- with the hopes of not having to deal with Cushings ever again. This time around has been a little more difficult just with being a mom and feeling sick- but I still continue to be amazingly blessed with a supportive family and husband and we are surrounded by love and support and for that I am beyond greatful.

I keep all of you in my prayers for relief and health- as I ( we all) know this no easy journey.

Many Blessings!

Fabiana

Update September 12, 2015

So to bring this up to date. My second pituitary surgery in 2011 was unsuccessful. January of 2012 I had both of my adrenal glands removed. Going to adrenal insufficiency was a very difficult transition for me. It took me nearly 2 years before I felt functional. As time went on I felt more human, but I haven’t felt healthy since that day. I can and do function, but at a lower expectation of what I used to be capable of….my “new normal”.

My husband and I decided to try for a second child…my pituitary was damaged from the second surgery and we needed fertility…after 8 months of fertility I got pregnant and we had our second son January of 2015.

In April of 2015 we discovered that my ACTH was increasing exponentially. MRI revealed a macroadenoma invading my cavernous sinus. The tumor is sitting on my carotid artery and milimeterrs away from my optic chasim. I was not a candidate for another surgery due to the tumors proximity to.both of those vital structures.

So September 1st of this year I started daily radiation treatments. I spent my 34th birthday getting my brain zapped. I am receiving proton beam therapy at the Hospital of the University of Pennsylvania. I am so lucky to live so close to an institute that has some of the rarest treatment options.

Again Cushing’s is disrupting our life, my husband goes with me every night to radiation while family takes turns watching the kids….I am now on my 18th year of fighting this disease. I never imagined it would get to this point.

But here we all are making the best of each day, fighting each day and trying to keep things as “normal” as possible. Blessings to all of you fighting this disease…my new go to saying is” ‘effing Cushing’s”! For you newbies…Fight, Advocate for yourselves, and find a doc who doesn’t dismiss you and hang on to them for dear life.

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A History of the Pituitary — Sylvia Asa, MD

Posted on May 31, 2015 by MaryO

2015 California Pituitary Conference
Dr. Sylvia Asa discusses the discoveries that have shaped our understanding of the pituitary gland, its significant and the diseases associated with it.

https://youtu.be/jzuuYe8hZmc

Filed under: Cushing's, pituitary, Video | Tagged: , , | Leave a comment »

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