A Retrospective Review of 34 Cases of Pediatric Pituitary Adenoma

Abstract

Purpose

The purpose of this paper is to study invasiveness, tumor features and clinical symptoms of pediatric pituitary adenoma, and to discuss some inconclusive results in prior studies.

Methods

We retrospectively reviewed 34 cases of children (<20 year-old) who were pathologically diagnosed with pituitary adenoma and surgically treated from 2010 to 2017. Data of general information, clinical symptoms, invasive behaviors, surgery approaches, and tumor features were collected and analyzed.

Results

Sixteen boys and 18 girls aged from 12 to 19 years old were included. Prolactinoma was most suffered, followed by GH-, none- and ACTH-secreting pituitary adenoma. Invasive behaviors were observed frequently and suprasellar extensions were most found. Macroadenoma account 70% of all cases. Meanwhile, unlike prior studies, a significant raise of incidence on invasive tumor and pituitary adenoma apoplexy were observed. Craniotomy and transsphenoidal surgery were both applied with zero mortality. Nine cases occurred with transient hypopituitarism and diabetes insipidus. Three cases of tumor recurrence received secondary surgery or radiotherapy.

Conclusions

Invasive behaviors were more frequent than previous prediction. Craniotomy is worth considering for total tumor removal. Pituitary adenoma apoplexy needs further studies since its different features between children and adults in present study. Specialized care and teamwork of neurosurgeons, pediatricians, and endocrinologists are important.

Keywords

Pediatric pituitary adenoma Invasion Pituitary apoplexy Transsphenoidal surgery 

Yes, You Need a Medical Alert Bracelet!

Shared with permission from https://aiunited.org/medicalbracelets/

Advice from a Volunteer Firefighter with Adrenal Insufficiency

My name is Jeannie, and I have been diagnosed with Secondary AI since March of 2015. To make a long story short, I was diagnosed with a pituitary adenoma in Feb of 2015. It was apron 8mm x 10mm at the time wit was found. On April 25th, 2015 I was getting ready for work in the early morning hours.. I passed out in my bathroom and was immediately rushed to the hospital by my husband. They did a secondary MRI and found that the tumor had tripled in size to 23mm x almost 41mm. My Cortisol was also so low it was undetectable by the lab. I was taken by ambulance to Emory University hospital in Atlanta where the Neurosurgeon I had been in consult with was. I arrived there on Sunday and was in Surgery Wednesday Morning. The surgery took 9 hours and recovery was close to 6.

Prior to this all taking place I was a volunteer firefighter, and had worked EMS for almost 16 years. I have been in nearly every situation possible. I see so many people that have our condition asking about Medic Alert bracelets, What kind they should have, what it should say on it, etc. So I have put together a short list to help out a little bit. This is coming from both someone who has this extremely rare disease, and also from the Emergency Medical Side of me. Knowing what We as medics look for in the field, How quickly things move, what we ask or need to know, etc.

Please know that this IS NOT Professional Medical advice, But this is advice coming from someone who can shed some light on how to potentially save your life if you should ever be alone, or without anyone who knows your condition and you fall unconscious or are unable to the the responders what your condition is.

First off if you are looking for a medic alert bracelet or wondering if you should get one.

**The answer is yes, If you have been diagnosed ANY TYPE of adrenal insufficient or are on replacement medication.. YES. you need one.**

Here are some of those reasons and some pointers on what they should look like / what they should say.

#1– If you are found unconscious, and there are not bystanders around to tell emergency crews what is wrong with you, You will go longer without your steroids. If we see on your bracelet that you are steroid dependent, it dissolves the ENTIRE guessing game of why you’re unconscious.

#2– It should have on there your emergency contact and a GOOD telephone number. That way if nothing else. We can call them. NOT EVERY EMS AGENCY HAS ACCESS TO THE “CLOUD” BASED SYSTEMS THAT STORE YOUR INFO. Please be sure that if your emergency contacts number changes.. You change it on your bracelet. There is nothing worse than wasted time calling a number that doesn’t belong to the person we NEED to talk to.

#3– DO NOT MAKE IT “PRETTY” OR “NOT SO OBVIOUS”. I can not stress this enough. Ladies I know that you want the cute ones that look like normal bracelets, and have pretty charms, etc on them… THE ENTIRE point of a medical alert bracelet is that someone needs to see it and know that they should look at it. If it looks like a regular bracelet or regular necklace and it isn’t obvious within the first 3 seconds once we get to you and look in the obvious places (neck/wrist). it will NOT get seen. I promise you, we are too busy trying to play the guessing game of why you are not responding, than to take a look at every single piece of regular jewelry and see if it might have a really small inscribing of what is wrong with you. Once again. Make it noticeable. We will see that we need to look at it. Once we do. The guessing game is more than likely OVER. and we can begin to treat you appropriately.

#4– Most EMT’s and Paramedics Don’t Understand or know about Addisons or the treatment involved. If nothing else, carry a letter from your doctor explaining what is wrong with you, etc. It is very rare, and NOT covered in most Paramedic courses. So please, for those of you that put “ADRENAL INSUFFICIENT” on your bracelet and NOT “STEROID DEPENDENT” please keep in mind that you may end up with the Paramedic that just graduated, is nervous, and will mistake adrenal insufficiency for Adrenaline insufficiency.. and try to give you epinephrine. Please understand that I have seen this almost happen. It is something that is easily misunderstood in the heat of the moment.

#5– If you have an emergency injection that you carry with you all the time, on your person, or somewhere close. PUT ON THERE THAT YOU HAVE IT! MOST ems agencies have standing protocol that they can assist with emergency medications (Don’t jump in here if you are one of those states that doesn’t allow it.. I said most) That way if we find you down, and look at your bracelet, AND see that you have emergency meds with you… guess what now, not only is the guessing game over, You’re ALSO getting the RIGHT EMERGENCY MEDS, instead of us having to either give you what we carry, or you having to wait until you get to the ER and the ER doc has to go through your file and figure out that you need the medication that’s been in your pocketbook the ENTIRE TIME.If you are unsure if your state allows this, or if you Local EMS agency can do this. Contact their local medical control and ask. If they do, Please offer to give a small talk on what the disease is and how to use the emergency kit. Most will know once they see the acto-vial, but if they do not, Please educate them. Explain to them that it can be the only thing that could save your life.

Please take the time and make sure that you have correct information on your bracelet. Secondary or Primary, the treatment in an Emergency situation is the same. So there is NO need for you to spell out if you are secondary or primary. Both get the emergency injection in case of a crisis. Both get fluid bolus, heart and blood pressure monitoring. Nothing is different when it comes to an emergency situation. If you have any questions on the wording or what to get on it. Be sure you at least have an emergency contact, That you are steroid dependent, and where your emergency injection is located.

IF you know that your local EMS agency uses the “cloud” for stored emergency info, you can spend the money to get it. But I worked for service that covered a county with over 100 sq miles, and we didn’t use it. It is unreliable and takes too much time to log in to the system, try to read the small number on your band, type it in, etc. When you can simply put the information on the band itself.

If you have any other further questions, You can refer to AIU’s emergency page.

Growth Hormone: Improving Patients’ Lives and Boosting Mature Product Portfolios

Jul 07, 2017
Volume 37, Issue 7

easypod—an automated drug delivery device manufactured by Merck KGaA, Darmstadt, Germany for its recombinant human growth hormone, Saizen—is the only electronic, fully automated injection device for growth hormone therapy. Its features include automated dose delivery and prescription tracking, which records injection history and any missed injections, and allows patients to know when to change their cartridge by displaying how much medicine is left in the device.

Speaking to Pharm Exec, Merck KGaA’s Chief Operating Officer of Biopharma, Simon Sturge, outlines the device’s development and highlights its position in the context of a changing treatment-adherence landscape that could bring benefits both to patients and mature product portfolios.

PE: Are digital interventions in patient adherence becoming more of a focus at your company?

STURGE: Absolutely. We are a major player in the area of diabetes, for example, and as we all know, lifestyle has a huge impact on the outcome of diabetes. How much we as a company should be able to offer a whole package that helps to support the lifestyle changes needed is a very important element of us preventing or delaying the onset of diabetes. In other areas, many people who are sick have a degree of depression. There are excellent apps that are reimbursed in some countries to help treat depression, and those sorts of things should be offered as part of a solution. We believe it is an essential part of our business to look holistically at the patient and bring to that patient as many practical things as possible to help them overcome their disease.

However, innovative drugs are also at the core of what we do. A few years ago, we established a clear strategy of driving innovation in the area of specialty products. This has taken quite some time from an R&D perspective, but it is now coming to fruition, with a focus on the areas of oncology, immuno-oncology, and immunology. We have a number of exciting innovative products coming to market, and what we’re also seeing is substantial growth on the portfolio of our established products, one of which is our growth hormone, Saizen.

PE: How much did you incorporate patients’ adherence behaviors in developing easypod?

STURGE: Quite a few of our products are biotech products that need to be given via injection. Understanding the patient need around that product, how they inject, what the issues are, particularly for children, has helped drive our e-health and digital platform. We have a number of different applications around our growth hormone product, but the most sophisticated is easypod. The device sends administration data such as time and dose to the cloud via a mobile device or home network, and then shares that data with the treating physician or carer, to be able to understand the usage of that product.

There are digital ways that you can track people and their activities, of course, but what we’ve found is that you can’t beat having somebody almost living with a patient. In some circumstances we do that. We use an external group, and they send an observer to stay with a family for several days to really understand the practical issues that surround the use of the product. It’s those kinds of insights that really help to provide solutions that are practical and that address genuine issues that the patient wants to overcome.

Adherence in using an injectable product in a chronic environment can be very low, as low as 25%, but we’ve seen in controlled studies that with easypod that we can take that up to close to 90%.

[Ramy Sourial, growth hormone franchise director at Merck KGaA, Darmstadt, Germany, adds: We worked with patient organizations and healthcare providers at different stages of planning the device, and we used focus groups and market researchers to identify the needs. During development, we conduct regular tests to check that we are on the right track. And when the product is on the market, we continue to improve the device, even small things like designing covers and designing smaller needles.]

PE: Can this higher adherence be sustained in a real-world setting?

STURGE: We’re moving to very elegant devices, more universal devices; physicians and caregivers are becoming a lot more comfortable using the data that is generated. Where the big transition needs to take place is still with the payers. The NHS (National Health Service) is one of the most sophisticated providers in terms of understanding usage of products on a more holistic basis and has a willingness to work with the pharma industry on pricing and payment mechanisms that ultimately link efficacy with payment. As governments, payers, and the industry work more closely together, this will be of benefit to all parties and especially patients.

Our responsibility as a pharma company is broader than just supplying the drug. We have worked with the NHS on schemes where they only pay if the drug is used. If adherence levels are low, they don’t pay. In some of the pilot schemes with the NHS in a real-world setting, we were getting those adherence rates of close to 90%; we think that is quite achievable in everyday use. But there’s always things you can add, adding digital gains into these things to encourage children to use these devices on a daily basis; it’s a dynamic process and our aim is to try and maintain these increased adherence rates.

PE: What would you say are the remaining challenges in patient adherence?

STURGE: One of the biggest challenges we face is data privacy, the different data privacy laws country by country. If you end up having to develop software that has to be different in every country, it becomes less meaningful. Respecting and understanding data privacy but having a broader global alignment on data privacy laws in our industry will help everybody.

It will remain a sticking point for quite some time; it’s a highly complex and politically emotive subject, for very good reasons. But our concern isn’t around data privacy, per se—it’s consistency of the regulations thereof.

 

Julian Upton is Pharm Exec’s European and Online Editor. He can be reached at julian.upton@ubm.com

From http://www.pharmexec.com/improving-patients-lives-and-boosting-mature-product-portfolios

For Pituitary Tumors, Gamma Knife™ Radiosurgery Offers Better Tumor Control

For many patients with pituitary tumors, initial surgical intervention is followed quickly by Gamma Knife™ radiosurgery. The benefits of using this radiosurgical intervention are many, and can offer better tumor control and a more positive long-term prognosis compared to surgery alone.

San Diego, CA (PRWEB) July 03, 2017

Worldwide, up to 20% of all brain tumors—those confined within the skull—are identified as pituitary adenomas.(1) Each year, almost 10,000 new pituitary tumors are diagnosed in the United States alone, with the vast majority of these tumors being pituitary adenomas. As efforts to improve treatment for patients with pituitary tumors continue, researchers are discovering just how powerful treatment with Gamma Knife™ radiosurgery can be, at facilities like the San Diego Gamma Knife Center® (SDGKC).

Recent studies have shown compelling results for patients who received treatment with Gamma Knife™ radiosurgery following traditional surgery for pituitary tumor removal. Overall, tumor control was achieved with Gamma Knife™ treatment in up to 94% of patients studied.(1) For up to 85% of these patients, tumor control was still achieved 10 years after treatment with the Gamma Knife™.(6)

Pituitary adenomas are typically benign, but they can still cause significant problems for patients due to their location in the brain.(3) Many of these tumors also secrete certain hormones, which can ultimately change the way patients’ bodies function.(3) In most cases, the first line of defense in dealing with pituitary tumors involves surgical removal of as much of the tumor as possible; however, residual tumor tissue can exist in the brain after this process.(4)

Even though many physicians choose surgery as an initial treatment, Gamma Knife™ radiosurgery can be either a primary or secondary choice for the management of pituitary tumors. Most often, Gamma Knife is used as a secondary treatment following surgery.(5) A growing number of studies show that treating patients with the Gamma Knife™ after surgical removal of pituitary tumors is extremely beneficial for patients and can dramatically improve their long-term tumor control results.

Dr. Kenneth Ott, neurosurgeon of SDGKC®, said, “The extreme accuracy of Gamma Knife radiosurgery allows effective treatment of residual pituitary tumors following surgery to remove much of the tumor volume. In my practice, residual tumors which I have operated on are treated with Gamma Knife radiosurgery which almost always stops further growth.”

Dr. Ott continued to explain that Gamma Knife radiosurgery is more effective than prior external beam methods of radiation and avoids complications to near-by sensitive structures. Tumors within a few millimeters of the optic nerves can be safely treated because of the frame-based fixation and software advantages of Gamma Knife. Tumors which are slowly growing can also be effectively treated without the need for surgery, as long as there is no visual loss from tumor compression of the optic nerves.

Patient outcomes can also be influenced by how soon after brain surgery they receive treatment with the Gamma Knife™. In many cases, patients benefit the most from early intervention with radiosurgery; some studies have suggested that patients who receive Gamma Knife treatment more than six months after their initial surgery are at a significantly greater risk of pituitary tumor progression nearly six years post-surgery.(7)

“Pituitary tumors, in general should be treated before the tumor compresses the overlying optic nerves,” said Dr. Ott. “The earlier the treatment begins, the better. The final decision regarding Gamma Knife should be made on a case-by-case basis by a surgeon who is experienced in surgery and radiosurgery.”
About San Diego Gamma Knife Center:
Since its opening, the San Diego Gamma Knife Center® has treated over 4,000 patients with various brain disorders from around the world. The facility is equipped to provide advanced radiosurgical treatment for a variety of conditions, including metastatic brain tumors, primary brain tumors, arteriovenous malformations, and functional disorders such as trigeminal neuralgia and cluster headaches.

On the campus of Scripps Memorial Hospital, the Center offers the use of its facilities to some of the top neurosurgeons and radiation oncologists in Southern California. It’s also a proud partner of the Neurosciences Department of Scripps Memorial Hospital La Jolla, helping to provide accurate diagnoses, treatment and support for a range of neurological conditions and disorders. To learn more about the San Diego Gamma Knife Center®, please visit http://www.sdgkc.com.

Sources:
1.    Sheehan J, et al. Gamma Knife radiosurgery for the management of nonfunctioning pituitary adenomas: A multicenter study. Journal of Neurosurgery. 2013;119:446. http://thejns.org/doi/full/10.3171/2013.3.JNS12766. Accessed June 20, 2017.
2.    What Are the Key Statistics About Pituitary Tumors? American Cancer Society. https://www.cancer.org/cancer/pituitary-tumors/about/key-statistics.html. Accessed June 20, 2017.
3.    What Are Pituitary Tumors? American Cancer Society. https://www.cancer.org/cancer/pituitary-tumors/about/what-is-pituitary-tumor.html. Accessed June 20, 2017.
4.    Radiation—Gamma Knife Radiosurgery for Pituitary Adenomas. Pituitary Network Association. https://pituitary.org/medical-resources/pavilions/diagnostics-scanning-and-radiological-pavilion/diagnostics-scanning-and-radiological-archive/radiation-gamma-knife-radiosurgery-for-pituitary-adenomas. Accessed June 20, 2017.
5.    Gamma Knife Radiation Therapy for Pituitary Tumors—Candidates for Gamma Knife Treatment. The Pituitary Society. http://pituitarysociety.org/patient-education/pituitary-disorders/gammaradiation/candidates. Accessed June 20, 2017.
6.    Lee C, et al. Initial Gamma Knife radiosurgery for nonfunctioning pituitary adenomas. Journal of Neurosurgery. 2014;120:647. http://thejns.org/doi/full/10.3171/2013.11.JNS131757. Accessed June 20, 2017.
7.    Gamma Knife Radiosurgery Safe in Early Pituitary Tumor. Medscape. http://www.medscape.com/viewarticle/879250. Accessed June 20, 2017.

For the original version on PRWeb visit: http://www.prweb.com/releases/2017/07/prweb14478413.htm

Drug trial begins for Cushing’s syndrome therapy

Participant enrollment has concluded for a phase 3 trial investigating the safety and efficacy of levoketoconazole, a cortisol synthesis inhibitor, for the treatment of endogenous Cushing’s syndrome, according to a press release from Strongbridge Biopharma, the drug’s developer.

The single-arm, open-label SONICS study will include the 90 enrolled participants and may allow a small number of other patients to enroll also, according to the release.

After titration to a therapeutic dose of levoketoconazole (Recorlev), participants will maintain treatment for 6 months, the primary efficacy endpoint. Longer-term evaluation for safety will extend to 1 year. A planned 6-month double blind, placebo-controlled, randomized withdrawal extension, dubbed LOGICS, will include approximately half of the participants from SONICS.

“The need for a safe and effective, next-generation cortisol synthesis inhibitor, such as Recorlev, in the treatment of Cushing’s syndrome is substantial. Through achieving target enrollment in the SONICS study, we are one step closer to better understanding the clinical value of Recorlev and potentially bringing a new therapeutic treatment option to this community,” said Matthew Pauls, president and chief executive officer of Strongbridge Biopharma.

The company expects to announce results of SONICS in the second quarter of 2018 and of LOGICS in the third quarter, according to the release.

For more information:

Clinicaltrials.gov/ct2/show/NCT01838551

From https://www.healio.com/endocrinology/adrenal/news/online/%7Bdddb8b5b-e4c8-412f-b4b8-82acde8f21a2%7D/drug-trial-begins-for-cushings-syndrome-therapy

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