“The human body is the best picture of the human soul.” – Tony Robbins

PNA Spotlight: Phillip Cem Cezayirli, MD

This month the PNA Spotlight focuses on neurosurgeon Dr. Philip Cem Cezayirli, part of the Haynes Neurosurgical Group in Birmingham, Alabama. Dr. Cezayirli is affiliated with four local medical centers: Princeton Baptist, Shelby Baptist, Grandview, and Brookwood Baptist Medical Centers.

Dr. Philip Cezayirli

He is a board-certified neurosurgeon with a focus on neurosurgical oncology and spine surgery and is licensed to practice neurosurgery in Alabama.

He earned his MD at the University of Alabama at Birmingham School of Medicine in Birmingham, Alabama. He spent a year studying with Dr. Uğur Türe at Yeditepe University and Hospital in Istanbul, Turkey. He did his neurosurgery residency at the Albert Einstein College of Medicine in Montefiore, Bronx New York. And he completed a neurosurgical oncology fellowship at MD Anderson Cancer Center in Houston, TX.

Dr. Cezayiri was kind enough to speak with the PNA about a range of issues. Here is the conversation, edited for clarity.

Read More Here

Expertise needed for tumors that invade the pituitary’s protective layer

The pituitary gland is surrounded by the cavernous sinus and internal carotid artery. A thin layer of connective tissue separates the sinus and artery from the pituitary gland. Pituitary tumors sometimes invade this protective layer, called the medial wall of the cavernous sinus (MWCS).

That infiltration puts pituitary tumors in close proximity to the internal carotid artery and to cranial nerves, significantly complicating treatment approaches — especially when a tumor needs to be entirely removed to achieve cure.

“Standard procedure would be to resect the pituitary tumor up to the medial wall, to avoid damaging the artery. But if there’s tumor in the wall, the patient won’t be cured,” says Mayo Clinic neurosurgeon Dr. Kaisorn L. Chaichana.

MWCS infiltration is more common with functioning adenomas, such as prolactinomas and tumors that cause acromegaly or Cushing’s disease. Incomplete removal of these tumors can lower the chances of hormone control and increase the risk of tumor regrowth.

Fortunately, neurosurgeons are learning how to meet these challenges. Surgically removing part of the MCWS, to completely resect pituitary tumors, can improve outcomes.

“It’s a formidable challenge,” Dr. Chaichana says. “It takes people well-versed in the surgery, and who have experience, to know where they can open that medial wall. If you’re too far to the side — even by less than a millimeter — you would cut the carotid artery, which would be catastrophic.”

Mayo Clinic neurosurgeons use Doppler ultrasound to guide entry into the cavernous sinus, allowing selective resection of the MWCS. “We listen to the sound of the carotid artery, find a space within the medial wall where the artery isn’t present, and make a small slit in that opening,” Dr. Chaichana says. “Then we remove the wall from the carotid artery.”

It’s also important to limit packing of the cavernous sinus after surgery. “Overpacking that site can cause pressure and injury to the cranial nerves, which could lead to facial numbness and eye motion problems,” Dr. Chaichana says.

A multidisciplinary team that includes ENT/head and neck surgeons, as well as neurosurgeons, provides additional anatomical expertise.

“Very few centers do this procedure. But we’ve seen higher rates of remission for functional tumors when we take out that wall,” Dr. Chaichana says. “For a lot of functional tumors, selective resection of the MWCS can mean the difference between tumor remaining and tumor being gone — and therefore, cure or not cure.”

Count on your Xeris CareConnection™ Team for unparalleled Cushing’s Support

Cushing’s can be challenging, but there is support so patients can feel like themselves again. The main goal of treating Cushing’s is to get cortisol levels back to normal. This Pituitary Awareness Month, Xeris Pharmaceuticals® is highlighting the importance of one-on-one support for patients living with Cushing’s Syndrome and support for HCPs treating Cushing’s Syndrome.

Sign up to get dedicated support:

Patients: Sign up for support | Recorlev® (levoketoconazole)

HCP’s: Connect with Xeris support | RECORLEV® (levoketoconazole)

Have more questions? Call for more support at 1-844-444-RCLV (7258)

Dr. Philip Cezayirli

This month the PNA Spotlight focuses on neurosurgeon Dr. Philip Cem Cezayirli, part of the Haynes Neurosurgical Group in Birmingham, Alabama. Dr. Cezayirli is affiliated with four local medical centers: Princeton Baptist, Shelby Baptist, Grandview, and Brookwood Baptist Medical Centers.
He is a board-certified neurosurgeon with a focus on neurosurgical oncology and spine surgery and is licensed to practice neurosurgery in Alabama.
He earned his MD at the University of Alabama at Birmingham School of Medicine in Birmingham, Alabama. He spent a year studying with Dr. Uğur Türe at Yeditepe University and Hospital in Istanbul, Turkey. He did his neurosurgery residency at the Albert Einstein College of Medicine in Montefiore, Bronx New York. And he completed a neurosurgical oncology fellowship at MD Anderson Cancer Center in Houston, TX.
Dr. Cezayiri was kind enough to speak with the PNA about a range of issues. Here is the conversation, edited for clarity.
Tell me about your practice – what do you want pituitary patients to know?
I am from Birmingham, Alabama and work here now, but I did med school here at U.A.B., and then I did my training for residency up in New York at Albert Einstein College of Medicine and Montefiore Medical Center. Then I did a fellowship in Istanbul, Turkey, with Professor Mahmut Gazi Yaşargil and his mentee, Dr. Uğur Türe, in microsurgical neuroanatomy and micro neurosurgery training.
Later, I went back to my chief residency year at Einstein at Montefiore where I trained with Dr. Vijay Agarwal, a pituitary specialist who trained with the Mayo Clinic. During my fellowship in neurosurgical oncology at MD Anderson in Houston, Texas, I worked with neurosurgeons who focus on cancers of the brain, spine, skull base and nerves. We spend a third of our time working on the skull base, a large part of it, pituitaries.

What do you want patients to understand about your approach?
I think the important thing is our job is to help the patient, and so sometimes that means we don’t even need to operate. We can just watch the problem. Then sometimes, if it’s causing issues, or we think it’s going to cause issues, then we kind of have to weigh the risks and benefits with the patient. We try to use a patient-first approach. Just because we see something on imaging, that doesn’t necessarily mean we have to do surgery.
We focus on what the patient wants and needs and where they are in their life and try to balance all that. We are comprehensive and highly involved with the patient’s workup. For instance, we’ve had patients with a fairly large lesion, but they didn’t notice any vision problems. So, we waited to make sure. We got the labs and got the vision checked. The vision was completely normal, even though it looked like it was pushing on the nerves. And the labs actually came back showing that that the patient had a prolactinoma. So the patient ended up getting medical treatment with for the prolactinoma, and now the lesions smaller and the patient is completely fine.
We had another patient who was pregnant and had headaches. She got an MRI which showed a lesion in the pituitary, pushing on the nerves. But her vision was fine, and since she was in the third trimester, we watched it, and she delivered their baby. A repeat MRI three months later showed the pituitary is normal size now. So, we’ll continue to watch patients like that, just to make sure, but we don’t always have to do surgery. Our goal is to do right by the patient and focus on what they need and want.
I think it’s important with pituitary care in particular, that we use a team-based approach, and rather than it being just a neurosurgeon, an endocrinologist, an eye doctor or an E-N-T alone. We all work together to improve our patients’ outcomes.
What are the misconceptions out there that you want to clear up?
I think that there are people who may be afraid to get imaging, or afraid to see a neurosurgeon, because they think if we find something on the imaging, they’ll be forced to do surgery. That’s not how we work.
You know the old adage, when you’re a hammer, everything looks like a nail…
Right? And maybe 30 or 40 years ago, that might have been the case, especially when MRIs first came out, we would find lesions, and think that we had to operate on it. But now we know a lot more. And so, I don’t think there’s much downside to getting imaging or seeing a neurosurgeon when in doubt. Especially if you go to someone who has experience in this and understands the natural history of the diseases. No one is going to insist on surgery.
Also, there’s no downside to getting your vision checked regularly. A lot of times with pituitary problems, the vision issues can be so subtle and so slow that that you might miss them. It’s better to closely watch your health with your endocrinologist, primary care doctor, eye doctor and neurosurgeon, who are all of what symptoms can be there rather than avoiding diagnosis out of fear of needing surgery. It is much easier to preserve vision when people are doing well, than when the vision has been lost for a long period of time.

Why are you a member of the PNA? Why do you think it’s a worthy venture?
I think it’s good to have an official resource for people with a pituitary lesion and to have a support group. It’s very important for patients to know where to go and who to see and how to follow up. It’s very nice that the PNA maintains a provider directory of people who focus on lesions in the pituitary. An official, respected resource is important, so people don’t believe everything they read on the internet or social media.
Reach Dr. Cezayirli:
Haynes Neurosurgical Group
Cell: 2054825198
Office: 2057878676
Fax: 2057857944u
Email: [email protected]
Website: https://haynesneurosurgery.com
Address:
801 Princeton Ave SW
P. O. B. I, Suite 310
Birmingham, AL 35211-1307

“The human body is the best picture of the human soul.” – Tony Robbins

PNA Medical Corner:

Endogenous Cushing’s and Cancer

Dr. Maria Fleseriu

This month the PNA Medical Corner features a study co-authored by Maria Fleseriu, a  longtime member of the PNA and Professor of Endocrinology and Neurological Surgery and Director of the Pituitary Center at Oregon Health & Science University.  The study finds that endogenous Cushing’s Syndrome is linked to higher risk of cancer.

Eur J Endocrinol

2024 Jul 27:lvae098.

 doi: 10.1093/ejendo/lvae098. Online ahead of print.

Endogenous Cushing’s Syndrome and Cancer Risk

Yaron Rudman ^1 2, Maria Fleseriu ³, Laura Dery ², Hiba Masri-Iraqi ^1 2, Liat Sasson ^1 2, Tzipora Shochat ⁴, Shiri Kushnir ⁵, Ilan Shimon ^1 2, Amit Akirov ^1 2

Affiliations Expand

• PMID: 39067000    DOI: 10.1093/ejendo/lvae098

Abstract

Objective: Cancer incidence in patients with endogenous Cushing’s syndrome (CS) has never been established. We aimed to assess the cancer risk in patients with CS, as compared with individually matched controls.

Design: A nationwide retrospective matched-cohort study of patients with endogenous CS diagnosed between 2000-2023, using the database of Clalit Health Services in Israel.

Methods: Patients with adrenal carcinoma or ectopic CS were excluded. Patients with CS were matched in a 1:5 ratio, with controls individually matched for age, sex, socioeconomic status, and body mass index. The primary outcome was defined as the first diagnosis of any malignancy following a CS diagnosis. Risk of malignancy was calculated using the Cox proportional hazards model with death as a competing event.

Results: A total of 609 patients with CS and 3018 controls were included [mean age at diagnosis, 48.0±17.2 years; 2371 (65.4%) women]. The median follow-up 14.7 years (IQR, 9.9-20.2 years).Patients with CS had an increased cancer risk, with hazard ratio (HR) of 1.78 (95% CI 1.44-2.20), compared with their matched controls. The risk of malignancy was elevated in patients with Cushing’s disease (251 cases and 1246 controls; HR 1.65, 95% CI 1.15-2.36) and in patients with adrenal CS (200 cases and 991 controls; HR 2.36, 95% CI 1.70-3.29). The increased cancer risk in patients with CS persists after exclusion of thyroid malignancies.

Conclusion: Endogenous CS is associated with increased malignancy risk. These findings underscore the need for further research to establish recommendations for cancer screening in this population.

Keywords: Adrenal; Cancer; Cushing’s disease; Cushing’s syndrome; Malignancy; Pituitary.

© The Author(s) 2024. Published by Oxford University Press on behalf of European Society of Endocrinology. All rights reserved. For commercial re-use, please contact [email protected] for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact [email protected].

Reversing vision loss with successful pituitary tumor surgery

Pituitary tumors often cause vision problems. That’s because the optic nerves, which connect the eye to the brain, are located just on top of the pituitary gland. Pituitary tumors can grow

in a way that causes them to compress the optic nerves, leading to vision loss.

“Often the visual loss starts with the peripheral vision and is quite subtle,” says Mayo Clinic neurosurgeon Dr. Maria Peris Celda. “Pituitary tumors usually grow slowly and over time, people can get used to the visual loss. They may not notice the problem until the larger tumor affects their central vision.”

Fortunately, vision loss is often reversed once the tumor is surgically removed. The most common procedure is endoscopic endonasal transsphenoidal surgery. The endoscope, a small surgical camera, and surgical instruments are placed through the nostrils to access the tumor through the sinuses.

“We don’t have to make any incisions in the face,” Dr. Peris Celda says. “Very often, the visual loss either improves or is completely resolved after the operation.” Mayo Clinic was among the first institutions to extensively research the endoscopic through-the-nose approach. Now standard practice, the procedure lowers discomfort and usually requires only an overnight stay in the hospital.

ENT nose and sinus surgeons work alongside neurosurgeons during these procedures. For challenging cases, patients can benefit from the care provided by neuro-ophthalmologists: subspecialists who treat vision issues linked to neurological conditions.

Successful pituitary tumor surgery requires detailed imaging. “The pituitary gland is located in a delicate area, surrounded by very important blood vessels and nerves,” Dr. Peris Celda says. “Using the latest MRI technology allows us to understand the relationship between the tumor, brain and other important structures.”

Vision problems are just one type of complication that pituitary tumors can cause. “A specialized team that focuses on skull base diseases can help provide patients with the best possible outcomes and return to normal quality of life,” Dr. Peris Celda says.

Mayo Clinic is one of the leading pituitary centers in the U.S.  For more information, please visit careinfo.mayoclinic.org/pituitary-tumor 

Count on your Xeris CareConnection™ Team for unparalleled Cushing’s Support

Cushing’s can be challenging, but there is support so patients can feel like themselves again. The main goal of treating Cushing’s is to get cortisol levels back to normal. This Pituitary Awareness Month, Xeris Pharmaceuticals® is highlighting the importance of one-on-one support for patients living with Cushing’s Syndrome and support for HCPs treating Cushing’s Syndrome.

Sign up to get dedicated support:

Patients: Sign up for support | Recorlev® (levoketoconazole)

HCP’s: Connect with Xeris support | RECORLEV® (levoketoconazole)

Have more questions? Call for more support at 1-844-444-RCLV (7258)

PNA Medical Corner: Rathke’s Cleft Cysts And IGF-1 and BMI  in Acromegaly

This month, the PNA Medical Corner features two studies co-authored by Dr. Paul Gardner, a neurosurgeon at the University of Pittsburgh School of Medicine and a member of the PNA.

The first study looks at neurosurgery for Rathke’s cleft cysts

https://pubmed.ncbi.nlm.nih.gov/38848597/

Endoscopic endonasal resection of Rathke cleft cysts: a single-institution analysis of 148 consecutive patients

Hanna N Algattas ¹, Zachary C Gersey ², David Fernandes Cabral ², Ali A Alattar ², Hussein Abdallah ², Nallammai Muthiah ², Anamil Khiyami ^3 4, Neha Mehrotra ³, Tiba Abdulwahid ³, Eric W Wang ⁵, Carl H Snyderman ⁵, Georgios A Zenonos ², Pouneh K Fazeli ³, Paul A Gardner ²

Affiliations expand

• PMID: 38848597 DOI: 10.3171/2024.3.JNS232740

Abstract

Objective: The traditional treatment of sellar Rathke cleft cysts (RCCs) generally involves transsellar drainage; however, suprasellar RCCs present unique challenges to appropriate management and technical complexity. Reports on overall outcomes for the endoscopic endonasal approach (EEA) for this pathology are limited. The EEA for RCCs allows three surgical techniques: marsupialization, fenestration, and fenestration with cyst wall resection.

Methods: The authors performed a retrospective review of consecutive patients with RCCs that had been treated via an EEA at a single institution between January 2004 and May 2021. Marsupialization entailed the removal of cyst contents while maintaining a drainage pathway into the sphenoid sinus. Fenestration involved the removal of cyst contents, followed by separation from the sphenoid sinus, often with a free mucosal graft or vascularized nasoseptal flap. Cyst wall resection, either partial or complete, was added to select cases.

Results: A total of 148 patients underwent an EEA for RCC. Marsupialization or fenestration was performed in 88 cases (59.5%) and cyst wall resection in 60 (40.5%). Cysts were classified as having a purely sellar origin (43.2%), sellar origin with suprasellar extension (37.8%), and purely suprasellar origin (18.9%). Radiological recurrence was demonstrated in 22 cases (14.9%) at an average 39.7 months’ follow-up (median 45 months, range 0.5-99 months), including 13 symptomatic cases (8.8%). Cases with cyst wall resection had no significantly different rate of recurrence (11.7% vs 15.9%, p = 0.48) or postoperative permanent anterior pituitary dysfunction (21.6% vs 12.5%, p = 0.29) compared to those of fenestrated and marsupialized cases. There was no significant difference in postoperative permanent posterior pituitary dysfunction based on technique, although such dysfunction tended to worsen with cyst wall resection (13.6% vs 4.0%, p = 0.09). Based on cyst location, purely suprasellar cysts were more likely to have a radiological recurrence (28.6%) than sellar cysts with suprasellar extension (12.5%) and purely sellar cysts (9.4%; p = 0.008). Most notably, of the 28 purely suprasellar cysts, selective cyst wall resection significantly improved the long-term (10-year) recurrence risk compared to fenestration alone (17.4% vs 80.0%, p = 0.0005) without any significant added risk of endocrinopathy.

Conclusions: Endoscopic endonasal marsupialization or fenestration of sellar RCCs may be the ideal treatment strategy, whereas purely suprasellar cysts benefit from partial cyst wall resection to prevent recurrence. Selective cyst wall resection reduced long-term recurrence rates without significantly increasing rates of hypopituitarism.

Keywords: Rathke cleft cyst; cyst resection; endoscopic endonasal approach; fenestration; marsupialization; peripheral nerve; suprasellar.

The second study looks at IGF-1 and finds that it is positively associated with BMI in patients with acromegaly.

https://pubmed.ncbi.nlm.nih.gov/36930352/

Anamil Khiyami ^1 2, Neha Mehrotra ¹, Sharini Venugopal ¹, Hussain Mahmud ¹, Georgios A Zenonos ³, Paul A Gardner ³, Pouneh K Fazeli ⁴

Affiliations expand

• PMID: 36930352 DOI: 10.1007/s11102-023-01307-6

Abstract

Purpose: Acromegaly is a disorder characterized by IGF-1 excess due to autonomous GH secretion. In individuals without acromegaly, IGF-1 is not only influenced by GH secretion but is also sensitive to other factors including nutritional status, as evidenced by the inverted U-shaped association between BMI and IGF-1; in low-weight individuals (BMI < 18.5 kg/m²) and those who are obese, IGF-1 levels may be frankly low. It is not known if this same relationship between BMI and IGF-1 is also observed in acromegaly.

Methods: Retrospective study including patients who underwent resection of a pituitary adenoma (n = 197) for either acromegaly (n = 32) or a nonfunctioning adenoma (NFPA, n = 165) at a large academic medical center between 1/1/2015 and 5/31/2021.

Results: Median BMI in acromegaly was 30.8 kg/m² (range 20.9-42.6 kg/m²). Percent upper limit of normal (%ULN) IGF-1 was 228.2% [159.0, 271.4] in acromegaly versus 32.2% [18.5, 50] in NFPA (p < 0.0001). There was a significant positive association between BMI and %ULN IGF-1 (R = 0.35, p < 0.05) in acromegaly. In contrast, there was no association between BMI and %ULN IGF-1 in the NFPA group as a whole (p = 0.22), but a significant inverse association between BMI and %ULN IGF-1 in NFPA patients with a BMI ≥ 35 kg/m² (rho = – 0.39, p = 0.02).

Conclusion: In contrast to individuals without acromegaly, BMI is significantly and positively associated with IGF-1 in acromegaly across the weight spectrum. Future studies are needed to determine if obese patients with acromegaly experience more significant symptoms related to their disease, or if patients with a low BMI may require different diagnostic criteria.

Keywords: Acromegaly; BMI; IGF-1; Obesity.

Mastering the Art of Complex Pituitary Tumor Surgery

Treatment for complex pituitary tumors often involves surgical removal. Surgery can be challenging, due to the pituitary gland’s location at the base of the skull. For optimal results, surgeons need deep knowledge of skull base anatomy.

“The anatomy is very complex, in terms of the carotid artery, other blood vessels and cranial nerve structures. But with profound understanding of that anatomy, what was very complex becomes less challenging,” says Mayo Clinic neurosurgeon Dr. Joao Paulo Cavalcante de Almeida.

Mayo Clinic’s Skull Base Lab provides education for surgeons and trainees from around the globe. A collaboration between Neurosurgery and ENT/Head and Neck Surgery, the lab is fully equipped for coursework and hands-on activities. The facility also serves as a hub for curriculum development, research and innovation in skull base surgery.

“People who come to our lab learn techniques that allow them to deliver high-quality care — which is the essential mission of our institution,” Dr. Almeida says. “This lab helps us not only to provide the best possible care for our own patients but also to spread education and innovation to other places.”

One example is training surgeons to treat pituitary tumors that invade a structure known as the medial wall of the cavernous sinus, or MWCS. These challenging tumors are associated with higher rates of recurrence. But surgical removal of the MWCS risks damaging blood vessels and nerves.

“Going into the cavernous sinus has always been a limiting factor for successful surgery of complex pituitary adenomas,” Dr. Almeida says.

The Skull Base Lab trains surgeons in selective, or partial, removal of the MWCS. The procedure can minimize complications while improving rates of tumor remission and hormone control. However, selective MWCS resection requires a profound understanding of anatomy.

“It is not a technique to be performed routinely. It should be used only by highly trained, experienced surgeons at a center of excellence with a team approach,” Dr. Almeida says. At Mayo Clinic, the team includes specialists in neurosurgery, ENT/head and neck surgery, endocrinology and radiation oncology.

Patient selection is key. The procedure is used mostly to treat functioning pituitary tumors that have clear indications of MWCS infiltration. “As we have grown more experienced, we have explored the use of selective MWCS resection in certain nonfunctioning pituitary tumors and for patients with recurrent pituitary tumors that are likely to have MWCS infiltration,” Dr. Almeida says.

Education is a core commitment at Mayo Clinic. “Successful surgery relies on the caliber of people and their training, and their ability to work as a team,” Dr. Almeida says. “Patients can benefit when we are at the top of our game.”

Mayo Clinic is one of the leading pituitary centers in the U.S.  For more information, please visit careinfo.mayoclinic.org/pituitary-tumor

Count on your Xeris CareConnection™ Team for unparalleled Cushing’s Support

Cushing’s can be challenging, but there is support so patients can feel like themselves again. The main goal of treating Cushing’s is to get cortisol levels back to normal. This Pituitary Awareness Month, Xeris Pharmaceuticals® is highlighting the importance of one-on-one support for patients living with Cushing’s Syndrome and support for HCPs treating Cushing’s Syndrome.

Sign up to get dedicated support:

Patients: Sign up for support | Recorlev® (levoketoconazole)

HCP’s: Connect with Xeris support | RECORLEV® (levoketoconazole)

Have more questions? Call for more support at 1-844-444-RCLV (7258)