“Take care of your body. It’s the only place you have to live in.”

— Jim Rohn

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

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