Johns Hopkins Pituitary Patient Education Day

Posted on June 16, 2025 by MaryO

October 25 @ 9:00 am – 1:00 pm

The annual Pituitary Patient Education Day is a free event that features presentations from Johns Hopkins pituitary experts.

To RSVP, please email pituitaryday@jhmi.edu. Space is limited. Each person can bring up to one guest. If you RSVP yes but you cannot make it, please inform us as soon as possible by email, so that the slot can be offered to someone else.

List of presentations will be posted when finalized. Topics covered in previous years include:

Free
1800 Orleans Street, Zayed 2117
Baltimore, Maryland 21287 United States

410-955-5000

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A Case Series of Bilateral Inferior Petrosal Sinus Sampling Using Desmopressin for Evaluation of ACTH-Dependent Cushing’s Syndrome in Pediatric Patients

Posted on June 11, 2025 by MaryO

Abstract

Background

Pediatric Cushing Syndrome (CS) is rare and difficult to diagnose, especially when distinguishing ACTH-dependent subtypes. Bilateral inferior petrosal sinus sampling (BIPSS) is an essential but technically challenging procedure for this purpose. Because corticotropin-releasing hormone (CRH), the standard stimulant, has limitations, desmopressin is being explored as an alternative. This study assesses desmopressin-stimulated BIPSS for its diagnostic accuracy and tumor localization in pediatric CS within an Iranian cohort, addressing a gap in pediatric-specific diagnostic strategies and offering insights into the applicability of desmopressin in this context.

Methods

Four pediatric patients with inconclusive pituitary imaging and suspected Cushing’s disease (CD) underwent BIPSS with desmopressin at Taleghani Hospital, Tehran, Iran, between August 2015 and March 2019. Sensitivity of BIPSS for CD diagnosis was assessed, and tumor localization accuracy was evaluated during surgery.

Results

Bilateral IPSS demonstrated a sensitivity of 100% for diagnosing CD in pediatric patients. However, accuracy for tumor lateralization was moderate, with only 50% concordance between BIPSS lateralization and surgical findings. Specifically, two out of four patients had correct lateralization confirmed during surgery, while one patient with left lateralization was consistent with hypophysectomy findings. These discrepancies highlight challenges such as anatomical and drainage variations that can lead to mislocalization.

Conclusion

Desmopressin enhances the sensitivity of BIPSS for diagnosing pediatric CD, presenting as a viable alternative to CRH stimulation. Despite high sensitivity, caution is advised when interpreting BIPSS results for tumor localization. Further research is needed to optimize diagnostic strategies for pediatric CS management.

From https://link.springer.com/article/10.1007/s40200-025-01634-4

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Utility of Intraoperative Ultrasound in Identifying Pituitary Adenoma Hidden Behind a Cystic Lesion in Cushing’s Disease

Posted on May 29, 2025 by MaryO

Highlights

  • •
    Intraoperative ultrasound enhances tumor localization during endoscopic pituitary surgery for MRI-negative Cushing’s disease.
  • •
    Our findings support intraoperative US as a valuable adjunct in cases where preoperative imaging fails to reveal a lesion.
  • •
    Further studies are expected to validate the efficacy of intraoperative ultrasound as a useful tool for MRI-negative Cushing’s disease.

Abstract

Cushing’s disease with inconclusive MRI findings presents a significant diagnostic and surgical challenge due to the difficulty in localizing the causative pituitary adenoma. This case report highlights the use of intraoperative ultrasound as an adjunct for tumor detection and successful resection in a Cushing disease patient with hidden adenoma. A 55-year-old female with a history of hypertension, diabetes, and a recent cerebral infarction presented with clinical and biochemical features of Cushing’s disease. Brain MRI revealed a 10 mm non-enhancing cystic lesion in the sella, making it difficult to confirm the underlying pathology. Inferior petrosal sinus sampling suggested a right-sided lesion, leading to an endoscopic endonasal transsphenoidal surgery. Intraoperatively, ultrasound was employed to assess the sellar region, initially identifying a cystic structure consistent with a Rathke’s cleft cyst. Following fluid drainage, ultrasound revealed an iso-echoic lesion with a distinct margin, which was subsequently resected and confirmed as a pituitary adenoma on histopathological examination.
The patient experienced postoperative biochemical remission, with normalization of ACTH levels and resolution of hypertension and diabetes. This case demonstrates that intraoperative ultrasound can be a valuable tool for tumor localization in suspicious MRI-negative Cushing’s disease. By aiding in the identification of adenomas obscured by cystic lesions or surrounding structures, intraoperative ultrasound may improve surgical outcomes. Further studies are warranted to validate its efficacy in routine clinical practice.

Introduction

Cushing’s disease is caused by an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma, leading to hypercortisolism and significant metabolic disturbances. Magnetic resonance imaging (MRI) is the primary imaging modality for detecting pituitary adenomas; however, in approximately 30–40 % of cases, no visible adenoma is detected, a condition known as MRI-negative Cushing’s disease [1,2]. The absence of a discernible lesion on MRI poses a diagnostic and therapeutic challenge, often necessitating additional testing such as bilateral inferior petrosal sinus sampling (IPSS) to confirm pituitary-dependent Cushing’s syndrome [3]. Given the limitations of imaging, treatment strategies for MRI-negative Cushing’s disease require a multimodal approach.
The first-line treatment for Cushing’s disease, regardless of MRI findings, is transsphenoidal surgery. However, MRI-negative cases are associated with lower remission rates due to the difficulty in localizing the microadenoma intraoperatively [4]. When surgery fails or is not feasible, alternative treatments such as repeat surgery, radiotherapy, bilateral adrenalectomy, or medical therapy are considered [5]. Despite these therapeutic options, long-term disease control remains challenging, highlighting the need for improved diagnostic tools and targeted treatment strategies.
The authors aim to report the use of intraoperative ultrasound in a Cushing disease patient with hidden adenoma, demonstrating its possibility to detect tumors that were not visible on MRI and facilitate successful tumor resection, ultimately leading to remission.

Section snippets

Case report

A 55-year-old female patient was referred from the endocrinology department for evaluation of Cushing’s disease. She had been on medication for hypertension and diabetes for the past seven years. Two months prior, she suffered an acute cerebral infarction, resulting in left-sided hemiparesis.
The endocrinology department diagnosed her with Cushing’s disease based on a dexamethasone suppression test. Brain MRI revealed a 10 mm non-enhancing cystic mass in the right side of the sella. (Fig. 1) Due…

Diagnostic approach for MRI-negative cushing’s disease

Diagnosing MRI-negative Cushing’s disease is particularly challenging due to the absence of a visible pituitary adenoma on standard imaging. A stepwise diagnostic approach is necessary to confirm the presence of ACTH-dependent hypercortisolism, differentiate between pituitary and ectopic sources, and localize the tumor. The initial step involves biochemical confirmation of endogenous hypercortisolism through tests such as the 24-hour urinary free cortisol, and the low-dose dexamethasone…

Conclusion

MRI-negative Cushing’s disease presents significant challenges not only for neurosurgeons but also for endocrinologists. The conventional hypophysectomy is invasive and has a high risk of causing other complications. The present case report showed that intraoperative ultrasound can be used effectively in a Cushing disease patient with hidden adenoma. We hope to derive more definitive conclusions through future case studies.

CRediT authorship contribution statement

Min Ho Lee: Writing – review & editing, Writing – original draft, Data curation, Conceptualization. Tae-Kyu Lee: Writing – review & editing, Conceptualization.

Ethics approval

The study was approved by the appropriate institutional research ethics committee and certify that the study was performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.
Informed consent was obtained from the patient included in this study.

Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

References (28)

More at https://www.sciencedirect.com/science/article/abs/pii/S0967586825002516

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New Advances For Treating Complex Pituitary Tumors

Posted on May 20, 2025 by MaryO

Pituitary tumors can vary widely in severity, with some requiring surgical intervention. While certain pituitary tumors are more straightforward to remove, those that extend beyond the medial wall of the cavernous sinus (MWCS) are considered complex. A delicate region near the pituitary gland, the MWCS contains critical structures like cranial nerves and the carotid artery, which present additional surgical challenges.

Historically, surgeons avoided entering the cavernous sinus due to the risk of damaging these vital structures, but new advancements have made it possible to remove tumors more effectively and safely. Innovative surgical techniques, such as endoscopic endonasal approaches, are allowing neurosurgeons to navigate these delicate areas with greater precision and confidence than ever before.

Neurosurgeon Dr. Kaisorn Chaichana, who has performed well over 100 such procedures at Mayo Clinic in Jacksonville, Florida, says, “The latest advancements in pituitary surgery are driven by improved camera optics. Angled scopes now let us see around corners, helping us distinguish the pituitary gland from the tumor with far greater precision than traditional microscopes. Doppler and ultrasound also allow us to identify critical structures like the carotid artery, ensuring safer, more complete tumor removal.”

If a tumor that extends into the MWCS is not removed, it may continue producing excessive hormones, leaving patients with persistent symptoms. “It’s almost as if they didn’t have surgery at all because there’s still that tumor there causing that hormonal imbalance. That’s why achieving total removal is so critical for long-term success,” said Dr. Chaichana.

Although the procedure is not entirely new, widespread adoption is relatively recent, and only a few institutions perform this surgery regularly. The complexity of the procedure requires expert knowledge of the surrounding anatomy, as well as careful coordination between neurosurgeons, ear, nose, and throat (ENT) specialists, and post-operative care led by an endocrinologist. “The cavernous sinus is an area a lot of surgeons aren’t comfortable with, and that’s why we specialize in that surgery here,” added Dr. Chaichana. Mayo Clinic’s multidisciplinary approach has improved patient outcomes, allowing for safer tumor removal with reduced complications.

For patients with recurrent tumors or those initially deemed inoperable, re-evaluating surgical options at a specialized center may provide new hope. If the tumor is confined to one side of the carotid artery, surgeons can often achieve a complete resection. However, if it extends beyond this point, additional treatments such as radiation or medical therapy may be required.

Patients facing pituitary surgery should seek a neurosurgeon with experience in endoscopic techniques at a specialized facility with extensive expertise in pituitary tumor treatment.

Choosing the right surgical team can significantly impact outcomes, particularly for complex cases. While not all tumors require MWCS resection, for those that do, this approach offers a path to better surgical success and long-term remission. With ongoing advancements in pituitary surgery, more patients than ever have access to safer, more effective treatment options.

For more information or to request an appointment, please visit Pituitary Tumor Care – Mayo Clinic

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The Role of Late-Night Salivary Cortisol in the Assessment of Endocrine Remission in Patients With Cushing’s Disease After Pituitary Radiotherapy

Posted on May 19, 2025 by MaryO

ABSTRACT

Context

Pituitary radiotherapy (RT) is used for persistent/recurrent Cushing’s disease (CD) after pituitary surgery. The utility of late-night salivary cortisol (LNSC) in evaluating endocrine remission after RT is unclear.

Objective

To identify the clinical characteristics and outcomes of patients with CD after RT, in endocrine remission based on normal LNSC (group 1) or 24 h urinary free cortisol (UFC; group 2).

Design and Setting

Retrospective cohort, observational study in academic medical centre. Patients (16−86 years old, n = 75) with CD who underwent RT were studied; group 1 (n = 16), group 2 (n = 18), and 41 patients not in remission. Outcome measures included within-group and between-group changes (group 1 vs. group 2) in clinical characteristics, endocrine data and time to remission.

Results

Seventy-five patients with CD, aged (median [range]: 50 years [16, 86], 71% female, BMI: 34.7 kg/m2 [19.1, 62.5], 63% with macroadenomas) underwent RT and 34 (45.3%) entered endocrine remission, including 16 (21.3%) in group 1 and 18 (24%) in group 2 (median: 56 months). From RT to remission, there were decreases in BMI (group 1: −3.9 ± 0.7 kg/m2, p = 0.0001; group 2: −5.2 ± 1.9 kg/m2, p = 0.0123) and systolic blood pressure (group 1: −7.9 ± 3.9 mmHg, p = 0.03; group 2: −10.1 ± 4.5 mmHg (p = 0.008). There were no between-group differences in BMI, blood pressure, HbA1c, number of antihypertensive or antihyperglycemic agents, UFC, median time to remission, recurrence risk.

Conclusion

In patients with CD following RT, LNSC appears to be clinically equivalent to UFC in the assessment of endocrine remission. These patients can be followed with LNSC, which is easier for patients to collect.

Conflicts of Interest

Helen A Shih has no conflict of interest related to this study. Other financial disclosures include: UpToDate (section editor, writer), MedLink Neurology (writer), AbbVie (grant support to institution), Advanced Accelerator Applications (advisory board), Servier Pharmaceuticals (consultant, advisory board, educational material development). Beverly M. K. Biller has served as an occasional consultant to Recordati and Xeris. The other authors declare no conflicts of interest.

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