CushieBlog

Investigating the Frequency of Active Adrenal Incidentalomas

Posted on October 7, 2023 by MaryO

Adrenal incidentalomas (AI) are associated with an increased risk of cardiometabolic complications due to adrenal hyperfunction. Obtaining accurate prevalence estimates of distinct types of functioning AIs is crucial for efficient resource allocation and effective management strategies. For a study, researchers sought to ascertain the prevalence of various forms of autonomous hormone secretion in individuals diagnosed with adrenal incidentaloma, including autonomous/possible autonomous cortisol secretion (ACS), primary aldosteronism (PA), pheochromocytoma (PHEO), and Cushing syndrome (CS).

A comprehensive and systematic search was conducted across multiple databases (PubMed, Ovid MEDLINE, Web of Science) up to February 2022. Among the 1,661 publications initially screened at the title and abstract levels, 161 articles underwent full-text examination, and ultimately, 36 studies were included for analysis. Three independent reviewers meticulously extracted clinical data from these selected studies.

The overarching prevalence of functioning adrenal incidentalomas was 27.5% (95% CI 23.0, 32.5). The highest prevalence was observed for ACS/possible ACS, with a rate of 11.7% (95% CI 8.6, 15.7), followed by PA at 4.4% (95% CI 3.1, 6.2). Subgroup analysis unveiled a greater prevalence of PA in patients from Asian regions than those from Europe/America. Conversely, the prevalence of ACS/possible ACS was comparatively lower in Asian countries. Meta-regression analysis elucidated that the proportion of female patients influenced the prevalence of ACS/possible ACS, while PA prevalence positively correlated with the proportion of patients with hypertension and the publication year. PHEO and CS demonstrated prevalences of 3.8% (95% CI 2.8, 5.0) and 3.1% (95% CI 2.3, 4.3), respectively.

The comprehensive meta-analysis offered valuable insights into the prevalence rates of diverse types of functioning adrenal incidentalomas and identified influential factors contributing to heterogeneity in these estimates. The findings contributed significantly to understanding clinical implications and aided in devising effective management strategies for individuals diagnosed with these adrenal disorders.

Source: academic.oup.com/jcem/article-abstract/108/7/1813/7015785?redirectedFrom=fulltext

Filed under: Addison's disease, adrenal, Cushing's, Rare Diseases | Tagged: adrenal Cushing's, adrenal incidentaloma, cortisol, pheochromocytoma, primary aldosteronism | Leave a comment »

Pituitary Surgery Outcome in Patients 75 Years and Older

Posted on October 5, 2023 by MaryO

Abstract

Background

As the population ages, the number of elderly patients with an indication for pituitary surgery is rising. Information on the outcome of patients aged over 75 is limited. This study reports a large series assessing the feasibility of surgical resection in this specific age range, focusing on surgical complications and postoperative results.

Methods

A retrospective cohort study of patients with pituitary adenomas and Rathke’s cleft cysts was conducted. All patients were aged 75 years or over and treated by a single expert neurosurgical team. A control population included 2379 younger adult patients operated by the same surgeons during the same period.

Results

Between 2008 and 2022, 155 patients underwent surgery. Indication was based on vision impairment in most patients (79%). Median follow-up was 13 months (range: 3–96). The first surgery was performed with an endoscopic transsellar approach, an extended endonasal transtuberculum approach and a microscopic transcranial approach in 96%, 3%, and 1% of patients, respectively. Single surgery was sufficient to obtain volume control in 97% of patients. From Kaplan-Meier estimates, 2-year and 5-year disease control with a single surgery were 97.3% and 86.2%, respectively. Resection higher than 80% was achieved in 77% of patients. No vision worsening occurred. In acromegaly and Cushing’s disease, endocrine remission was obtained in 90% of non-invasive adenomas. Surgical complications were noted in 5% of patients, with 30-day mortality, hematoma, cerebrospinal fluid leak, meningitis, and epistaxis occurring in 0.6%, 0.6%, 1.9%, 0.6%, and 1.3% respectively. New endocrine anterior deficits occurred in only 5%, while no persistent diabetes insipidus was noted. Compared with younger patients, the complication rate was not statistically different.

Conclusions

Surgery beyond the age of 75, mainly relying on an endoscopic endonasal transsellar approach, is effective and safe, provided that patients are managed in tertiary centers.

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Abbreviations

CSF:: Cerebrospinal fluid
ASA:: American Society of Anesthesiologists Physical Status Classification System

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Author information

Authors and Affiliations

Department of Neurosurgery, La Pitié-Salpêtrière University Hospital, Assistance Publique-Hôpitaux de Paris, 47-83 Boulevard de L’Hôpital, 75013, Paris, France
Marta Garvayo, Vincent Reina, Stephan Gaillard & Bertrand Baussart
Department of Neurosurgery, University Hospital of Lausanne and University of Lausanne, Lausanne, Switzerland
Marta Garvayo & Mahmoud Messerer
Université Paris Cité, CNRS, INSERM, Institut Cochin, 75014, Paris, France
Chiara Villa, Anne Jouinot, Jérôme Bertherat, Guillaume Assié & Bertrand Baussart
Department of Neuropathology, La Pitié-Salpêtière University Hospital, AP-HP, Sorbonne University, Paris, France
Chiara Villa
Department of Endocrinology, Assistance Publique-Hôpitaux de Paris, Hôpital Ambroise Paré, Boulogne Billancourt, France
Mirella Hage & Marie-Laure Raffin-Sanson
Université de Versailles Saint-Quentin-en-Yvelines UFR Des Sciences de La Santé Simone Veil, Montigny-Le-Bretonneux, France
Mirella Hage & Marie-Laure Raffin-Sanson
Department of Endocrinology and Reproductive Medicine, Centre de Référence Des Maladies Endocriniennes Rares de La Croissance Et du Développement, CRMERC, Endo-ERN, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne University, Paris, France
Carine Courtillot & Anne Bachelot
Université Paris-Saclay, Inserm, Physiologie Et Physiopathologie Endocriniennes, Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Service d’Endocrinologie Et Des Maladies de La Reproduction, Centre de Référence des Maladies Rares de L’Hypophyse, Le Kremlin-Bicêtre, France
Peter Kamenicky & Philippe Chanson
Sorbonne University, Endocrine Unit, Reproductive Medicine, Centre de Référence Des Maladies Endocriniennes Rares de La Croissance Et du Développement (CRMERC), Endo-ERN (Id 739527), Saint-Antoine Hospital, AP-HP, Paris, France
Camille Vatier & Sophie Christin-Maitre
Inserm UMRS938, Saint-Antoine Research Center, Sorbonne University, 75012, Paris, France
Camille Vatier
INSERM UMR-833, Trousseau Hospital, Paris, France
Sophie Christin-Maitre
Department of Endocrinology, Center of Rare Adrenal Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France
Jérôme Bertherat & Guillaume Assié

Corresponding author

Correspondence to Bertrand Baussart.

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Filed under: Cushing's, pituitary, Treatments | Tagged: Pituitary adenoma, Rathke’s cleft cyst, Transsphenoidal surgery | Leave a comment »

Cushing’s Syndrome in Pregnancy in Which Laparoscopic Adrenalectomy was Safely Performed by a Retroperitoneal Approach

Posted on October 2, 2023 by MaryO

Abstract

Introduction

Laparoscopic adrenalectomy is the standard treatment for adrenal tumors caused by Cushing’s syndrome. However, few pregnant women have undergone adrenalectomy because of the risk of general anesthesia and surgery.

Case presentation

A 28-year-old woman presented with gradually worsening Cushing’s signs at around 12 weeks of pregnancy. Magnetic resonance imaging displayed a 38-mm left adrenal tumor, which was the cause of the adrenal Cushing’s syndrome. Metyrapone was started, which increased androgen levels. Since the management of Cushing’s syndrome by medication alone is challenging, unilateral laparoscopic adrenalectomy by a retroperitoneal approach was performed at 23 weeks of the pregnancy. No perioperative complications were noted.

Conclusion

Adrenalectomy is considered safe in pregnant women with Cushing’s syndrome. Laparoscopic adrenalectomy by retroperitoneal approach should be chosen and performed between 14 and 30 weeks of pregnancy to prevent mother and fetal complications.

Abbreviations & Acronyms

CS

Cushing’s syndrome

MRI

magnetic resonance imaging

Keynote message

We report a rare case of adrenalectomy performed via a retroperitoneal approach for Cushing’s syndrome in a pregnant woman. Cushing’s syndrome may affect the fetus, and surgery can be considered in addition to medical management. Adrenalectomy should be performed in the second trimester of pregnancy. Pneumoperitoneal pressure, position, and surgical approaches must receive careful attention.

Introduction

CS is characterized by excessive cortisol secretion and characteristic symptoms such as full moon-like facial features and central obesity. Premenopausal women with CS rarely become pregnant because excessive glucocorticoid secretion inhibits the synthesis of gonadotropins, leading to impaired ovarian and endometrial function, and causing amenorrhea or oligomenorrhea.¹ Furthermore, even when women with CS become pregnant, the incidence of severe complications is high. CS can cause maternal hypertension, diabetes/glucose intolerance, osteopenia/osteoporosis, preeclampsia, pulmonary edema, heart failure, opportunistic infections, and even death. Additionally, CS can potentially cause stillbirth, prematurity, and intrauterine fetal growth restriction.^1–6 Therefore, CS must be detected at an early stage in pregnancy; however, CS may go undetected because of the overlapping signs of preeclampsia and/or gestational diabetes.

A cortisol-secreting adrenal tumor is the underlying cause of CS, and laparoscopic adrenalectomy is the standard treatment to it. Medical treatment of CS can include medications that inhibit 11β-hydroxylase, such as metyrapone and osilodrostat, but surgical treatment is considered if the disease is difficult to control with medical treatment. Nonobstetric surgery during pregnancy is performed in 1%–2% of pregnant women.⁷ Although general anesthesia is relatively safe during pregnancy, the indication for the surgery must be carefully considered because of potential risks such as neurodevelopmental delay, sudden death, etc.

Herein, we present a case of a pregnant woman diagnosed with CS who underwent unilateral laparoscopic adrenalectomy by a retroperitoneal approach without any problems.

Case presentation

The patient was a 28-year-old primiparous woman. Since around 12 weeks of pregnancy, she has experienced facial and lower limb edema; gained 6-kg weight in 1 month; increased facial acne; and experienced subcutaneous bleeding on the forearms, red abdominal dermatitis, proximal muscle weakness, palpitations, insomnia, and decreased vision in eyes. Her symptoms gradually worsened from 14 weeks, and she was referred to our hospital to clarify the cause at 18 weeks of pregnancy.

Adrenal CS was suspected on the basis of her Cushing’s signs, cortisol 25 μg/dL, and adrenocorticotropic hormone <1.5 pg/mL. She had hypokalemia, hypogammaglobulinemia, and liver dysfunction, and her condition was rapidly worsening. Given her pregnant state, she was admitted for intensive testing for the case of CS from 19 weeks of pregnancy. MRI revealed a well-defined 38-mm left adrenal tumor, which was the cause of the adrenal CS (Fig. 1). She was started on metyrapone with 250 mg per day, which increased androgens (0.53–0.69 ng/mL in 1 week). We considered that the management of CS by medication alone would be challenging and performed adrenalectomy during her pregnancy. The dose of metyrapone was increased to 1000 mg per day eventually.

Fig. 1

Open in figure viewer PowerPoint

Magnetic resonance imaging on admission shows a left adrenal tumor with a long axis of 38 mm (arrowhead). Signal reduction was partially observed on opposed-phase images, leading to diagnosis of cortical adenoma.

She was admitted to the hospital at 23 weeks and 2 days of gestation, and laparoscopic left adrenalectomy was performed via a retroperitoneal approach in the right lateral and jackknife position on the following day (Fig. S1). During the surgery, blood pressure was carefully controlled by an anesthesiologist and the patient’s position and fetal heart rate were monitored by an obstetrician. The operation time, insufflation time, and general anesthesia time were 68, 59, and 123 min, respectively, and the blood loss volume was 75 mL, without any complications. Pathological findings revealed an adrenocortical adenoma. The specimen was positive for one of the nine Weiss criteria (Fig. 2).

Fig. 2

Open in figure viewer PowerPoint

(a) Intraoperative findings of the retroperitoneal approach. Arrowheads indicate the tumor. (b) Gross appearance of the resected adrenal tumor; a brownish-toned, substantial mass, 60 × 34 × 15 mm in size. (c, d) Hematoxylin–eosin staining showed that nodular lesion with a fibrous capsule, with foci of homogeneous cells with eosinophilic or pale, foamy sporangia and small round nuclei.

Postoperatively, metyrapone was discontinued and both lower leg edema, facial acne, fatigue, and muscle weakness improved. Metyrapone was discontinued after surgery. Hydrocortisone, which had been administered at 150 mg/day during the perioperative period, was reduced every few weeks and was taken at 30 mg/day at delivery. She delivered by cesarean section at 38 weeks and 2 days of gestation, with good outcomes for the mother and her infant. Hydrocortisone was discontinued 15 weeks after delivery.

We showed the changes in cortisol and ACTH from the first visit to postpartum (Fig. 3).

Fig. 3

Open in figure viewer PowerPoint

The transition of Cortisol and ACTH. Cortisol decreases rapidly after surgery and rises again before delivery. As cortisol improved, ACTH also increased.

Discussion

CS seldom occurs during pregnancy. Symptoms such as weight gain, skin striae, fatigue, and a round face can also occur in normal pregnancies. The dexamethasone suppression test can result in false positives because of ACTH produced by placenta in normal pregnancy. During pregnancy, there is a physiological state of high cortisol levels. The disappearance of diurnal rhythm is a useful indicator for diagnosis of CS in pregnancy because circadian rhythm is maintained in normal pregnancy. Useful diagnostic criteria include urine cortisol levels greater than three times the upper limit of normal, loss of diurnal cortisol rhythm, and presence of adrenal tumors on MRI.

The pharmacologic treatment of endogenous cortisol is complex, and hormonal management is challenging. While the management of the cortisol levels is important, metyrapone is a risk factor for gestational hypertension and may inhibit fetal cortisol production by crossing the placenta.^{1–6, 8–12}

In this case, because androgens were also elevated and drug management was expected to be challenging, the surgery was aggressively considered. Despite the reports of successful adrenalectomy is after 28 weeks of gestation,^6, 13, 14 The surgery should be performed by an experienced team between 14 and 30 weeks of pregnancy, that is, after organogenesis phase and before the fetus grows too large.^1, 13, 15

A few pregnant women with adrenal CS undergo adrenalectomy. However, the laparoscopic approach is safe, and maternal and fetal complications were higher in women who did not undergo surgery.¹⁶ Less postoperative pain, faster wound healing, and faster postoperative recovery are the main advantages of laparoscopic surgery.¹⁷

In pregnant women, pneumoperitoneal pressure should be kept <12 mmHg because increased intraabdominal pressure decreases placental blood flow and can cause fetal acidosis due to the absorption of carbon dioxide used for insufflation.

Laparoscopic adrenalectomy can be safely performed through both transperitoneal and retroperitoneal approaches.¹⁸ However, in pregnant women, performing the surgery by the retroperitoneal approach in the lateral position is preferable to prevent putting pressure on the fetus during the surgery. The retroperitoneal approach is advantageous, as less pressure is placed on the uterus and adhesions are prevented. After taking the lateral position, the obstetrician is advised to check the position and confirm that the abdomen is not compressed and that the fetal heart rate is normal.

Conclusions

We present a case of a pregnant woman diagnosed with adrenal CS who underwent a unilateral laparoscopic adrenalectomy by a retroperitoneal approach without any problems. Adrenalectomy is a useful treatment when CS is difficult to control despite metyrapone and other medical support.

Author contributions

Nobuyoshi Takeuchi: Conceptualization; methodology; project administration; writing – original draft. Yusuke Imamura: Conceptualization; methodology; supervision; writing – review and editing. Kazuki Ishiwata: Data curation; supervision. Manato Kanesaka: Data curation; supervision. Yusuke Goto: Data curation; supervision. Tomokazu Sazuka: Data curation; supervision. Sawako Suzuki: Data curation; supervision. Hisashi Koide: Data curation; supervision. Shinichi Sakamoto: Data curation; supervision. Tomohiko Ichikawa: Data curation; supervision.

Conflict of interest

The authors declare no conflicts of interest.

Approval of the research protocol by an Institutional Reviewer Board

Not applicable.

Informed consent

Informed consent for the release of the case report and accompanying images has been obtained from the patient.

Registry and the Registration No. of the study/trial

Not applicable.

From https://onlinelibrary.wiley.com/doi/10.1002/iju5.12637

Filed under: adrenal, Cushing's, Treatments | Tagged: adrenal, Cushing's Syndrome, laparoscopic adrenalectomy, Metyrapone, MRI, pregnancy | Leave a comment »

Birthday of the Message Boards

Posted on September 30, 2023 by MaryO

September 30, 2000 – Birth of the Message Boards

Today is the birthday, or anniversary, of the boards starting September 30, 2000 (The rest of the site started earlier that year in July)

As of today, we have 73,357 members who have made well over 380,324 posts.

Find the message boards here: http://cushings.invisionzone.com/

Filed under: adrenal, adrenal crisis, Cancer, Clinical trials, Cushing's, Diagnostic Testing, FAQ, growth hormone, Helpful Doctors, Interview, Meetings and Conferences, pituitary, podcast, Rare Diseases, Thankfulness, Treatments | Tagged: anniversary, birthday, members, message boards | Leave a comment »

The Role of Endocrinology in Managing Polycystic Ovary Syndrome and Diabetes

Posted on September 28, 2023 by MaryO

Introduction to Endocrinology

Endocrinology is a medical specialty that focuses on the diagnosis and treatment of diseases related to hormones. Endocrinologists are experts in managing and treating diseases related to the endocrine system, which includes the thyroid, pituitary, adrenal glands, and pancreas. Endocrinologists are trained to diagnose and treat conditions such as diabetes, thyroid disorders, pituitary disorders, and other conditions related to hormones. Endocrinologists also specialize in reproductive health and fertility issues, including PCOS.

Endocrinology is a complex field that requires a deep understanding of the endocrine system and its role in regulating the body’s hormones. Endocrinologists must be able to interpret laboratory tests and understand the underlying causes of endocrine disorders. They must also be able to develop individualized treatment plans to address the specific needs of each patient.

Diagnosing PCOS and Diabetes

Endocrinologists are experts in diagnosing and managing PCOS and diabetes. PCOS is a hormonal disorder that affects the ovaries, and it is characterized by irregular menstrual cycles, excess facial and body hair, and infertility. To diagnose PCOS, an endocrinologist will perform a physical exam and order laboratory tests to measure hormone levels. The endocrinologist will also ask the patient about her symptoms and family history to determine if PCOS is the cause.

Diabetes is a chronic condition that affects the body’s ability to process sugar. To diagnose diabetes, an endocrinologist will perform a physical exam and order laboratory tests to measure blood sugar levels. The endocrinologist may also order imaging tests to check for signs of diabetes-related complications.

Treating PCOS and Diabetes

Once the endocrinologist has diagnosed PCOS or diabetes, they will develop an individualized treatment plan to address the patient’s specific needs. For PCOS, the endocrinologist may recommend lifestyle changes such as weight loss, exercise, and dietary changes to help manage symptoms. The endocrinologist may also prescribe medications to regulate hormone levels and improve fertility.

For diabetes, the endocrinologist may recommend lifestyle changes such as weight loss, exercise, and dietary changes to help manage blood sugar levels. The endocrinologist may also prescribe medications to help regulate blood sugar levels. In addition, the endocrinologist may recommend regular check-ups to monitor the patient’s progress and to adjust the treatment plan if needed.

Conclusion

Endocrinology plays an important role in managing PCOS and diabetes. Endocrinologists are experts in diagnosing and treating these conditions, and they are trained to develop individualized treatment plans that address the specific needs of each patient. By working with an endocrinologist, patients can get the help they need to manage their PCOS or diabetes and achieve their health goals.

Endocrinology is a complex field that requires a deep understanding of the endocrine system and its role in regulating the body’s hormones. An endocrinologist can help patients with PCOS and diabetes manage their conditions and achieve their health goals. By working with an endocrinologist, patients can get the help they need to manage their PCOS or diabetes and achieve their health goals.

From https://www.diabetesincontrol.com/the-role-of-endocrinology-in-managing-polycystic-ovary-syndrome-and-diabetes/

Filed under: adrenal, General Health, pituitary | Tagged: adrenal, diabetes, endocrinology, pancreas, PCOS, pituitary, thyroid | Leave a comment »

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Pituitary Surgery Outcome in Patients 75 Years and Older

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Cushing’s Syndrome in Pregnancy in Which Laparoscopic Adrenalectomy was Safely Performed by a Retroperitoneal Approach

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Birthday of the Message Boards

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The Role of Endocrinology in Managing Polycystic Ovary Syndrome and Diabetes

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