Laparoscopic Bilateral Transperitoneal Adrenalectomy For Cushing Syndrome

Surgical Laparoscopy, Endoscopy & Percutaneous Techniques, 07/16/2013  Clinical Article

Aggarwal S et al. –

Laparoscopic adrenalectomy is well established for treatment of adrenal lesions. However, bilateral adrenalectomy for Cushing syndrome is a challenging and time–consuming operation.

The authors report their experience of laparoscopic bilateral adrenalectomy for this disease in 19 patients. Laparoscopic bilateral adrenalectomy for Cushing syndrome is feasible and safe. It confers all the advantages of minimally invasive approach such as less postoperative pain, shorter hospitalization, lesser wound complications, and faster recovery.

The advantages of the laparoscopic approach have led to an earlier referral for bilateral adrenalectomy by endocrinologist in patients with failed pituitary surgery.

 

This article is available on PubMed

Cushing’s Disease – Rare Disease Quick Facts

cushings-diagnosis

 

 

Cushing’s disease is a rare condition due to excess cortisol levels that result from a pituitary tumor secreting adrenocorticotropic hormone (ACTH), which stimulates cortisol secretion.  Cushing’s disease should not be confused with Cushing’s syndrome which is increased cortisol levels but that increase can be due to any number of factors. However, Cushing’s disease is the most common form of Cushing’s syndrome.

Symptoms

The symptoms related to Cushing’s disease and Cushing’s syndrome are the same, since both are related to an excess of cortisol. Also, symptoms vary extensively among patients and that, with the inherent fluctuation in hormone levels make it difficult to diagnosis both conditions.

Changes in physical characteristics of the body

  • Fullness and rounding of the face
  • Added fat on back of neck (so-called “buffalo hump”)
  • Easy bruising
  • Purplish stretch marks on the abdomen (abdominal striae)
  • Excessive weight gain, especially in abdominal region
  • Red cheeks
  • Excess hair growth on the face, neck, chest, abdomen and thighs

Changes in physiology/psychology

  • Generalized weakness and fatigue
  • Menstrual disorder
  • Decreased fertility and/or sex drive
  • High blood pressure that is often difficult to treat
  • Diabetes mellitus
  • Mood and behavior disorders

Diagnosis

The early stages of Cushing’s disease may be difficult to recognize. However, if it is suspected, diagnosis is generally a 2 stage process. First to determine if cortisol levels are high, and if so, why they are high.

Tests to confirm high cortisol levels:

  • 24-hour urine cortisol
  • Dexamethasone suppression test (low dose)

Tests to determine cause:

  • Blood ACTH level
  • Brain MRI
  • Corticotropin-releasing hormone test
  • Dexamethasone suppression test (high dose)
  • Petrosal sinus sampling

Treatment

Surgery

  • Most patients with Cushing’s disease undergo surgery to remove the pituitary adenoma offers.
  • If the tumor is isolated to the pituitary, cure rates of 80-85% are common.
  • If the tumor has spread to nearby organs, cure rates of 50-55% are common.

Medicine (approved orphan drugs)

Signifor (pasireotide)

  • Approved for patients with Cushing’s disease for whom pituitary is not an option or surgery has been ineffective.
  • Signifor is a somatostatin receptor agonist that leads to inhibition of ACTY secretion (and subsequently decreased cortisol levels).

Korlym (mifepristone)

  • Approved for patients with Cushing’s syndrome who have type 2 diabetes or glucose intolerance and have failed surgery (or not candidates for surgery).
  • Korlym is a glucocorticoid receptor antagonist which in turn blocks the effects of the high levels of cortisol in the body. Korlym is used to treat high glucose levels due to elevated cortisol.

Medicines used but not indicated for Cushing’s disease include

Mitoden

ketoconazole

Metyrapone

Etomidate

Radiation

  • Radiation therapy may be used in some patients and can be very effective in controlling the growth of these tumors.

Prognosis

In most cases, treatment can cure Cushing’s disease. If not treated properly, the chronic hypercortisolism can lead to excess morbidity and mortality due to increased cardiovascular and other risk factors.

For more information

National Library of Medicine, National Institute of Health

Cushing’s Disease Information (provided by Novartis Pharmaceuticals)

 

Images courtesy of the open access journal Orhanet Journal for Rare Diseases.  Castinetti et al. Orphanet J Rare Dis. 2012 7:41   doi:10.1186/1750-1172-7-41

– See more at: http://www.raredr.com/front-page-medicine/articles/cushings-disease-rare-disease-quick-facts-0

Cushing Disease: A Multidisciplinary Treatment Update

Share this info with your endo in case he/she missed it!

This activity is intended for endocrinologists, primary care physicians, nurses, nurse practitioners, and pharmacists.

The goal of this activity is to review the diagnosis and treatment of Cushing disease from a multidisciplinary perspective.

Upon completion of this activity, participants will be able to:

  1. Outline the rationale for a multidisciplinary approach to the diagnosis and treatment of patients with Cushing disease
  2. Review the safety and efficacy of current management strategies for patients with Cushing disease
  3. Describe the diagnostic workup for Cushing disease and the reasons why timely diagnosis and treatment are important

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Laurence Katznelson, MD

Professor of Medicine and Neurosurgery, Stanford University; Medical Director, Pituitary Center, Stanford Hospital and Clinics, Stanford, California

Disclosure: Laurence Katznelson, MD, has disclosed the following relevant financial relationships:
Received grants for clinical research from: Corcept Therapeutics Inc.; Novartis Pharmaceuticals Corporation

Dr Katznelson does not intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Katznelson does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Brooke Swearingen, MD

Associate Professor of Neurosurgery, Harvard Medical School; Associate Visiting Neurosurgeon, Massachusetts General Hospital, Boston, Massachusetts

Disclosure: Brooke Swearingen, MD, has disclosed the following relevant financial relationships: Served as an advisor or consultant for: Novartis Pharmaceuticals Corporation
Owns stock, stock options or bonds from: Novartis Pharmaceuticals Corporation; Pfizer Inc; Amgen Inc; Roche

Dr Swearingen does not intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Swearingen does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Nicholas Tritos, MD

Assistant Professor of Medicine, Harvard Medical School; Staff, Neuroendocrine Unit, Massachusetts General Hospital, Boston, Massachusetts

Disclosure: Nicholas Tritos, MD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Corcept Therapeutics Inc; Pfizer Inc
Received grants for clinical research from: Pfizer Inc; Ipsen

Dr Tritos does intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Tritos does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Susan Cornell, PharmD, CDE

Associate Professor, Pharmacy Practice, Midwestern University-Chicago, Downers Grove, Illinois; Clinical Pharmacist/Certified Diabetes Educator, DuPage Community Clinic, Wheaton, Illinois

Disclosure: Susan Cornell, PharmD, CDE, has disclosed the following relevant relationships:
Served as a speaker or member of a speakers bureau for: Johnson & Johnson Diabetes Institute

Dr Cornell does intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Cornell does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Rita Pach, RN, MSN

Nurse, Johns Hopkins Pituitary Center, Baltimore, Maryland

Participation by Mrs Pach in the development of this product does not constitute or imply endorsement by the Johns Hopkins University or the Johns Hopkins Hospital and Health System.
Disclosure: Rita Pach, RN, has disclosed no relevant financial relationships.

Mrs Pach does not intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Mrs Pach does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Kristin M. Richardson

Group Scientific Director, Medscape, LLC

Disclosure: Kristin M. Richardson has disclosed no relevant financial relationships.

David Modrak, PhD

Freelance editor, Montville, New Jersey

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Nafeez Zawahir, MD

CME Clinical Director, Medscape, LLC

Disclosure: Nafeez Zawahir, MD, has disclosed no relevant financial relationships.

Laurie E. Scudder, DNP, NP

Nurse Planner, Continuing Professional Education Department, Medscape, LLC; Clinical Assistant Professor, School of Nursing and Allied Health, George Washington University, Washington, DC

Disclosure: Laurie E. Scudder, DNP, NP, has disclosed no relevant financial relationships.

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Continue to activity: http://www.medscape.org/viewarticle/806559

Pasireotide for the treatment of Cushing’s disease

Posted online on June 17, 2013. (doi:10.1517/21678707.2013.807731)

Annamaria Colao Chiara Simeoli Monica De Leo Alessia Cozzolino Rosario Pivonello

Department of Clinical Medicine and Surgery, Section of Endocrinology, Federico II University, Via Sergio Pansini 5,

80131 Naples

, Italy +39 0817462132; +39 0815465443; colao@unina.it

Author for correspondence

Introduction: Pasireotide, a novel multireceptor targeted somatostatin analog is the first drug approved for treatment of adult patients with Cushing’s disease (CD) for whom pituitary surgery is not an option or has not been curative.

Areas covered: The review describes published data on efficacy and safety of pasireotide in CD patients. In particular, the review focuses on a Phase III study (CSOM230B2305) evaluating the outcomes of treatment with pasireotide at the doses of 600 and 900 µg twice daily for 12 months in 162 CD patients. This clinical trial reported a decrease in urinary free cortisol levels in the majority of patients, with a substantial reduction in nearly half and a normalization in > 25% of patients included in the study, accompanied by an improvement in clinical picture as well as a significant reduction in pituitary tumor size. Hyperglycemia appears as the most important side effect, requiring a careful monitoring and a prompt administration of glucose-lowering medications.

Expert opinion: Pasireotide seems to have a promising role as medical option for CD patients who experienced a failure or not candidate for neurosurgery; its employment will probably induce in the near future significant changes in the therapeutic approach to CD.

Read More: http://informahealthcare.com/doi/abs/10.1517/21678707.2013.807731