Resolution of the physical features of Cushing’s syndrome in a patient with a cortisol secreting adrenocortical adenoma after unilateral adrenalectomy

Posted on May 1, 2016 by MaryO

A 37-year-old woman developed clinical manifestations of Cushing’s syndrome over a span of 2 years. Physical examination revealed features that best describe Cushing’s syndrome, such as wide purple striae (>1 cm) over the abdomen, facial plethora and easy bruisability.1  Other features observed were hypertension, moon facies, acne, a dorsocervical fat pad, central obesity and dyslipidaemia.

The diagnosis of hypercortisolism was confirmed using a 1 mg overnight dexamethasone suppression test (19.7 ng/dL, N: <1.8) and 24 h urine free cortisol (185.9 μg/24 h, N: 3.5–45). A suppressed adrenocorticotropic hormone (ACTH) level (4 pg/mL, N: 5–20) and a lack of hyperpigmentation suggested ACTH-independent Cushing’s syndrome. Further work up using CT with contrast of the adrenals showed a 2.4×2.3×2.4 cm right adrenal mass. The patient then underwent laparoscopic adrenalectomy of the right adrenal gland. Steroids was started postoperatively and tapered over time. Histopathology results were consistent with an adrenocortical adenoma (2.5 cm widest dimension). Six months after surgery, there was resolution of the physical features, weight loss and improvement in blood pressure.

Figure 1 is a serial photograph of the physical features seen in Cushing’s syndrome, such as moon facies, a dorsocervical fat pad and wide purple striae, taken preoperatively, and at 3 and 6 months after surgery. With treatment, physical and biochemical changes of Cushing’s syndrome both resolve through time.2 The time course of the resolution of these changes, however, is varied.2 ,3 We observed that the physical features were ameliorated at 3 months and resolved at 6 months.

Learning points

  • Physicians as well as patients should be aware that improvement of the features of Cushing’s syndrome after treatment does not occur immediately.

  • Dramatic resolution of the physical features of Cushing’s syndrome, however, can be observed as early as 6 months after surgery.

Figure 1

View larger version:

Figure 1

Physical features of Cushing’s syndrome (top to bottom: moon facies, a dorsocervical fat pad and wide purple striae (>1 cm) over the abdomen) documented before surgery, and at 3 and 6 months after surgery.

Footnotes

  • Twitter Follow John Paul Quisumbing at @jpquisumbingmd

  • Contributors JPMQ worked up the case and wrote the case report. MASS reviewed the case report and critically appraised it. JPMQ incorporated his suggestions.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

References

  1. ↵
    1. Ross EJ,
    2. Linch DC

    . Cushing’s syndrome-killing disease: discriminatory value of signs and symptoms aiding early diagnosis.Lancet 1982;2:646–9. doi:10.1016/S0140-6736(82)92749-0

    [CrossRef][Medline][Web of Science]
  2. ↵
    1. Sippel RS,
    2. Elaraj DM,
    3. Kebebew E, et al

    . Waiting for change: symptom resolution after adrenalectomy for Cushing’s syndrome. Surgery2008;144:1054–61. doi:10.1016/j.surg.2008.08.024

    [CrossRef][Medline][Web of Science]
  3. ↵
    1. Mishra AK,
    2. Agarwal A,
    3. Gupta S, et al

    . Outcome of adrenalectomy for Cushing’s syndrome: experience from a tertiary care center. World J Surg2007;31:1425–32. doi:10.1007/s00268-007-9067-6

    [CrossRef][Medline]

From http://casereports.bmj.com/content/2016/bcr-2016-215693.short?rss=1

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Cushing’s: Update on signs, symptoms and biochemical screening

Posted on October 11, 2015 by MaryO

10.1530/EJE-15-0464

  1. Lynnette Nieman⇑

+Author Affiliations


  1. L Nieman, RBMB, NIH, Bethesda, 20817-1109, United States
  1. Correspondence: Lynnette Nieman, Email: niemanl@mail.nih.gov

Abstract

Endogenous pathologic hypercortisolism, or Cushing’s syndrome, is associated with poor quality of life, morbidity and increased mortality. Early diagnosis may mitigate against this natural history of the disorder.

The clinical presentation of Cushing’s syndrome varies, in part related to the extent and duration of cortisol excess. When hypercortisolism is severe, its signs and symptoms are unmistakable. However, most of the signs and symptoms of Cushing’s syndrome are common in the general population (e.g. hypertension and weight gain) and not all are present in every patient.

In addition to classical features of glucocorticoid excess, such as proximal muscle weakness and wide purple striae, patients may present with the associated co-morbidities that are caused by hypercortisolism. These include cardiovascular disease, thromboembolic disease, psychiatric and cognitive deficits, and infections. As a result, internists and generalists must consider Cushing’s syndrome as a cause, and endocrinologists should search for and treat these co-morbidities.

Recommended tests to screen for Cushing’s syndrome include 1 mg dexamethasone suppression, urine free cortisol and late night salivary cortisol. These may be slightly elevated in patients with physiologic hypercortisolism, which should be excluded, along with exogenous glucocorticoid use. Each screening test has caveats and the choice of tests should be individualized based on each patient’s characteristics and lifestyle.

The objective of this review was to update the readership on the clinical and biochemical features of Cushing’s syndrome that are useful when evaluating patients for this diagnosis.

Read the entire manuscript at http://www.eje-online.org/content/early/2015/07/08/EJE-15-0464.full.pdf+html

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Could you Shed Some Light on Cushing’s Disease?

Posted on April 14, 2014 by MaryO

Dear Dr. Roach: Could you shed some light on Cushing’s disease? Four people in the same family have it. The doctors say it has something to do with the thyroid gland.

— Anon.

A: Cushing’s syndrome, which is different from Cushing’s disease, is an excess of cortisone or similar corticosteroids. It can be caused by taking too much steroid for too long, usually as treatment for a serious medical condition. Cushing’s disease is a special case of Cushing’s syndrome, when the excess cortisone is caused by a tumor in the pituitary gland, which spurs the adrenal gland to make excess amounts of hormone. Weight gain, almost exclusively in the abdomen, a striking round “moon” face, a fat pad on the back of the neck and upper back (“buffalo hump”), diabetes, pigmented stretch marks and high blood pressure are common findings in any form of Cushing’s syndrome.

It is very unusual for Cushing’s disease to run in families. Also, it does not affect the thyroid, although thyroid conditions can sometimes mimic Cushing’s (and vice versa). I suspect that what this might be is a rare condition called multiple endocrine neoplasia type I (MEN-1). This does run in families, and combines risk for pituitary, parathyroid and pancreatic islet cell tumors. (The parathyroid glands sit on top of the thyroid gland and secrete parathyroid hormone, responsible for calcium metabolism. The pancreatic islet cells are where insulin is made.) Not everybody with MEN-1 will have tumors in all of these glands. Parathyroid tumors are the most common.

An endocrinologist is the expert in Cushing’s and the MEN syndromes.

​Dr. Keith Roach writes for North America Syndicate. Send letters to Box 536475, Orlando, FL 32853-6475 or email ToYourGoodHealth@med.cornell.edu.

From http://herald-review.com/news/opinion/editorial/columnists/roach/dr-keith-roach-teeth-grinding-is-common-in-the-elderly/article_bef63ba4-9b5e-5bff-b66a-3530be158857.html

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When to think Cushing’s syndrome in type 2 diabetes

Posted on July 28, 2013 by MaryO

ESTES PARK, COLO. – Diabetes mellitus, osteoporosis, and hypertension are conditions that should boost the index of suspicion that a patient with some cushingoid features may in fact have endogenous Cushing’s syndrome, Dr. Michael T. McDermott said at a conference on internal medicine sponsored by the University of Colorado.

An estimated 1 in 20 patients with type 2 diabetes has endogenous Cushing’s syndrome. The prevalence of this form of hypercortisolism is even greater – estimated at up to 11% – among individuals with osteoporosis. In hypertensive patients, the figure is 1%. And among patients with an incidentally detected adrenal mass, it’s 6%-9%, according to Dr. McDermott, professor of medicine and director of endocrinology and diabetes at the University of Colorado.

“Endogenous Cushing’s syndrome is not rare. I suspect I’ve seen more cases than I’ve diagnosed,” he observed. “I’ve probably missed a lot because I failed to screen people, not recognizing that they had cushingoid features. Not everyone looks classic.”

There are three screening tests for endogenous Cushing’s syndrome that all primary care physicians ought to be familiar with: the 24-hour urine cortisol test, the bedtime salivary cortisol test, and the overnight 1-mg dexamethasone suppression test.

“I think if you have moderate or mild suspicion, you should use one of these tests. If you have more than moderate suspicion – if a patient really looks like he or she has Cushing’s syndrome – then I would use at least two screening tests to rule out endogenous Cushing’s syndrome,” the endocrinologist continued.

The patient performs the bedtime salivary cortisol test at home, obtaining samples two nights in a row and mailing them to an outside laboratory. The overnight dexamethasone suppression test entails taking 1 mg of dexamethasone at bedtime, then measuring serum cortisol the next morning. A value greater than 1.8 mcg/dL is a positive result.

Pregnant women constitute a special population for whom the screening method recommended in Endocrine Society clinical practice guidelines (J. Clin. Endocrinol. Metab. 2008;93:1526-40) is the 24-hour urine cortisol test. That’s because pregnancy is a state featuring high levels of cortisol-binding globulins, which invalidates the other tests. In patients with renal failure, the recommended screening test is the 1-mg dexamethasone suppression test. In patients on antiepileptic drugs, the 24-hour urine cortisol or bedtime salivary cortisol test is advised, because antiseizure medications enhance the metabolism of dexamethasone.

Dr. McDermott said that “by far” the most discriminatory clinical features of endogenous Cushing’s syndrome are easy bruising, violaceous striae on the trunk, facial plethora, and proximal muscle weakness.

“They’re by no means specific. You’ll see these features in people who don’t have Cushing’s syndrome. But those are the four things that should make you really consider Cushing’s syndrome in your differential diagnosis,” he stressed.

More widely recognized yet actually less discriminatory clinical features include facial fullness and the “buffalo hump,” supraclavicular fullness, central obesity, hirsutism, reduced libido, edema, and thin or poorly healing skin.

Endogenous Cushing’s syndrome can have three causes. An adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma accounts for 80% of cases. A cortisol-secreting adrenal tumor is the cause of 10%. And another 10% are due to an ectopic ACTH-secreting tumor, most commonly a bronchial carcinoid tumor.

Once the primary care physician has a positive screening test in hand, it’s typical to refer the affected patient to an endocrinologist in order to differentiate which of the three causes is present. This is accomplished based upon the results of a large, 8-mg dexamethasone suppression test coupled with measurement of plasma ACTH levels.

Dr. McDermott recommended as a good read on the topic of evaluating a patient with endogenous Cushing’s syndrome a recent review article that included a useful algorithm (N. Engl. J. Med. 2013;368:2126-36).

He reported having no financial conflicts.

bjancin@frontlinemedcom.com

From http://www.clinicalendocrinologynews.com

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