Rare Malignant Tumor of Adrenal Gland Led to Cushing’s, Girl’s Death

Posted on May 5, 2018 by MaryO

While adrenocortical carcinoma — a malignant tumor of the adrenal gland — appears only rarely in children, the tumor may cause secondary Cushing’s syndrome in these patients, a new case report shows.

Early diagnosis of the causes of Cushing’s syndrome could improve the prognosis of these children, researchers say.

The study, “Cushing Syndrome Revealing an Adrenocortical Carcinoma,” was published in the Open Journal of Pediatrics.

Adrenocortical carcinoma is a malignant tumor that develops in the cortex of the adrenal gland. It usually is identified by increased amounts of hormones that are produced by the adrenal glands, like cortisol.

This tumor type is very rare in children, representing fewer than two in every 1,000 pediatric tumors.

Researchers at the University Hospital Center Souro Sanou, in Burquina Faso (West Africa), described the case of a 10-year-old girl who developed this rare cancer.

The patient’s first symptoms were loss of consciousness and recurrent seizures without fever. The patient also had experienced excessive weight gain in the preceding months. At admission she was in a light state of coma and showed obesity in the face and trunk.

An initial analysis of blood, urine, and cerebrospinal fluid failed to detect any alterations, with no diabetes, kidney damage, or infection identified. And, even though no lesions or alteration were seen in the pituitary gland region, brain swelling was detected.

While in the hospital, the patient’s condition continued to deteriorate. She developed fever and difficulty speaking, while showing persistent seizures.

In the absence of a diagnosis, physicians focused on the safeguard of major vital function, control of seizures, and administration of large-spectrum antibiotics. Her condition improved slightly, regaining consciousness and control of seizures.

One month later, however, the patient developed symptoms that are commonly associated with increased levels of cortisol and male sex hormones, including obesity and early development of pubic hair.

After confirming high cortisol levels, physicians examined the patient’s abdominal region,  which revealed a tumor in the left adrenal gland.

The patient received a ketoconazole treatment and a surgery to remove the tumor was planned. But her condition worsened, with development of malignant hypertension and convulsive illness, which led to her death before the tumor was removed.

“The delay in the diagnosis and the insufficiency of the therapeutic means darken the prognosis in our context,” the researchers wrote.

“[Adrenocortical carcinoma] diagnosis should be considered in presence of virilization and early signs of puberty,” the researchers suggested. “Early diagnosis and multidisciplinary management of adrenocortical carcinoma could improve the prognosis in children.”

From https://cushingsdiseasenews.com/2018/05/04/rare-malignant-tumor-adrenal-gland-caused-cushings-case-report/

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Cushing’s Patient Exhibits Cortisol-Secreting Lesions in Both Adrenal Glands

Posted on May 4, 2018 by MaryO

In rare cases, Cushing’s syndrome may be caused by cortisol-secreting masses in both adrenal glands, a case report shows.

The study with that finding, “ACTH-independent Cushing’s syndrome with bilateral cortisol-secreting adrenal adenomas: a case report and review of literatures” was published in BMC Endocrine Disorders.

Cushing’s syndrome results from the prolonged secretion of excess cortisol. While most cases are caused by tumors in the pituitary gland, up to 20 percent result from tumors in the adrenal glands.

Occasionally, Cushing’s syndrome is caused by masses in both adrenal glands, which may be similar or display different properties. “Determining the nature and function of bilateral adrenal masses is always a challenge in clinical practice,” researchers said.

Now, physicians at Sichuan University in China, reported the case of a 55-year-old woman who complained of difficulty breathing for more than 10 years.

The patient had developed obesity of the trunk and face over the past two years, and had been diagnosed with hypertension 10 years before. She also had high lipid levels for the past five years.

The patient was taking Avapro (irbesartan), Lopressor (metoprolol), Procardia XL (nifedipine), and statins for these disorders. No other health conditions or treatments were reported.

Physical examination showed a moon-shaped face, truncal obesity, and accumulation of fat in the back of the neck and upper back (aka buffalo hump). She also had discoloration of the lower limbs, with slight fluid accumulation and muscle weakness.

Routine blood analysis did not reveal significant changes, but hormone analysis showed high cortisol levels and low adrenocorticotropic hormone (ACTH) was low.

The amount of 24-hour urine-free cortisol was almost five times higher than the upper normal limit. Also, the patients had reduced response to corticosteroids treatment, showing even higher cortisol levels upon treatment with 1 mg dexamethasone. Additional evaluations revealed reduced bone mineral density, indicative of osteoporosis.

Together, the findings led to a diagnosis of ACTH-independent Cushing’s syndrome.

To identify what was causing Cushing’s syndrome, the team looked at the adrenal glands. They detected three lesions, one on the right side measuring 2.5 centimeters, and two on the left side, with 2.3 cm and 0.6 cm respectively. The masses in both sides were actively producing cortisol in similar proportions.

These results confirmed that the patient had Cushing’s syndrome induced by bilateral adrenal excessive cortisol secretion.

Because the patient had poor cardiac function, researchers planned a two-step operation. First, they removed the right adrenal gland laparoscopically, followed by the left adrenal gland two months later. The patient started replacement therapy with hydrocortisone, and her cortisol levels improved significantly, returning to normal levels. She also lost 4 kilograms (8.8 pounds) of body weight in the following year.

“The optimal treatment for patients with bilateral cortisol-secreting adenomas remains uncertain,” the researchers wrote. Although there are no reports of recurrence after surgical treatment, the long-term outcome of these patients remains unclear, and “lifelong follow-up of the patient is required,” they added.

Bilateral cortisol-secreting adrenal adenomas are rare, having been reported in only 15 other studies, the team wrote. Interestingly, some features reported in this study also were identified by other researchers, including the fact that bilateral cortisol-secreting adrenal lesions are more predominantly found in females during adulthood. Also, the size of the lesions commonly range between 1 to 5 centimeters when detected, and appear at approximately the same time in both adrenal glands.

Although there are no treatment guidelines for these cases, surgical removal of the lesions or adrenal glands, plus glucocorticoid replacement therapy, is the mostly used therapeutic approach. Importantly, the researchers noted that patients who underwent partial gland removal were able to withdraw from glucocorticoid replacement therapy during follow-up.

From https://cushingsdiseasenews.com/2018/05/03/cushings-patient-has-cortisol-secreting-lesions-in-both-adrenal-glands/

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Steroid Medication for Nasal Obstruction in Infants May Cause Cushing’s Syndrome

Posted on March 10, 2018 by MaryO

Intranasal steroid drops used to treat nasal obstruction may cause Cushing’s syndrome and adrenal insufficiency in infants, a case study of two patients suggests.

The study, “Iatrogenic Cushing’s syndrome and adrenal insufficiency in infants on intranasal dexamethasone drops for nasal obstruction – Case series and literature review,” was published in the International Journal of Pediatric Otorhinolaryngology.

Children with nasal obstruction may have severe delays in development and can face life-threatening complications later in life such as obstructive sleep apnea and cardiopulmonary problems.

While intranasal steroid drops have become increasingly popular as a substitute for surgery, they can have adverse effects. In addition to suppressing the immune system and changing metabolism, high levels of corticosteroids in the blood may cause Cushing’s syndrome.

Researchers at Weill Cornell Medical College presented two cases of adrenal gland insufficiency and Cushing’s syndrome caused by intranasal dexamethasone drops. Dexamethasone is a type of corticosteroid medication.

First, they described the case of a 3-month-old boy who was taken to the hospital following a life-threatening episode at home after feeding. A physical evaluation revealed nasal congestion with no additional anatomic abnormalities.

Treatment with nasal dexamethasone drops three times a day improved his breathing. While the dosage was later decreased to three drops once daily, a congestion episode led the mother to increase the dose back to the initial recommendation.

After seven weeks of treatment, the boy was noted to have facial puffiness, leading to an endocrine evaluation that revealed low cortisol levels. The dose was eventually reduced, and the boy’s cortisol levels returned to normal after several months.

The second case was a 6-week-old boy with a history of chronic congestion and difficulty feeding. He had severe nasal obstruction and required intubation due to respiratory distress. A nasal exam revealed damaged mucosa with severe nasal cavity narrowing, and he began treatment with three ciprofloxacin-dexamethasone drops three times a day.

After two and a half weeks of treatment, the boy’s cortisol levels were considerably low, and adrenal insufficiency was diagnosed. The treatment dose was reduced in an attempt to improve cortisol levels, but nasal obstruction symptoms continued.

The child then underwent surgery to resolve his nasal obstruction, and the treatment with steroid drops was discontinued. While his cortisol levels subsequently improved, they continued to be low, suggesting that he may have a hormone-related disease.

Despite the benefits of steroid-based nasal drops, small infants are more sensitive to steroid compounds. In addition, nasal drops are more easily absorbed than nasal sprays, suggesting that infants taking these medications should be better controlled for side effects.

“Patients started on this therapy must be closely monitored in a multi-disciplinary fashion to ensure patient safety and optimal symptom resolution,” the researchers suggested.

From https://cushingsdiseasenews.com/2018/03/09/cushing-syndrome-infants-can-be-caused-by-steroid-based-nasal-drops-study-suggests/

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Cushing’s Patients at Risk for Autoimmune Diseases After Condition Is Resolved

Posted on March 8, 2018 by MaryO

Children with Cushing’s syndrome are at risk of developing new autoimmune and related disorders after being cured of the disease, a new study shows.

The study, “Incidence of Autoimmune and Related Disorders After Resolution of Endogenous Cushing Syndrome in Children,” was published in Hormone and Metabolic Research.

Patients with Cushing’s syndrome have excess levels of the hormone cortisol, a corticosteroid that inhibits the effects of the immune system. As a result, these patients are protected from autoimmune and related diseases. But it is not known if the risk rises after their disease is resolved.

To address this, researchers at the Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) examined 127 children with Cushing’s syndrome at the National Institutes of Health from 1997 until 2017.

Among the participants, 77.5 percent had a pituitary tumor causing the disease, 21.7 percent had ACTH-independent disease, and one patient had ectopic Cushing’s syndrome. All patients underwent surgery to treat their symptoms.

After a mean follow-up of 31.2 months, 7.8 percent of patients developed a new autoimmune or related disorder.

Researchers found no significant differences in age at diagnosis, gender, cortisol levels, and urinary-free cortisol at diagnosis, when comparing those who developed autoimmune disorders with those who didn’t. However, those who developed an immune disorder had a significantly shorter symptom duration of Cushing’s syndrome.

This suggests that increased cortisol levels, even for a short period of time, may contribute to more reactivity of the immune system after treatment.

The new disorder was diagnosed, on average, 9.8 months after Cushing’s treatment. The disorders reported were celiac disease, psoriasis, Hashimoto thyroiditis, Graves disease, optic nerve inflammation, skin hypopigmentation/vitiligo, allergic rhinitis/asthma, and nerve cell damage of unknown origin responsive to glucocorticoids.

“Although the size of our cohort did not allow for comparison of the frequency with the general population, it seems that there was a higher frequency of optic neuritis than expected,” the researchers stated.

It is still unclear why autoimmune disorders tend to develop after Cushing’s resolution, but the researchers hypothesized it could be a consequence of the impact of glucocorticoids on the immune system.

Overall, the study shows that children with Cushing’s syndrome are at risk for autoimmune and related disorders after their condition is managed. “The presentation of new autoimmune diseases or recurrence of previously known autoimmune conditions should be considered when concerning symptoms arise,” the researchers stated.

Additional studies are warranted to further explore this link and improve care of this specific population.

From https://cushingsdiseasenews.com/2018/03/06/after-cushings-cured-autoimmune-disease-risk-looms-study/

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Cushing’s and Hairy Nipples

Posted on March 3, 2018 by MaryO

Hairy nipples are a common condition in women. The amount of hair on the nipples varies, but some women find that the hair becomes long, coarse, and dark, which can be distressing.

Hairy nipples are rarely a cause for concern and are usually not a sign of any underlying health issues. However, occasionally they can signify something more serious, in which case, it is essential to consult a doctor.

Almost every part of a person’s skin is covered in hair and hair follicles. On certain parts of the body, such as the top of the head, the hair usually grows longer and thicker, while on other parts, it is thin and transparent.

Fast facts on hairy nipples:

  • It is not known how common hairy nipples are or how many women have them.
  • Many women do not report the condition and instead manage it themselves.
  • It is possible for hair that used to be fine and light to turn coarse and dark with age.

Causes of hairy nipples in women

There are several underlying reasons that might cause nipple hairs to grow. These are:

Cushing’s syndrome

Cushing’s syndrome is another condition caused by hormonal imbalance. When it occurs, there is an excess of cortisol in the body. In this case, a person may experience several symptoms, such as:

  • increased hair growth
  • abnormal menstrual periods
  • high blood pressure
  • a buildup of fat on the chest and tummy, while arms and legs remain slim
  • a buildup of fat on the back of the neck and shoulders
  • a rounded and red, puffy face
  • bruising easily
  • big purple stretch marks
  • weakness in the upper arms and thighs
  • low libido
  • problems with fertility
  • mood swings
  • depression
  • high blood glucose level

Cushing’s syndrome is fairly rare, and the cause is usually associated with taking glucocorticosteroid medicine, rather than the body overproducing the hormone on its own.

It is possible, however, that a tumor in the lung, pituitary gland, or adrenal gland is the cause.

Also:

Hormonal changes and fluctuations

Hormonal changes in women can cause many different symptoms, one of which is changes in nipple hair growth and color.

Some common hormonal changes happen during pregnancy and menopause.

However, hormonal changes can also occur when a woman is in her 20s and 30s, which may cause nipple hair to change appearance or become noticeable for the first time.

Overproduction of male hormones

It is possible for hormonal imbalances to cause hairy nipples. Overproduction of male hormones, including testosterone, can cause hair growth, while other symptoms include:

  • oily skin that can lead to breakouts and acne
  • menstrual periods stopping
  • increase in skeletal muscle mass
  • male pattern baldness, leading to a woman losing hair on her head

If overproduction of male hormones is suspected, it is a good idea to make an appointment with a doctor who can confirm this with a simple test.

What are the early signs of pregnancy?
Changes in nipple appearance or sensitivity can result from hormonal changes, such as those occurring in pregnancy. Learn more about the signs of pregnancy here.
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Polycystic ovary syndrome

Polycystic ovary syndrome (PCOS) occurs because of a hormonal imbalance. PCOS is a condition that affects the way the ovaries work.

Common symptoms of PCOS are:

  • infertility
  • irregular menstrual periods
  • ovarian cysts
  • excessive hair growth in unusual places, such as the nipples

PCOS is believed to affect around 1 in 5 women.

Medication

The side effects of particular medicines can cause unusual hair growth.

Medicines, such as testosterone, glucocorticosteroids, and certain other immunotherapy drugs may cause hairy nipples.

What are the treatment options?

Treatment for hairy nipples is not usually necessary for health reasons.

However, many women with the condition prefer to try and reduce or get rid of the appearance of hair on their nipples for cosmetic purposes.

There are several methods by which they can try and do this:

Trimming the nipple hair

Trimming the nipple hair may be enough to reduce its appearance. Small nail scissors are ideal, and hair can be cut close to the skin. It is essential to do this carefully and avoid catching the skin.

Trimming will need to be carried out regularly when the hair grows back.

Tweezing the nipple hair

Tweezing nipple hair is an effective way to get rid of unwanted nipple hair. However, this option can be painful as the skin around the nipple area is particularly soft and sensitive.

It is also important to bear in mind that the hair will return, and tweezing the hair increases the risk of infection and ingrown hairs.

Shaving the nipple hair

Shaving is another option to reduce the appearance of nipple hair. However, it is advisable to do so with caution to avoid nicking the sensitive skin.

This option also carries an increased risk of developing ingrown hairs and infection.

Waxing

Sugaring or waxing is a good hair removal option, though either one is likely to be painful. A salon is the best place to get this treatment type, as doing this at home may cause damage to the skin. Infection and ingrown hairs are again a risk.

Laser hair removal

These popular treatments can help to reduce the hair growth and slow or even prevent regrowth for a while. However, they can be painful, too.

Laser treatment is by far the most expensive option, as it will need to be performed by a plastic surgeon or cosmetic dermatologist.

Hormonal treatment

If a hormonal imbalance is the cause of hairy nipples, a doctor may prescribe or adjust a woman’s medication therapy to restore a healthy hormonal balance.

Other treatments and how to choose

The above treatments are all commonly used to remove and reduce nipple hair and usually have minimal side effects.

Bleaching or using hair removal cream to treat the condition, however, is not advised as these methods are usually too harsh for this sensitive area and may cause irritation and damage.

At what point should you see a doctor?

Hairy nipples in women are quite common, and there is usually no need to see a doctor. However, if they are accompanied by any other unusual symptoms, it is a good idea to make an appointment.

A doctor will be able to perform tests to determine whether an underlying cause, such as PCOS, is causing the growth of nipple hair. If so, they will give advice and medication therapy to help manage the condition.

A doctor will also be able to advise how to remove nipple hair safely.

Takeaway

For the majority of women, nipple hair may seem unsightly, but it is not a cause for any concerns about their health.

However, because some medical conditions can cause nipple hair to darken and grow, it is important to see a doctor if any other symptoms are experienced.

Nipple hair can usually be easily treated and managed, should a woman choose to try to remove the hair for cosmetic reasons.

Adapted from https://www.medicalnewstoday.com/articles/320835.php

 

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