What is Cushing’s syndrome, and why are we suffer this disease?

What is Cushing’s syndrome, and why are we suffer this disease?

What is Cushing’s syndrome, and why are we suffer this disease?

Cushing’s syndrome is a condition in which cortisol hormone is produced more than normal consumption level of the body. Symptoms often appear gradually and as a result, their detection will be hard in some cases. The disease was first introduced by a Neurologist and surgeon named “Harvey Cushing”.

Cortisol is produced by adrenal glands (which are placed in the upper side of kidneys), and is completely vital, and has several actions that is mentioned below:

  • Helping in regulation of blood pressure
  • Helping in regulation of immune system
  • Helping in regulation of insulin effects for maintenance of natural blood pressure
  • Helping in stress responses of the body.

As it is mentioned, this disease is a result of more than normal secretion of cortisol hormone, and affected patients should be tested from several sides for diagnosis of exact cause of their disease.

According to performed researches, prevalence of this disease is 1 to 200000. The cause of complexity of the disease is that the signs have wide spectrum, so diagnosis could be delayed in the early stages of that.

Based on statistical studies, number of affected women is more than affected men; however, the cause of this finding it not clear, yet. The age range of patients is often between 30 to 40 years old. Although the prevalence of Cushing’s syndrome is rare in children, some cases of children affected by the disease are also reported in the mean age of 6 years.

It should be mentioned that special environmental factor that could have significant effect on the disease, has not been reported.

What are the symptoms of Cushing’s syndrome?

Signs of the disease are very variable, however, signs that are common in the patients are:

  • Sudden weight gaining in the trunk (extremities may be remained without any changes, or are thinner than other parts)
  • Muscular weakness, especially in legs
  • The face could be more blushed than a normal person (it so called “face moon”)
  • Development of pimple in the surface of skin
  • Bones may be weakened (a X-ray scan can display this)
  • Blood pressure may be higher than standard limit and diabetic signs will be appear
  • Lines similar to pregnancy may be appeared on the abdominal skin surface, and/ or may easily bruise.
  • Some women may experience disruptions in the menstruation or stopping it.
  • Hair growing may become more than normal (especially at the face of women), and sexual trends may decrease in men and women.
  • Psychological problems such as continuous changes in mental states and signs of depression and anxiety may be appeared.
  • In children may result to stopped grow and increased body weight.

As Cushing’s syndrome appears gradually, in several cases may remain completely silent and result to depression signs

Regarding reports of the patients, many of them think that their signs has appeared during last 1 or 2 years before their referral to endocrinologist. In general, lack of public awareness about Cushing’s syndrome resulted to delay in diagnosis of several patients.

Cushing’s syndrome specifically is classifies to two groups:

  • Dependent to ACTH hormone: In this case, body produces more ACTH hormone that results to excessive increase of cortisol hormone, and hypophysis tumors are placed in this group.
  • Independent to ACTH hormone: In this case, adrenal gland secrets more than normal cortisol hormone, ACTH hormone is low, and could be a result of adrenal tumor.

How Cushing’s syndrome could be treated?

Treatment is selected based on the cause of disease. The specialist may diagnose that using medicines may be effective, and in some cases, using medicine may decrease production of cortisol hormone in adrenal gland and/or production of ACTH in hypophysis gland.

Hypophysis tumors with diagnosis of Cushing’s syndrome, may be malignant that requires surgery, and the neurologist surgeon may propose radiotherapy or chemotherapy after surgery.

 If a tumor is the cause of Cushing’s syndrome development, specialist may do surgery. Hypophysis tumors often could be removed through nose (endoscopic).

After surgery, patient should continue therapeutic process via medicines to decrease the rate of secreted cortisol to a balanced rate. In most of cases, patients after doing mentioned steps will reach to the normal adrenal hormone rate, and specialist will stop treatment via medicine.

How could surgeon find tumor though nose?

Surgery through nose could be an appropriate and effective method for hypophysis tumors. Surgery area is approximately 0.5 inch (1.25 cm), so the mentioned tumor could not be directly seen. As technology progresses, new tools has developed to see surgery area from a small hole.

This surgery is performed via a strong microscope that is specialized for surgery, and/or via fiber-optic endoscope. Endoscopic method provides more and wider view, and surgeon sees the desired area in an installed monitor.

How tumor could be removed?

Hypophysis tumors often have soft tissue and could be removed via tiny surgery tools. If the tumor is very big, it is required to divide mentioned tumor to small sections to remove it completely. In Cushing’s syndrome, tumors are often small and could be hardly observed in MRI scan.

It is an important point that if the mentioned tumor is extended to the cavernous sinus and/or Dural sinus, there is no possibility to completely empty it, and complementary therapies (such as radiotherapy, adrenalectomy, and/or using medicines) should be followed after surgery to reduce cortisol hormone to its normal level.

How much is the recovery chance?

Recovery chance in Cushing’s syndrome, after surgery by an experienced surgeon is estimated to be between 80% to 90%.

 

 

Which recoveries could be achieved after treatment steps?

Totally, all physical and mental problems related to Cushing’s syndrome will be disappeared in most of patients, after one or two years.

How many is the percent of Cushing’s syndrome recurrence?

The possibility of its recurrence is low (5-10%). The specialist should continuously follow up patient after surgery.

How to choose the best neurologist for hypophysis tumor surgery?

This is highly related to experience of surgeon in successful surgeries of hypophysis tumors. In general, highly experienced surgeons in surgery of hypophysis tumors will have a high chance in treatment of the disease. Specialized team of Dr. Giv Sharifi, currently perform weekly hypophysis tumor surgery (through nose) in the Erfan and Loghman hakim hospitals (Shahid Beheshti university of medical sciences), and have published several articles in this field. Also, several presentations performed by Dr. Sharifi, about this surgery in Iran, in foreign international conferences.

Introduction of a case with Cushing’s syndrome, which has tolerated the surgery:

The patient was a 22 years old with abnormal weight gaining in limited time, and mental signs (depression) that were referred to endocrinologist. After hormonal examinations it was showed that cortisol level in not normal, and MRI scan approved the presence of a 7.5 mm mass in the hypophysis area.

After referral to Dr. Sharifi’s clinic, the diagnosis showed that surgery is required for this patient. Surgery performed by surgical team of Dr. Sharifi in Erfan hospital. The surgery was successful and mentioned tumor were completely removed.

Therapeutic actions after surgery performed as usual rules of Dr. Sharifi’s clinic. Next follow up showed that the patient had was good and his problems were disappeared.

You can see his after and before surgery scans here:

 

 

Patient’s MRI before surgery

 

A 7.5 mm tumor could be seen in the left of adenohypophysis (attached figure includes coronal, axial, and sagittal views).

Patient’s MRI after surgery

Observations and results of MRI after surgery:

  • In the MRI of the patient after surgery, alterations after surgery are seen as bone defects in the floor of bonny Sella, and sphenoid sinuses with partial sepectomy.
  • Thickness of mucosa is seen in sphenoid sinuses and some of ethmoid air cells in both sides.
  • Hypophysis gland is seen as a delicate crescent shape structure at the floor of bonny Sella.
  • Hypophysis stalk is in the natural status and is ended at the middle line to upper surface of the gland.

amir bigdeli
amir bigdeli
it manager in paytakht medical imaging center

1 Comment

  1. Thanks for the article post.Really thank you! Great.

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