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Cushing's disease is a difficult pituitary tumor.

jkcfzk @ 2019/07/12


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70% of Cushing's syndrome is derived from the pituitary gland.
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In 1921, Cushing, the father of the American Department of Neurosurgery, discovered Cushing syndrome. This is a clinical syndrome caused by a variety of causes of chronic glucocorticoids secreted by the adrenal cortex.

Professor Bian Liuguan, chief physician, Department of Neurosurgery, Ruijin Hospital Affiliated to Shanghai Jiaotong University School of Medicine The main clinical manifestation is centripetal obesity, namely abdominal obesity, round face, but small limbs. Fat pad on the back. Medicine is called buffalo back. Facial flush, beard and temples are thick. The body has purple stripe, similar to the stretch marks, but the stretch marks will fade with time and the width is not large. The purple lines of Cushing's syndrome will be pigmented and red, with a width of about 1 centimeters. These are typical clinical manifestations of Cushing's syndrome.

Bian told him that Cushing syndrome was caused by several sources, including adrenal glands. Ectopic sources, such as lung cancer, can secrete adrenocorticotropic hormone releasing hormone. There are also sources of pituitary.

He pointed out that Cushing's syndrome, which is 70% of pituitary origin, is called Cushing's disease. Cushing's disease accounts for 5%~10% in hormone secreting pituitary adenomas. It is pituitary ACTH adenoma or ACTH cell hyperplasia. Excessive secretion of ACTH leads to hyperplasia of adrenal cortex and hypercorticosteria, leading to a series of metabolic disorders and pathological changes.

Bian said that Cushing's disease will not only cause changes in appearance, it can involve multiple organs and systems throughout the body, and the clinical risk is serious. The mortality rate is four times higher than that of the normal population. In addition, some patients with Cushing's disease have brain atrophy and cognitive impairment. And some patients have serious psychological disorders. They need antidepressant and anti anxiety therapy for a long time. Therefore, this is a kind of disease that needs attention.

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It is difficult to diagnose Cushing's disease.
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Many hospitals were diagnosed with many hospitals. Bian said the diagnosis of Cushing's disease is rather difficult, and it is a disease that needs multidisciplinary collaboration.

First of all, it must have typical clinical manifestations. How can we determine whether obesity is simple obesity or obesity in Cushing's disease? Bian said, obesity of Cushing's disease may also merge some other physical problems, such as fungal infection, it is difficult to cure, the most common is the treatment of onychomycosis; osteoporosis, prone to pathological fracture; long duration of disease, accompanied by hypertension and diabetes. He also mentioned that some of the patients were not treated because of obesity. Many of them were treated because of their bad nails. They broke down at an early age or had high blood pressure and diabetes. Finally, they discovered that Cushing's disease was the cause.

Apart from some clinical manifestations, diagnosis is the most important one, and the most important is biochemical examination. This requires Department of Endocrinology examination.

First, check the level of corticotropin secretion and the level of pituitary adrenocorticotropic hormone (ACTH).

If cortisol levels are not high and ACTH levels are normal, this obesity may not be ill.

Second, dexamethasone suppression test.

Under normal circumstances, the body has a negative feedback mechanism. Once suspected, a small dose of steroid inhibition test was first given. For example, given 1 mg or 2 mg of dexamethasone, if cortisol secretion is inhibited, then it is not Cushing syndrome. If 1 mg or 2 mg of dexamethasone is given, cortisol secretion is not inhibited, then it is proved to be Cushing's syndrome.

Next, we need to find out the source of Cushing's syndrome and give a 8 mg dexamethasone suppression test. If the cortisol level is suppressed after use, then we can prove that it is diagnosed as Cushing's disease from the pituitary gland.

Third, collecting blood from inferior petrosal sinus.

But sometimes the inhibition test can not fully diagnose Cushing's disease. Some patients at the critical point are also hard to ascertain that they must be from the pituitary gland, and they can not find the cause of the adrenal gland, and can not find the cause of ectopic. From bilateral femoral vein catheterization to the inferior petrosal sinus, blood samples from different parts were collected to determine the level of ACTH in different parts. If the pituitary is derived, then the level of ACTH in the inferior petrosal sinus is high. This is the gold standard for diagnosis.

Fourth, imaging examination.

Pituitary enhanced MRI can be done to see if there is any tumor. CT of the adrenal gland to see if there are hyperplasia of two adrenal glands and whether there are tumors. The lungs should be shot at CT to see if there is any lung condition. Sometimes even PET-CT is needed to find other heterotopic tumors.

Bian told him that it was precisely because of the complexity of the diagnosis that many patients were misdiagnosed and mistreated.

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Surgical treatment is the first choice for the treatment of Cushing's disease.
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Bian said, for the treatment of Cushing's disease, surgery is the first choice. Although there are drugs, but drugs mainly inhibit cortisol levels, is a temporary solution, clinical symptoms will improve, but not cure, some patients may lead to increased pituitary tumor after taking drugs.

Bian director, Endoscopic sinus surgery is the main procedure for Cushing's disease. Although some patients are estimated to be pituitary sources before surgery, magnetic resonance imaging does not necessarily detect tumors, which requires the experience of neurosurgeons. Therefore, surgery must be carried out in hospitals that are highly specialized in the treatment of Cushing's disease.

What is the effect of Cushing's disease after surgical treatment? Bian said, from the surgical patients in Ruijin hospital, the cortisol levels in the 5 years after operation were all above 90%, which is at a relatively good level both domestically and internationally.

In addition, Bian pointed out that Cushing's disease also requires lifelong management and lifelong follow-up.

The increase of endogenous cortisol is the direct cause of Cushing's syndrome. If the operation is successful, the cortisol level will decrease to a very low risk level. But cortisol is a hormone necessary for life. A very low level of cortisol can directly lead to death. However, if it does not reach this dangerous state, it means that the operation has not completely removed the tumor. Therefore, hormone levels should be monitored and hormone replacement therapy should be carried out in time. The first month after operation is very dangerous. Need to be followed up in the hospital for drug adjustment.

Some patients are in adolescence, surgery on the gonadal development will be affected, then regular follow-up, the original disease control, can be done at the appropriate time, gonadal reconstruction, after completion of the life stage.

In addition, some patients had brain atrophy and cognitive dysfunction before operation. High cortisol levels can also cause psychological problems in patients. Therefore, for patients with psychological disorders before surgery, it is also necessary to follow up whether psychological problems can be improved.

In conclusion, Cushing's disease is a disease that requires multidisciplinary collaboration, so that patients can better get back to society and live and work like normal people.



Introduction to experts

Bian Liu Guan Now he is director, chief physician, professor and doctoral supervisor of Department of Neurosurgery, Ruijin Hospital Affiliated to Shanghai Jiaotong University School of Medicine. He is mainly engaged in clinical and basic research of intracranial tumors and cerebrovascular diseases. Specializes in single nostril transsphenoidal resection of pituitary tumor, skull base tumor, hemifacial spasm, microvascular decompression of trigeminal neuralgia, intracranial aneurysm clipping, brain stem tumor, intramedullary tumor, acoustic neuroma, foramen magnum tumor, jugular foramen tumor, etc.


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