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“Cortisolism” is not always “pituitary ACTH adenoma”.
“Pituitary ACTH adenoma, also known as Cushing’s disease, has a clinical manifestation called Cushing’s syndrome and is a disease that currently has a chance of being cured through surgical treatment.
Cushing’s syndrome, or cortisolism, is a collective term for three separate pathological syndromes (pituitary, adrenal, and ectopic) whose clinical symptoms are primarily caused by excessive cortisol production. In pituitary Cushing syndrome (Cushing disease), pituitary ACTH adenomas account for approximately 80% of the symptoms.1. Clinical signs of Cushing syndrome The relationship between the symptoms of Cushing syndrome and hormones is not yet understood. The elevation of cortisol can only cause obesity and mental disorders, but through the inhibition of pituitary gonadotropin secretion and lead to a significant increase in testosterone in female patients, manifesting as three major symptoms of menstrual disorders, hirsutism and acne; on the contrary in men, it leads to a decrease in testosterone and impotence, scarcity of semen and loss of libido. The excessive decomposition of protein presents symptoms such as muscle atrophy and weakness, osteoporosis, low back pain, thinning of the skin, redness of the face and purple lines on the abdomen. The clinical diagnosis and differential diagnosis of ACTH adenoma is not difficult, but the etiology of Cushing’s syndrome requires an in-depth analysis of three different conditions, which should be treated differently.
(1) Determine whether there is Cushing syndrome or not: measure the blood and urine cortisol level, and any abnormal level can be defined as Cushing syndrome.
(2) Identification of the three types of Cushing syndrome: Measurement of blood ACTH values (RIA method) and identification according to the results: a Blood ACTH values are lower than normal and are adrenal in nature.
b ACTH values above 200 pg/ml are ectopic. Some malignant tumors can produce ACTH. c Normal or slightly high ACTH: Pituitary. This is due to a negative feedback effect, resulting in a relative decrease in ACTH secretion, so that it actually exceeds normal values.
d ACTH values between 100-200 pg/ml need to be differentiated by dynamic function tests, radiological examinations and determination of ACTH congeners (β-LPH, etc.).
(3) Large and small doses of dexamethasone inhibition experiment: before and after taking the drug, blood cortisol (blood F), urinary free cortisol (urinary FF), urinary 17 hydroxysteroids (urinary 17-OH), urinary 17 ketosteroids (urinary 17=O) were measured respectively. Cushing’s syndrome; when large dose (DXM 8mgP.O), drop more than 50% for pituitary sex, drop less than 50% for adrenal sex. And note the circadian rhythm changes.
(4) Confirmation of diagnosis of pituitary ACTH adenoma: In pituitary Cushing syndrome, ACTH adenoma accounts for 80%, so once pituitary Cushing syndrome is identified, even if there is no indication in radiological imaging, direct transsphenoidal microsurgical exploration is mostly advocated. 3. For those who are unable to locate the tumor by imaging, the transsphenoidal approach should be used. For those who are unable to locate the tumor by imaging, the tumor should be explored under high magnification of the operating microscope, and the tumor can be found mostly. However, tumors less than 3 mm in diameter are easily missed during surgery, and ACTH adenomas are highly invasive, so some scholars also advocate extended selective tumor resection, i.e., resection of adenomas followed by removal of thin layers of peritumoral pituitary tissue. If no tumor is found on pituitary exploration, some experts believe that subtotal or complete excision of the pituitary gland is feasible.
The failure of surgery or recurrence may be related to the following factors: 1) wrong diagnosis, i.e. non-pituitary Cushing’s syndrome misdiagnosed as Cushing’s disease; 2) invasive nature of ACTH adenoma, the tumor is very small and has invaded the peripheral tissues of the saddle base, which cannot be completely removed; 3) multiple ACTH adenomas in the pituitary gland, which cannot be completely removed; 4) pituitary hyperplasia without total pituitary resection.
Internal treatment: At present, there are three major types of drug treatment options available, including pituitary targeting drugs, adrenal enzyme inhibitors and glucocorticoid receptor inhibitors. In particular, the new growth inhibitor analogue pareotide has been a hot spot for the treatment of Cushing’s disease in recent years. Patients can choose to be treated by the endocrinology department of a reputable large public hospital.
Gamma-knife therapy and general radiotherapy: Due to the damaging effects on normal pituitary gland and optic cross, general radiotherapy has been basically not recommended. Gamma-knife therapy, as a highly regarded treatment method, is more often recommended to patients by primary hospitals and doctors who have no in-depth research on pituitary tumors due to the addition of commercial factors, but authoritative expert opinions have rejected the practice of recommending gamma-knife therapy to patients as the first choice. Therefore, gamma-knife treatment can be used as a useful complementary treatment for patients who are “not suitable for surgery due to their age and physical condition, and whose tumors remain after surgery due to tumor characteristics”.
Evaluation of treatment results: Surgical resection is the treatment of choice for ACTH adenomas. Experts believe that surgery is too late, difficult to cure, and has a poor prognosis for patients with large adenomas with enlarged butterfly saddles and visual cross compression. Therefore, surgery is mainly for early stage microadenoma cases, which can achieve the dual purpose of biological cure and preservation of pituitary function by selective removal of microadenoma. In the case of successful radical surgery, the postoperative pituitary-adrenal axis showed temporary hypofunction, suggesting successful selective adenoma removal, and suggesting that the hypothalamic-pituitary-adrenal axis has entered a lazy phase of response to the removal of ACTH adenomas after a long-lasting hypercortisolism, which takes about one year to recover gradually. Therefore, this is the best indicator of surgical eradication of ACTH microadenoma. After subtotal pituitary resection, permanent replacement therapy is mostly required.
Surgical treatment selection: Since ACTH adenoma is usually just a microadenoma, even a type of tumor that is “difficult to identify with the naked eye” on MR films, and the thoroughness of surgery has extraordinary significance for the prognosis of the disease, the technical requirements for surgery require extremely strict screening, and it is generally recommended that individuals with an average annual It is generally recommended that a surgeon with an annual average of more than 50 pituitary tumor surgeries, including more than 20 microadenoma surgeries, be the chief surgeon, and the use of microscope or endoscope is not limited. In addition, the hospital where the surgeon works should be considered a large medical school hospital with complete neurosurgical facilities and strong endocrinology support.
What should the patient do?
The first step is to complete the diagnosis of “cortisolism” in the endocrinology department of a large tertiary care hospital, and if possible, to clarify the diagnosis of central (pituitary) tumor and find the tumor.
The second step is to find a unit and surgeon with rich experience in the treatment of pituitary tumors, and complete the surgical treatment under the guidance of the surgeon to achieve total removal of the tumor as much as possible.
The third step is to return to the endocrinology department for disease monitoring and efficacy follow-up after postoperative review (usually 3 months after surgery, or earlier in special cases).