Concept of acromegaly pituitary gland
Patients with growth hormone (GH) adenoma (or mixed adenoma mixed with other hormones) overproduce GH and clinically present with progressive enlargement of hands and feet, head, thorax and limbs, hypertrophy of palms, thickening of fingers, spherical distal
The forehead bulges, the orbits, cheekbones and jaws protrude significantly, the gap between the teeth widens, the teeth bite incorrectly, the mouth and lips thicken, the nose is wide and flat, the ears become larger (hats, shoes and socks, gloves are often replaced with larger sizes); the skin is rough, sagging, oily
excessive sweating, hyperpigmentation, sleep apnea, hypertension, diabetes mellitus, colon polyps, and skin redundancy are among the manifestations of this group of manifestations clinically referred to as acromegaly. Although the diagnosis of acromegaly is not difficult, many patients
Many patients present to the hospital 7 to 8 years after the onset of symptoms. Patients with acromegaly can die prematurely if they do not receive treatment, mostly from cardiovascular disease. Therefore, early diagnosis and treatment are key to reducing the risk of disease and early mortality.
The key to reducing the risk and early mortality is early diagnosis and treatment. The way to reduce the risk of early death is to reduce the GH level in blood to normal as soon as possible. Diagnosis of acromegaly in Chen Ge, Department of Neurosurgery, Xuanwu Hospital, Capital Medical University Excessive GH can cause elevated serum insulin-like growth factor (IGF-1) levels, and serum GH levels do not decrease sufficiently after oral glucose administration (glucose tolerance test). Serum IGF-1 levels correlate with the patient’s age and gender. GH secretion and serum IGF-1 concentrations decrease with age and are higher in women than in men. IGF-1 level testing is a useful method for screening patients with suspected acromegaly. Pituitary GH adenomas may not manifest early in the course of acromegaly, but may be accompanied by excessive production of lactogen, manifesting as overflow, menstrual disorders and hyperprolactinemia, and should be screened for acromegaly in such young women. Confirmation of the diagnosis of acromegaly is indicated by a rise in IGF-1 concentration and a serum GH level >1.0ng/ml (ug/L) even after 75g or 100g of oral glucose (2.5mU/L). Oral glucose tolerance test: blood GH levels were measured every half hour before and every 2 hours after the start of glucose ingestion (5 blood samples). Patients should undergo pituitary MRI after definite GH elevation. Most patients with acromegaly have pituitary macroadenomas and some patients have microadenomas. Treatment of acromegaly limbs
The first treatment for acromegaly is surgical removal of the adenoma by a neurosurgeon experienced in pituitary surgery, the outcome of which depends on the experience and expertise of the neurosurgeon. Although pituitary macroadenomas, especially when the tumor invades the hippocampal sinus or bony structures, can be very difficult to treat.
Although it is almost impossible to cure a large pituitary adenoma by surgery alone, especially if the tumor invades the sinus or bone structures, surgical removal of part of the lesion is still needed to relieve pressure on the visual pathway to avoid loss of vision and to set the stage for the next step of radiotherapy. The surgical cure rate is 50% to 70%. A significant proportion of patients still require other adjuvant treatments. Post-operative evaluation is possible because of the short GH half-life (only 19 min), so an oral glucose tolerance test can be performed in the early post-operative period (within 1 week) to see if blood GH levels are <1.0 ng/ml (ug/L) (2.5 mU/L). In contrast, IGF-1 concentration decreases slowly and must be measured after 3 months to be accurate and reliable. The most desirable outcome of treatment is if the blood IGF-1 level reaches a level compatible with normal age and sex. Medications Current useful medications include dopamine agonists and growth inhibitor analogs. Bromocriptine (15-40 mg/d orally, not effective in all patients; bromocriptine sensitivity test: efficacy in those with >50% decrease in GH
Dopamine agonists such as bromocriptine (15-40 mg d, oral, not effective in all patients; bromocriptine sensitivity test: better in those with >50% GH decrease) can provide symptomatic relief in 90% of patients, but less than 20% of patients will have normal blood IGF-1. Growth inhibitors are only effective when given continuously intravenously. Growth inhibitor analogs can be administered
Subcutaneous or intramuscular injections, including octreotide and sertraline (20mg/28d, intramuscular), are effective. These drugs not only improve symptoms in 90% of patients, but also reduce blood GH
and IGF-1 to normal levels. However, the drugs are expensive. A newly developed GH antagonist, Pegvisomant, has been reported to be 20 mg/d, injected subcutaneously by the patient himself.
It was reported that 89% of 112 patients had their blood IGF-1 suppressed to normal levels. It has also been reported that 90% of 90 patients with a course of treatment longer than 12 months achieved normal serum IGF-1 concentrations. With the drug
Afterwards, blood GH levels increased and reached a plateau level at about 6 months. In this study, 2 patients had enlarged pituitary adenomas and the rest had no change in tumor size. This drug is currently the most effective. Radiotherapy Pituitary radiotherapy should be used as an adjuvant treatment for residual postoperative lesions. Because radiation therapy usually takes months to years to bring GH and IGF-1 down to normal levels, patients should be given medications while waiting for radiation therapy to take effect
Treatment. In recent years, it has been reported that gamma knife treatment is more effective than normal radiotherapy. The side effects of radiotherapy are: progressive hypopituitarism, reduced secretion of some or all pituitary hormones; the earliest and most common deficiency is gonadotropin (LH,FSH).
(LH,FSH) and secondary hypogonadism. At 4 years of follow-up, gonadotropin deficiency has been reported in 67%, ACTH deficiency in 67%, and TSH deficiency in 55%. Patients receiving pituitary radiation therapy should be followed up regularly (at least every 6 months) with hormone level testing and replacement therapy if necessary. In conclusion, from a therapeutic point of view, surgical treatment is considered first, and transsphenoidal sinus surgery is the preferred method. Neuroendoscopic transnasal pterygoid surgery has more advantages than conventional surgical microscopic transsphenoidal sinus surgery: less trauma, faster recovery, safer and more complete tumor removal, less postoperative patient discomfort, and no increase in treatment cost. Endoscopic transsphenoidal resection of pituitary tumors for acromegaly has become a trend of treatment at home and abroad.