Polyendocrine adenomatosis
Contents:
Description:
Family polyendocrine adenomatosis (SPEA) - the disease which is characterized by development of tumors in two and more closed glands, a thicket in Langerkhans's islands of a pancreas and epithelial body (a source - the main cells).
Symptoms of Polyendocrine adenomatosis:
Distinguish 3 SPEA types:
- Type I (Vyormer's syndrome, 131100, 1Ts13, MEN1 gene, R):): epithelial bodies, Langerkhans's islands of a pancreas and a hypophysis are involved
- The hyperparathyreosis arises approximately at 90% of patients (at a quarter from them - a hyperplasia of all epithelial bodies)
- Tumors of insular tissue of pancreas find at 80% of patients (as a rule, a gastrinoma, a glucagonoma or an insulinoma)
- Adenomas of a hypophysis observe in 65% of cases
- The stomach ulcers caused by pancreatic gastrinomy.
- Type II (Sippl's syndrome, #171400, 10qll.2, an oncogene of RET, [164761], R): [164761], it is necessary to suspect a disease at any relative of the patient having medullary cancer of a thyroid gland
- The medullary carcinoma of a thyroid gland is found in all patients
- Pheochromocytomas observe approximately at 40% of patients. Tumors are usually bilateral, sometimes malignant. In most cases manifestations of a pheochromocytoma arise later, than symptoms of cancer of thyroid gland
- The hyperplasia of epithelial bodies develops at 60% of patients.
- Type III (#162300, 10qll.2, RET oncogene, R)) regard as II option (sometimes designate the lib type, then Sippl's syndrome is designated as the IIA type)
- As well as at II SPEA, medullary cancer of a thyroid gland and a pheochromocytoma develops. The most characteristic signs: deformations of a skeleton and multiple neurinoma of mucous membranes
- SPEA III is shown at younger age (more often up to 20 years) and proceeds much more aggressively; earlier diagnosis is necessary.
Reasons of Polyendocrine adenomatosis:
The disease has a hereditary etiolgiya.
Treatment of Polyendocrine adenomatosis:
SPEA OF I
First of all it is necessary to eliminate a hyper parathyroid state. Gastrin secretion can be as a result reduced that favors to healing of stomach ulcers. The subtotal parathyroidectomy as the disease usually proceeds with a hyperplasia of all four glands is necessary. If the gipergastrinemiya and a peptic ulcer of a stomach do not respond to treatment, it is necessary to remove the tumor producing gastrin. If the tumor of a pancreas does not manage to be removed, and use of H2 blockers does not lead to healing of ulcers, carry out a resection of a stomach or a gastrectomy. Tumors of a hypophysis delete by a transsphenoidal hypophysectomy.
SPEA II
Medullary carcinoma of a thyroid gland (treatment is effective at a precancer stage [a hyperplasia S-kle-tok], the total thyroidectomy is shown). Pheochromocytoma or hyperplasia of a brain part of an adrenal gland: first of all carry out their treatment (to a thyroidectomy), otherwise when performing operation on a thyroid gland gipertenzionny crisis is possible. The hyperparathyreosis can be cured by a total thyroidectomy.
SPEA III. Therapy is similar to type II. As the type III proceeds especially aggressively, early and radical treatment is necessary.
