Multiple endocrine neoplasia Men type 2
All information given here is only of a general nature, tumor therapy always belongs in the hands of an experienced oncologist!
Synonyms
Medical: hormone-producing tumor
Also read:
- Multiple endocrine neoplasia
definition
Multiple endocrine neoplasia type 2 is characterized by the occurrence of tumors in the parathyroid gland, the adrenal medulla and the C cells of the thyroid gland.
frequency
Approx. 5% of all thyroid carcinomas are C-cell tumors. About 25% of these are familial and therefore MEN-2 associated.
Classification
Type 2a: combination of a C-cell carcinoma of the thyroid gland with an adrenal carcinoma on both sides. This type occurs in about 70% of the cases and is also known as Sipple syndrome.
Type 2b: Clear symptoms of a collagen defect (Marfan syndrome) with adenomas of the mucous membrane (e.g. the tongue) and a giant colon (megacolon). At the same time, 10% of the patients still have thyroid carcinoma, and about 50% have adrenal carcinoma.
FMTC only: Only medullary thyroid carcinoma is present. This occurs in around 20% of MEN-2 cases.
Symptoms
Type- 2a: The C-cell carcinoma of the thyroid runs for a long time without clinical abnormalities. Therapy-resistant diarrhea is a late symptom in about 1/3 of patients. Of the Adrenal tumor goes with one chronic high blood pressure hand in hand.
Type- 2b:
This guy manifests as Marfan's Syndrome with very big hands and feet, such as long arms and legs. Since the vessels are also affected by the collagen defect, the diagnosis has often already been made. In addition, small neuromas can be seen on the tongue.
Diagnosis
More reliable C-cell carcinoma tumor markers the thyroid is significantly increased Calcitonin. In addition, there is an increase in the adrenal cortex hormones with a corresponding tumor. If one of the symptoms exists, the other tumors should always be searched for in order to initiate therapy as early as possible. In addition, genetic diagnostics should be carried out to family disposition To be recognized early and to treat those affected accordingly (multiple endocrine neoplasia type II).
therapy
The therapy of MEN-2 tumors consists in the removal of the tumors. The thyroid gland is completely removed in C-cell carcinoma. The metastases of thyroid carcinoma are also completely removed. By means of family screening in the case of a predisposition to multiple endocrine neoplasia, thyroid carcinomas can be detected at a very early stage and the patient can be healed by removing the thyroid gland. The adrenal gland tumor, which is often present on both sides, should also be completely removed. The operation is therefore the only and very effective therapy for multiple endocrine neoplasia.
forecast
In sporadic cases and the index case in a family, a cure is usually not possible. However, surgery can significantly extend and improve patient survival. However, through family screening, it is possible to operate on patients at very early stages and thereby heal them. Nevertheless, the various tumor markers should be checked annually in these patients.
