All information given here is of general nature only, a tumor therapy always belongs in the hands of an experienced oncologist!
Synonyms
Medical: hormone producing tumor
Definition
Multiple endocrine neoplasia type 2 is characterized by the appearance of tumors in the parathyroid gland, the adrenal medulla and the C-cells of the thyroid gland.
Frequency
About 5% of all thyroid carcinomas are tumors of the C-cells. Of these, about 25% are familial and associated with MEN- 2.
Classification
Type- 2a:
Symptoms
Type- 2a: The C-cell carcinoma of the thyroid gland runs for a long time without clinical signs. Late symptom is a therapy-resistant diarrhea in about 1/3 of the patients. The adrenal cortex tumor is associated with chronic high blood pressure.
Type- 2b: This type manifests itself as Marfan syndrome with very large hands and feet, as well as long arms and legs. Since the vessels are also affected by the collagen defect, the diagnosis is often already made. In addition, small neuromas are visible on the tongue.
Diagnostics
A reliable tumor marker of C-cell carcinoma of the thyroid gland is the significantly elevated Calcitonin. In addition, there is an increase in adrenal cortex hormones in the case of a corresponding tumor. If one of the symptoms persists, the other tumors should always be searched for in order to be able to initiate therapy as early as possible. In addition, genetic diagnostics should be performed to detect a familial disposition at an early stage and to treat affected individuals accordingly (multiple endocrine neoplasia type II).
Therapy
The therapy of the MEN- 2 tumors consists in the removal of the tumors. The thyroid gland of C-cell carcinoma is completely removed. The metastases of the thyroid carcinoma are also completely removed.
Family screening in case of predisposition for multiple endocrine neoplasia allows to detect thyroid carcinoma at a very early stage and to cure the patient by removal of the thyroid gland. The adrenal cortex tumor, which is often present on both sides, should also be completely removed. Surgery is therefore the only and very effective therapy for multiple endocrine neoplasia.
