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
Introduction
Multiple endocrine neoplasia is an autosomal dominant inherited disease. It is associated with hormone release and is a rare syndrome. MEN (multiple endocrine neoplasia) manifests itself in different organs and therefore leads to different clinical pictures depending on the hormone exposure of the organ.
Classification
Multiple endocrine neoplasia is divided into 2 types, which manifest themselves in different organs. MEN type 1, also known as wormer syndrome, is distinguished from MEN type 2. For the sake of clarity, the two types with their symptoms, therapy and diagnosis are considered separately below.
Men Type 1-Wermer Syndrome
Type 1 multiple endocrine neoplasia is characterized by a familial cluster of tumors of the pituitary, parathyroid and pancreas.
Frequency
Multiple endocrine neoplasia is a rare disease. The prevalence is about 1 in 100 000.
Cause
The clinical picture of the wormer syndrome (multiple endocrine neoplasia) can be familial, but also sporadic. In some cases endocrine neoplasia is based on a gene mutation in the menin gene. This gene is located on chromosome 11 and suppresses tumor growth (tumor suppressor) when functioning properly. This gene is available for gene analysis and can therefore be tested in familial cases of multiple endocrine neoplasia.
Symptoms
The symptoms of multiple endocrine neoplasia depend on the location of the tumors. Basically, the tumors in the pituitary gland, parathyroid gland and pancreas all occur over time, but in different chronological order. As a rule, the initial manifestation of the tumor determines the symptoms.
Also, not every tumor produces all the hormones listed below. Accordingly, the clinical picture is composed of the different hormone effects, depending on the tumor manifestation and function. In addition, both benign and malignant tumors can occur in combination.
Pituitary gland
The pituitary gland produces various hormones. These include the growth hormone STH. In the case of overproduction, large growths (acromegaly) occur clinically.
The feet and hands as well as the nose and ears are particularly affected. In addition, the internal organs also grow more and more and can thus lead to space problems and functional limitations. Furthermore, the pituitary gland produces the adrenocorticotropic hormone (ACTH).
This stimulates the adrenal cortex and thus leads to Cushing’s syndrome. The reason for this is an excessive production of cortisol, which leads to a typical external appearance with moon face, trunk obesity and bull neck. Prolactin is also produced by the pituitary gland.
This hormone is responsible for milk production during pregnancy. If prolactin is overproduced by a tumor, irregularities or the absence of menstruation occur. In addition, milk production and secretion of milk via the mammary glands can occur. This is of course only clinically noticeable if there is no pregnancy or if the patient is not breast-fed. However, prolactin overproduction can also occur without symptoms.
