Multiple endocrine neoplasias What we need to know

Multiple endocrine neoplasias are a group of congenital pathologies that affect the endocrine system. It includes benign or malignant tumors and hyperplastic processes of endocrine glands.

There are two main types of multiple endocrine neoplasia: type I and type II. Multiple endocrine neoplasia type II includes two subgroups: Men2A and Men2B

Type 1  Multiple endocrine neoplasia (MEN)

Multiple endocrine neoplasia type I – Wermer's syndrome – is a rare condition in which tumors occur in the endocrine glands, small intestine, and stomach. Tumors from the endocrine glands develop mainly in the pancreas, thyroid gland, and pituitary gland. The tumor produces hormones in excess, which leads to the development of the corresponding diseases. In most cases, tumors are benign. The disease is congenital.

Type 1 ME is most characterized by hyperparathyroidism (i.e. hyperfunction of the thyroid glands), the symptoms of which are:

• Kidney stone
• strong weakness
• bone pain

Multiple endocrine neoplasia (MEN) type I is associated with:

• Pituitary adenoma (characterized by headache, menstrual cycle disorders, galactorrhea from mammary glands)
• Pancreatic neuroendocrine (islet cell) tumors (hypoglycemia, weakness, altered consciousness, coma, abdominal pain, diarrhea, nausea-vomiting)
• Adrenal gland tumors (with possible damage to bronchi, thymus, connective tissue, fatty tissue)

Symptoms

The excess of hormones causes various clinical manifestations. Symptoms depend on which gland the tumor is in and which hormone excess occurs.

• Weakness and fatigue
• bone pain
• fractures
• Kidney stone disease
• Stomach and intestinal ulcer disease

Diagnosis

Multiple endocrine neoplasia type I diagnostics requires physical examination, collection of disease and family anamnesis, and instrumental-laboratory studies.

Laboratory diagnostics is based on the study of hormones, the excess of which is synthesized due to the tumor of the glands (parathyroid hormone, prolactin, growth hormone, gastrin, etc.)

Treatment

In multiple endocrine neoplasia type I, tumors are found in the parathyroid glands, pancreas, and pituitary gland, and the excess hormones produced by them lead to the development of various conditions. The treatment plan is determined by the location of the tumor and clinical indicators.

Pituitary tumors - Treatment is usually surgical. Some tumors can also be treated with medication.

Hyperparathyroidism – In the case of tumors with excess of parathyroid hormone, surgical excision of most of the parathyroid glands is recommended.

Neuroendocrine tumors - Tumors located in the pancreas or duodenum. Treatment depends on the type and stage of the tumor.

hypoglycemic syndrome - It develops in the presence of insulin-producing tumors - insulinomas. At this time, the glucose level may drop critically and a life-threatening condition may develop. Treatment is usually surgical, with resection of the pancreas along with the tumor.

Zollinger-Ellison syndrome – It is carried out during gastrinomas, which cause increased gastric secretion and the development of diarrhea and ulcer diseases. Treatment is surgical or medical.

Other neuroendocrine tumors of the pancreas - These tumors produce other hormones, the excess of which causes various pathologies.

Metastatic neuroendocrine tumors – Metastases spread through the blood or lymphatic system. Treatment is surgical.

Adrenal gland tumors - Most of these tumors are hormone-producing. Treatment is mainly carried out by surgical method.

Carcinoid tumors – Multiple endocrine neoplasms in patients with type I carcinoid syndrome develop in the lungs, thymus (breast gland), and digestive system. Treatment is carried out by surgical removal of the tumor. Depending on the type of tumor, chemotherapy, radiotherapy, hormone therapy and others are performed.

Multiple endocrine neoplasias Type 2A 

The same as Menn 2A-syndrome – during Sieple's syndrome medullary cancer of the thyroid gland, pheochromocytoma, and sometimes diseases of the parathyroid glands develop.

Sipple syndrome is diagnosed if the patient or his close relatives (parents, siblings) have two or more of these symptoms:

Medullary tumor of the thyroid gland, characterized by:

• Swelling of the anterior surface of the neck
• Difficulty swallowing and breathing
• feeling of suffocation).

Pheochromocytoma  - Adrenal medulla tumor, characterized by:

• High blood pressure
• increased pulse rate
• headache
• Sweating
• Dizziness
• Irritability

Parathyroid gland disease (increase in size of parathyroid glands, benign tumor). Its symptoms are:

• Hypercalcemia
• Abdominal and bone pain
• fractures
• Swelling of the anterior surface of the neck
• Difficulty speaking and swallowing.

             Family anamnesis of the mentioned pathologies - Presence of similar tumors in two or more family members.

The prognosis is generally favorable. RET gene mutation is associated with medullary thyroid tumor in Men2A syndrome.

Multiple endocrine neoplasia type 2B 

Patients with multiple endocrine tumors type 2B may have a thin, asthenic build with long limbs. Small tumors in the mucous membrane are often observed. Men2B syndrome is characterized by the following pathologies:

·         Medullary thyroid tumor (rapidly growing, aggressive tumor)

·         Hyperplasia of parathyroid glands

·         Adenoma

·         Pheochromocytoma

·         Nerve cell tumors in mucous membranes and other areas

The prognosis in Men2B syndrome is not very good, because the medullary cancer progresses more aggressively than in Men2A syndrome.

Diagnosis

Diagnosis and treatment depends on the clinical form and stage of the tumor.

Blood biochemistry

Hormone studies

Genetic tests

Study of biochemical and hormonal components in 24-hour urine

Imaging studies: ultrasound, CT, MRI, PET-CT,

"Synevo" offers diagnostic tests for multiple endocrine neoplasias:

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