Multiple endocrine neoplasia (MEN) and endocrine tumors
What is it?
Multiple Endocrine Neoplasia (Multiple Endocrine Neoplasia, MEN) is a rare syndrome, often hereditary, characterized by tumors (benign or malignant) in several endocrine glands, called endocrine tumors (Neuro-Endocrine Tumors, NET).
Endocrine tumors can occur not only as multiple endocrine neoplasms, but also as isolated tumors of any kind, in so-called sporadic forms. The most common endocrine tumors are:
- pancreas and gastrointestinal tract: insulinoma, gastrinoma, glucagonoma, VIPoma, PPoma, carcinoids, and non-secreting tumors;
- thyroid gland: medullary thyroid cancer;
- parathyroid glands: adenoma/hyperplasia of parathyroid glands;
- thymus and respiratory tract: thymic, bronchial, pulmonary carcinoid;
- pituitary gland: non-secreting pituitary adenoma, prolactinoma, GGoma, ACTHoma, TSGoma
- adrenal glands: adenomas, adrenal carcinomas, pheochromocytoma;
- adipose tissue: lipomas, liposarcomas;
- nerve tissue: neurinomas.
How is it diagnosed?
Suggested exams
How is it treated?
In forms with a genetic predisposition, there is a 50 percent risk that the affected parent will pass the disease to his or her offspring in every pregnancy.
This implies the need of:
- early diagnostics;
- testing family members with endocrinology tests to detect the presence of asymptomatic hyperplasia/neoplasia and genetic tests to identify carriers of the diseased gene;
- diagnostic and therapeutic monitoring.
A major difficulty in the clinical management of patients with multiple endocrine neoplasms and endocrine tumors is identifying symptoms that are often not present or when patients are carriers of rare clinical syndromes and therefore cannot be easily identified.
T.E.A.M., a group for the study and treatment of endocrine tumors using a multidisciplinary approach, has been established at the San Raffaele Hospital. Its goal is to ensure that the patient has a definite and effective diagnostic and therapeutic pathway.
The second problem is the need to monitor the clinical situation of family members who may be carriers of these diseases or genetic mutations. To this end, the Oncologic Endocrinology/Endocrine Tumor Clinic/MEN Outpatient Clinic led by T.E.A.M. has been established to provide a program of diagnostic and therapeutic care for both patients undergoing treatment and their families.
Where do we treat it?
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