Multiple endocrine neoplasia

One of several inherited syndromes caused by a defect in tumor suppressor genes that produces benign and malignant tumors of many endocrine glands. Angiofibromas and collagenomas of the skin also are common findings. This group of diseases has been classed according to the glands affected. In MEN type I (MEN I), there are tumors of the parathyroid, pituitary, and islet cells of the pancreas.


The presence of anomalous tissue growth on one or more of the endocrine glands, responsible for hormone secretion, characterizes this condition.


The term used to describe a cluster of rare, autosomal dominant genetic conditions that lead to the growth of tumors in multiple endocrine glands. Each of these disorders results from a distinct genetic abnormality. In one manifestation of these Multiple Endocrine Neoplasia (MEN) disorders, tumors emerge in the pancreas, parathyroid glands, pituitary gland, and sometimes in the adrenal and thyroid glands. Another less prevalent form gives rise to tumors in the adrenal, thyroid, and parathyroid glands. Tumors might manifest concurrently or at separate intervals spanning several years. The affected glands might excessively produce hormones. While a type of thyroid tumor that secretes the hormone calcitonin is often malignant, the majority of tumors in other endocrine glands are not cancerous.


Tumors are typically extracted through surgical procedures, followed by post-operative monitoring to identify any subsequent endocrine irregularities. Relatives of the individual might also be provided with the option of undergoing screening for the abnormal gene or potential impacts of the disorder.


 


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