Multiple Endocrine Neoplasia (MEN) Type 1

Multiple endocrine neoplasia (MEN) is a rare genetic disorder characterized by numerous tumours and/or malignancies of endocrine system glands and tissues. They are of 2 types; namely MEN Type 1, and MEN Type 2.

MEN Type 1 is a hereditary disorder in which numerous tumours impact various components of the endocrine system whereas MEN Type 2 is a hereditary polyglandular cancer condition (many glands).

The most common affected areas in MEN 1 are parathyroid glands (most common), gastroenteropancreatic tract, and pituitary gland. Most MEN type 1 tumours are noncancerous (benign), but some can be cancerous (malignant) and spread to other parts of your body (metastasize).

Signs and Symptoms

MEN type 1 symptoms differ depending on which glands are impacted by tissue overgrowth (hyperplasia) or a tumour and can cause the affected glands to produce excess hormones than the body requires. Symptoms may vary widely from person to person in patients with MEN type 1.

If MEN type 1 is associated with parathyroid gland hyperplasia and tumors, the symptoms include joint pain, muscle weakness, fatigue, depression, trouble concentrating, loss of appetite, nausea and vomiting, confusion and forgetfulness increased thirst, and frequent urination. Constipation, bone pain.

If MEN type 1 is associated with pancreas and duodenum tumors (gastrinomas), the symptoms include confusion, shakiness, sweating, hunger, anxiety, heart palpitations, and temporary vision changes.

Symptoms related to pituitary gland tumors in women include changes in menstruation such as irregular periods (menstruation) or no periods (amenorrhea), infertility, milky discharge from the nipples when not pregnant or breastfeeding, loss of interest in sex. Loss of interest in sex associated with low levels of testosterone, erectile dysfunction (ED), infertility in men.

If the prolactinoma is substantial, headaches, vomiting, and/or nausea, changes in eyesight, such as double vision or diminished peripheral vision, may occur.

Diagnosis

A person is diagnosed with MEN type 1 if they have at least two of the three related endocrine tumours (parathyroid tumour, pituitary tumour, and/or gastroenteropancreatic tumour) or if they have one of the associated tumours along with a family history of MEN type 1. A number of blood tests can identify high levels of particular hormones, which can indicate the presence of certain tumours. Imaging tests such as computed tomography (CT) scans or magnetic resonance imaging(MRI) scans may also help find and diagnose tumors.

Treatment

The treatment of MEN depends entirely on what endocrine glands and organs are affected