Is Multiple Endocrine Neoplasia Type 1 Cancer?

MEN type 1 syndrome (also called Wermer syndrome) is a rare, hereditary disorder of the endocrine system. They are responsible for developing benign or cancerous tumors in the endocrine glands, which include:

• Parathyroid glands
• Islet cells of the pancreas
• Pituitary gland

Some of the endocrine and nonendocrine tumors associated with MEN type 1 include:

• Adrenal cortical tumors
• Neuroendocrine tumors
• Pheochromocytomas
• Facial angiofibroma (tumor of blood vessels and fibrous tissue)
• Collagenoma (flesh-colored tumor on the skin)
• Lipoma (fatty tumor)
• Leiomyoma (smooth muscle tumor)
• Meningioma (tumor from nervous system tissue)
• Ependymoma (tumor from nervous system tissue)

Symptoms are usually caused by excess hormone production in the body.

Signs and symptoms of MEN type 1 could be due to hyperparathyroidism and include:

• Cognitive changes, such as depression or forgetfulness
• Bone symptoms, including pain, tenderness, or bone thinning
• Feeling ill, including fatigue or weakness
• Gastrointestinal symptoms, such as nausea or vomiting
• Urinary symptoms, such as increased urinary frequency, higher amount of urine, or kidney stones

The MEN1 gene is can be passed from one generation to the next in a family. MEN1 gene is associated with MEN type 1 tumor. A mutation in the MEN1 gene can increase the risk of endocrine tumors and other symptoms of MEN type 1.

It is an autosomal dominant disorder; only one parent with a mutated gene can pass the gene to their children. If one parent has mutated the MEN1 gene, there is a 50 percent chance that the child will have the disorder.

The treatment may vary depending on the tumor's location; different sites can present with different symptoms. 

Treatment options for MEN type 1 may include:

• Pituitary tumors: Often treated with surgery or drug therapy. Rarely, radiation therapy could be used.
• Hyperparathyroidism: Surgery is used to remove most of the parathyroid gland to suppress excess parathyroid hormone. If surgery does not solve the issue, the physician may prescribe medicines to control calcium levels.
• Neuroendocrine tumors: Usually found in the pancreas or duodenum (small intestine). Treatment could be based on the type and stage of the tumor.
• Hypoglycemic syndrome: Tumors produce excessive insulin, leading to insulinomas. Insulinomas can lead to extremely low glucose levels, which can be life-threatening. The physician may recommend surgery to remove a portion of the pancreas.
• Zollinger-Ellison syndrome: Can cause tumors that oversecretes gastric acid, causing ulcers and diarrhea. Physicians may prescribe medication or surgery as treatments.
• Other pancreatic neuroendocrine tumors: At times, these tumors may secrete other hormones that can lead to various health issues. Treatment options for these types of tumors may include:
  • Medication
  • Surgery
  • An ablation procedure
  • An ablation procedure destroys maximum abnormal tissue at the tumor site
• Metastatic neuroendocrine tumors: If tumors have spread to lymph nodes or the liver, the physician may use surgery to remove them. Surgery options for these types of tumors include:
  • Liver surgery
  • Radiofrequency ablation: High-frequency energy passes through a needle, heats the nearby tissue, and kills these cells.
  • Cryoablation: Freezes the tumors.
  • Chemoembolization: Injecting strong chemotherapy drugs directly into the liver to destroy the tumor.
  • Chemotherapy or hormone-based treatments: When surgery does not eliminate the tumor, physicians may use these.
• Adrenal tumors: Physicians usually monitor these tumors rather than treat them. If the tumor secretes hormones and is large enough to be considered cancerous, they suggest removing them using minimally invasive surgery.
• Carcinoid tumors: Develop in the lungs, thymus glands, and digestive tract. Surgery is usually recommended when the tumor has not spread to other areas. Some treatment options to destroy carcinoid tumors include:
  • Chemotherapy
  • Radiation therapy
  • Hormone-based therapy for advanced tumors