Prognosis and Treatment
Without treatment, persons with MEN2B die prematurely. Details are lacking, owing to the absence of formal studies, but it is generally assumed that death in the 30s is typical unless prophylactic thyroidectomy and surveillance for pheochromocytoma are performed (see below). The range is quite variable, however: death early in childhood can occur, and it is noteworthy that a few untreated persons have been diagnosed in their 50s. Recently, a larger experience with the disease "suggests that the prognosis in an individual patient may better than previously considered."
Thyroidectomy is the mainstay of treatment, and should be performed without delay as soon as a diagnosis of MEN2B is made, even if no malignancy is detectable in the thyroid. Without thyroidectomy, almost all patients with MEN2B develop medullary thyroid cancer, in a more aggressive form than MEN 2A. The ideal age for surgery is 4 years old or younger, since cancer may metastasize before age 10.
Pheochromocytoma - cancer of the adrenal glands - is also present in 50% of cases. Affected individuals are encouraged to get yearly screenings for thyroid and adrenal cancer.
Because prophylactic thyroidectomy improves survival, blood relatives of a person with MEN2B should be evaluated for MEN2B, even if lacking the typical signs and symptoms of the disorder.
The mucosal neuromas of this syndrome are asymptomatic and self limiting, and present no problem requiring treatment. They may, however, be surgically removed for aesthetic purposes or if they are being constantly traumatized.
Read more about this topic: Multiple Endocrine Neoplasia Type 2b
Famous quotes containing the word treatment:
“A regular council was held with the Indians, who had come in on their ponies, and speeches were made on both sides through an interpreter, quite in the described mode,—the Indians, as usual, having the advantage in point of truth and earnestness, and therefore of eloquence. The most prominent chief was named Little Crow. They were quite dissatisfied with the white man’s treatment of them, and probably have reason to be so.”
—Henry David Thoreau (1817–1862)