Prognosis

Neuroendocrine tumors can recur as new clonal tumors, meaning lifelong monitoring is required even for patients who have undergone successful surgery.

MEN1

Death is largely due to malignancies that develop within the pancreas and duodenum (e.g., gastrinomas, malignant insulinomas). Significant morbidity is related to excess hormone production from all types of syndromal tumors. However, with the exception of increased insulin secretion, hormone overproduction can generally be well managed medically in those patients for whom a surgical cure is not possible.

Surgery on the parathyroid glands, duodenum, pancreas, or pituitary does not remove all susceptible tissue, meaning recurrence is possible. The recurrence rate for hyperparathyroidism is up to 50% at 10 years, even with total parathyroidectomy.[94]

MEN2

Patient prognosis is tumor specific. MEN2B generally has a worse prognosis because tumors are more aggressive, metastasize earlier, and grow more rapidly.

Medullary thyroid cancer prognosis depends on disease staging at first operation and the extent of initial surgery. In many cases, medullary thyroid cancer is slow growing, and 10-year survival rates approach 70%, even in patients with persistent disease.[95] However, many patients do well for many years with substantial tumor burden. Medullary thyroid cancer can be particularly aggressive in MEN2B. Evidence suggests that medullary thyroid cancer mortality has decreased to <5% and patients identified by carrier status may be cured by prophylactic thyroidectomy and that in general mortality may be improving over time.[86][96]

Pheochromocytoma prognosis has also improved in recent years due to earlier lesion detection with imaging and biochemical screening.[12] However, adrenal insufficiency following bilateral adrenalectomy can lead to death due to adrenal crisis.

Surgery on the parathyroid glands, duodenum, pancreas, or pituitary does not remove all susceptible tissue, meaning recurrence is possible. The recurrence rate for hyperparathyroidism is up to 50% at 10 years, even with total parathyroidectomy.[94]

Surgical complications such as hypoparathyroidism or laryngeal nerve damage can cause infrequent morbidity but are rarely a cause of death.

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