Corticotropin Producing Tumor

Cushing's syndrome associated with a pancreatic neuroendocrine tumor that secretes corticotropin usually occurs in patients with neuroendocrine tumors that secrete another peptide and cause another syndrome.55 Malignant neuroendocrine tumors commonly secrete more than one peptide. Excessive production of corticotropin by a pituitary tumor may occur in patients with MEN 1 but is usually mild and clinically insignificant.55 Cushing's syndrome has also been reported in 5% of patients with Zollinger-Ellison syndrome.55 In these patients, Cushing's syndrome is severe, and they usually have metastatic disease. Cushing's syndrome is due

ItaBLE 84-6. Syndrome, Location, Malignant Potential, and Incidence of MEN 1 in Very Rare Pancreatic

1 Endocrine Tumors

Located in

Located in

Pancreas

Duodenum

Malignant

Tumor

Syndrome (%)

(%)

(%)

MEN 1 <%)

GRFoma

Acromegaly 90

10

30

30

Corticotropin-producing tumor

Cushing's syndrome 100

100

Nonfunctioning

None 100

60

50

MEN 1 = multiple endocrine neoplasia type 1 ; GRF - growth hormone-releasing factor.

to ectopic production of corticotropin by the neuroendocrine tumor.69 These patients seldom respond to chemotherapy and have a poor prognosis. In our experience, patients with corticotropin-producing pancreatic neuroendocrine tumors are usually unable to be rendered surgically free from disease. Medical management of the hypercortisolism in these patients is usually inadequate. Therefore, either debulking surgery or bilateral adrenalectomy may be indicated to control the severe signs and symptoms of hypercortisolism.69

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