Internal Medicine

0521779407-14 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:16

1010 Multiple Endocrine Neoplasia 1

differential diagnosis

■Straightforward if hyperparathyroidism and family history of MEN1

■Can see familial hyperparathyroidism and overlap syndromes

■Hypercalcemia from isolated hyperparathyroidism may elevate

serum gastrin levels

management

What to Do First

■Detailed medical and family history (identify index case: important

for screening)

General Measures

■History and physical exam dictate biochemical testing

■Measure calcium, gastrin, prolactin

■Significant morbidity/mortality from malignant islet cell tumors:

maintain high index of suspicion

specific therapy

■Hyperparathyroidism: for calcium >11 mg/dL, or end-organ damage

(kidney stones, osteitis, osteoporosis, renal failure, hypercalciuria),

surgical removal of 3 1/2 glands with tagging or transplant of remain-

ing tissue; hyperplasia of all glands, can have more than 4 glands

■Islet cell tumor: proton pump inhibitors for gastrinoma, surgi-

cal excision, including partial pancreatectomy (multifocal tumors),

chemotherapy if malignant (streptozotocin, somatostatin, others)

■Pituitary tumors: bromocriptine, cabergoline (prolactinoma), trans-

phenoidal resection, somatostatin (acromegaly)

Side Effects

■Parathyroidectomy: hypoparathyroidism, recurrent laryngeal nerve

injury

■Proton pump inhibitors: no increased risk of carcinoid

■Pancreatectomy: high morbidity, diabetes, pancreatic insufficiency

■Streptozotocin: diabetes

■Somatostatin: hyperglycemia, gallstones

■Bromocriptine, cabergoline: nausea, orthostatic hypotension.

■Transphenoidal resection: hypopituitarism, diabetes insipidus, CSF

leak, infection

follow-up

During Treatment

■Assess response to interventions: