0521779407-14 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:16
Multiple Endocrine Neoplasia 1 1011➣Parathyroidectomy: 75–90% of subjects with initial response
many recur; follow calcium for hypoparathyroidism shortly after
surgery, hungry bone syndrome (hypocalcemia) immediately
postop; recurrent hypercalcemia
➣Islet cell tumor: symptoms and hormone levels for response to
intervention (surgery, medical)
➣Pituitary tumor:
Prolactinoma: monitor prolactin and tumor size
Acromegaly: monitor GH and IGF-1 levels; GH target <1.0
mcg/L after oral glucose challenge (surgery or somatostatin);
IGF-1 levels should be in normal age-and sex-adjusted range
Surgery: short-term monitor for posterior and anterior pitu-
itary hypofunction (esp pituitary/adrenal and pituitary/
thyroid axis)Routine
■Monitor recurrence by symptomatology, lab screening, and imaging
■Screen for development of other tumors yearly:
➣Pancreatic tumors in 30–80% (gastrin or other hormone, depend-
ing on symptoms and signs)
➣Pituitary tumors in 15–90% (prolactin or other)complications and prognosis
■Most serious: malignant islet cell tumor; most gastrinomas
malignant; 10% of insulinomas malignant
■Due to hormone overproduction syndromes and therapies:
➣Hyperparathyroidism: kidney stones, renal failure, osteitis,
osteoporosis
➣Pancreatic tumor:
Gastrinoma: luminal perforation, hemorrhage
Insulinoma: hypoglycemia with neuoglycopenic symptoms
including seizures
➣Pituitary tumor: mass effect, optic chiasm compression, hypopi-
tuitarism
Prolactinoma: hypogonadism, galactorrhea
Acromegaly: osteoarthritis, sleep apnea, cardiomyopathy, glu-
cose intolerance, colonic polyps and cancer, increased mor-
tality
Cushing disease: increased risk of infection, osteoporosis,
hypogonadism, hypertension, diabetes mellitus