32-year-old female complains of milky discharge from her nipples and pain with urination. Upon further questioning, she notes that she has not had a period in the past 3 months. Other than white discharge from her nipples, her physical exam is unremarkable. Her pregnancy test is negative. Her urine analysis shows microscopic blood without evidence of nitrites, leukocyte esterase, RBC casts, or WBC casts. A renal ultrasound shows a dilated left ureter. Labs are sent and are as follows:WBC: 11,500Hgb: 13.5Platelets: 250,000Ca++: 12.5Phosphorus: 2.0 What other medical condition is often associated with the one most likely found in this patient?
Addison’s disease
Diabetes mellitus type II
Gastrinoma
Medullary carcinoma of the thyroid
Pheochromocytoma
Correct answer
Gastrinoma
Feedback
orrect answer: C
This patient is mostly likely suffering from the syndrome of multiple endocrine neoplasia type I (MEN I) which typically affects the pituitary (here, prolactinoma causing galactorrhea and amenorrhea), parathyroid (here, hyperparathyroidism causing kidney stones seen by increased calcium, low phosphrus, and left hydroureter), and pancreatic neoplasms(gastrinoma, option C). MEN I have an autosomal dominant inheritance, so it is important to find out about family history.
Medullary carcinoma of the thyroid (option D) and pheochromocytoma (option E) are manifestations of MEN II and MEN III, both of which are autosomal dominant inherited. MEN II also is associated with parathyroid tumors, and MEN II is associated with mucosal neuromas.
Addison’s disease (option A) is caused by adrenal atrophy, and affects all 3 layers of adrenal production (glucocorticoids, mineralocorticoids, and androgens). Patients present with hypotension, and skin hyperpigmentation (due to melanocyte stimulating hormone, a by product of ACTH production). Treatment includes both glucocorticoids and mineralocorticoids.
Diabetes mellitus type II (option B) is caused by insulin resistance. Although it is a genetic disease that tends to run in families, it is not part of the MEN I syndrome.