Sipple and Steiner described the association of thyroid cancer with pheochromocytoma and hyperparathyroidism, respectively
Bailey says MTC combined with phaeochromocytoma alone is called Sipple’s syndrome (page 856)
MEN2A is characterized by MTC, pheochromocytoma (50%) and hyperparathyroidism (25%).
Associated with mutations in codon 634 in the RET proto-oncogene.
Wermer- MEN 1 is characterised by the triad of tumours in the anterior pituitary gland, mostly presenting as prolactinomas or non-functioning tumours, hyperplasia of the parathyroids causing primary hyperparathyroidism (pHPT) and pancreaticoduodenal endocrine tumours (PETs)
Patients with MEN 2B do not develop pHPT
Q2.) Male patient comes with Gynecomastia, Blindness and nipple discharge? Which hormone level will you do
a) Testosterone
b) Estrogen
c) Prolactin
d) BHCG
Ans 2) c HIgh Prolactin due to Prolactinoma
Women with prolactinomas may present with amenorrhea and/or galactorrhea, whereas men with prolactinomas may present with sexual dysfunction and/or gynecomastia. A serum prolactin level >200 ng/mL and an associated adenoma on MRI confirm the diagnosis of prolactinoma.
symptoms of local compression such as headache, visual field deficits, hypopituitarism, temporal lobe epilepsy, mild hyperprolactinemia from stalk compression, and cranial nerve III or VI dysfunction
Q3) Which of the following is not true for Pheochromocytoma
a) 10% bilateral
b) Extra adrenal cases are more malignant
c) Arises from adrenal cortex
d) Patient may be normotensive
Ans c
Neuroendocrine tumour of the chromaffin cells of the adrenal medulla.
Hypertension and hyperglycaemia are often found
10% of cases are bilateral. 10% occur in children. 11% are malignant (higher when tumour is located outside the adrenal).
10% will not be hypertensive.
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