Important cancers Flashcards
Inheritance pattern of MEN
autosomal dominant
What glands are implicated in MEN and what adaptation do they undergo
Hyperplasia of several endocrine glands
List the 3 variants of MEN
Men I
Men II or IIa
Men IIb or III
What glands are implicated in MEN I
Parathyroid
Pancreatic
Pituitary
What glands are implicated in MEN II
Pheo/ andreal medullary hyperplasia
Parathyroid hyperplasia
Thyroid - Medullar cancer and C - cell hyperplasia
What are the metabolic problems associated with MEN (5)
Zollinger-Ellison syndrome, hyperinsulinism, pheochromocytoma, Cushing’s syndrome,
hyperparathyroidism
What glands are implicated in MEN IIb or III
Pheo
Medullay thyroid hyperplasia
Medullar Ca and C cell hyperplasia of the thyroid
Mucosal neruomas
Marafanoid features
What life threatening cancers are associated with MEN
Medullary thyroid
Pancreatic
2 life threatening sequelae of MEN
- Hypertension
- Relentless peptic ulcer syndrome
Testing for MEN (what to use and what not to use
To use
- Urinary catecholamine estimation
- Plasma calcitonin
Not
- Plasma calcium estimation
- Not serum ionized calcium/phosphate ratio
What cells is medullary carcinoma of the thyroid derived from, what do they secrete
Parafollicular cells or C cells
Secrete calcitonin
- may also secrete serotonin and prostaglandin
- sometimes carcinoid, rarely ACTH
Are MEN genetic
arise sporadically 80% of cases, the other 20% from genetic associations (MEN IIa & b)
What gene is implicated in medullary thyroid carcinoma (when due to MEN)
germ line mutations in the RET proto-oncogenes
What is the stroma of medullar carcinoma of the thyroid rich in
Amyloid
Medullary thyroid carcinoma slow or fast growing
slow
Biochemically what will people with medullary thyroid carcinoma have or not have
Will have high circulating calcitonin
WONT have
- Hypocalcemia
- Hypercalcemia
- Hypothyroidism
What is the most common thyroid cancer in adults and children
Papillary carcinoma of the thyroid
What is papillary thyroid carcinoma strongly assocaited with
Radiation - especially in childhood
How do people with papillary thyroid cancer often present
With nodal metases (50% at time of diagnosis)
What is the prognosis of papillary thyroid cancer
90% in 20 yrs > the best survival with thyroid neoplasia
What dystrophic calcification is papillary thyroid cancer a/w
pasmmoma bodies
What is the growth pattern of papillary thyroid cancer
papillary (often plus follicular) growth pattern > but co-existing with follicular DOES NOT worsen prognosis
What hormone is papillary thyroid cancer dependent on and what implications does this have
TSH dependent > implications for therapy, relapse during pregnancy, etc…
Where do carcinoid tumours arise from (structurally and what type of cell)
GIT (including pancreas),
Also bronchi
Thymus
Occasionally ovary
Neuroendocrine cells