Multiple Endocrine Neoplasia (MEN) Flashcards

1
Q

Type 1 MEN

A

primary hyperparathyroidism (>90%),

pancreatic (enteropancreatic tumors) (60-70%),

can have gastrinomas pituitary tumors (10-20%)

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2
Q

Type 2 A MEN

A

Medullary Thyroid Cancer (>90%)

Pheochromocytoma (40-50%)

Parathyroid hyperplasia (10-20%)

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3
Q

Type 2 B MEN

A

Medullary Thyroid cancer,

pheochromocytoma,

OTHER: mucosal intestinal neuromas and marfanoid habitus

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4
Q

If diagnosed with medullary thyroid cancer need to evaluate for:

A

metastatic dx, coexisting tumors identify possible germline RET mutations

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5
Q

Where does medullary carcinoma spread to?

A

local lymph nodes or cause metastasis

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6
Q

What lab can you follow w/ medullary cancer?

A

calcitonin amount of calcitonin correlates with dx activity

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7
Q

When to get staging CT of neck, chest and upper abdomen for medullary cancer?

A

when calcitonin is >400 or there’s local spread to lymph nodes

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8
Q

When to order triphasic bone scan for medullary cancer?

A

when there’s suspected skeletal metastasis

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9
Q

Who gets RET mutation testing?

A

anyone with newly diagnosed medullary thyroid cancer. helps with prognosis and all three MEN have autosomal dominant inheritance and so RET testing helps to determine if relatives need to be tested too.

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10
Q

Where do we see RAS mutations

A

follicular thyroid cancers, and follicular varient papillary thyroid cancers

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11
Q

which cancer makes thyroglobulin?

A

it’s an excellent tumor marker for differentiated epithelial thyroid cancer.

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12
Q

what do we do prevent reoccurrence of differentiated thyroid cancer (high risk papillary cancer) in post thyroidectomy pts?

A

give levothyroxine to suppress TSH stimulation - low normal for people with complete thyroidectomy to prevent reoccurrence. w/ incomplete resection of primary tumor or known metastasis aim for aggressive TSH suppression (keep TSH <0.1 mU/L)

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13
Q

when trying to suppress TSH stimulation in post thyroidectomy thyroid cancer patients what are some of the side effects?

A

Creates a slight hyperthyroid state (to keep TSH low) - results in risk for HTN, afib, accelerated bone loss and puts patients as risk for osteoporosis.

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14
Q

how to prevent osteoporosis from accelerated bone loss from hyperthryoidism

A

adequate calcium and vitamin D through helath diet and oral supplementation. May also need bisphosphonate if necessary.

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15
Q

necrolytic migratory erythema is a

A

bronze colored blistering rash involved in the mucous membranes that is classic for glucagoma seen for MEN1 starts as pruritic painful erythematous papules and plaques that coalesce and enlarged after 7-14 days then develop central clearing with bronze colored blisters and crusting commonly found on mucous membranes. Glossitis, angular chelitis, stomatitis, blepharitis with subsequent hair and nail loss can be confused for other conditions.

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16
Q

Wermer syndrome is also known as

A

MEN1

17
Q

autonomal dominant disease seen with only adenomas (pituitary, parathyroid glands and pancreas)

A

MEN1

18
Q

glucagonoma is a rare

A

functional neuro-endocrine tumor of the pancreas within the alpha cells responsible for making glucagon.

19
Q

when a medullary thyroid cancer is diagnosed they need to be screened for MEN2 with

A

calcium levels and plasma fractionated metanephrines. Try to screen for pheochromocytoma and parathyroid hyperplasia

20
Q

when you start phenoxybenzamine for a pheochromocytoma before surgery?

A

10-14 days before surgery

titrate to intolerable orthostasis and nasal stuffiness.

need to be eating a high sodium diet as pts will become volume depleted and prone to orthostasis.

Beta blocker is added after maximum alpha blockade is acheived for a BB >70-80 heart rates.