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58

NCCN Guidelines for Patients

®

:

Thyroid Cancer, Version 1.2017

6

Treatment guide

Medullary thyroid cancer

Guide 20. Primary treatment for inherited medullary

Mutation Tests Primary treatment

MEN 2A/

Familial

medullary

thyroid

cancer

• Calcitonin

• CEA

• Neck ultrasound

(if not done before)

• Screen for

pheochromocytoma

(tumor in adrenal

glands)

• Calcium with

or without PTH

(

p

ara

t

hyroid

h

ormone) blood

test

ª

Measure calcium with

or without PTH:

• No primary

hyperparathyroidism

ª

• Total thyroidectomy

• Consider neck dissection

(same or both sides) if high

calcitonin or abnormal CEA

or ultrasound results

• Consider preventative neck

dissection

• Levothyroxine (after

surgery) to normalize TSH

ª

Measure calcium with

or without PTH:

• Primary

hyperparathyroidism

ª

• See treatment in box above

• During surgery:

• Explore parathyroid

to remove disease,

autotransplant of

parathyroid tissue in

muscle (usually arm), or

leave tissue the size of

one parathyroid gland

• Consider

cryopreservation

(freezing) of parathyroid

tissue

MEN 2B

• Calcitonin

• CEA

• Neck ultrasound

(if not done before)

• Screen for

pheochromocytoma

(tumor of adrenal

glands)

• Calcium with

or without PTH

(

p

ara

t

hyroid

h

ormone) blood

test

ª

• Total thyroidectomy

• Consider central neck

dissection (both sides or

tumors larger than 0.5

cm in diameter)

• Consider radiation

therapy (EBRT/IMRT)

after surgery (adjuvant

therapy - rarely

recommended)

• Levothyroxine (after

surgery) to normalize

TSH