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Medullary Carcinoma Thyroid

Medullary carcinoma of the thyroid (MTC) arises from parafollicular C-cells and produces calcitonin. MTC does not concentrate iodine, making radioiodine therapy ineffective. Approximately 25% of MTC cases are hereditary and associated with familial MEN syndromes caused by RET gene mutations. Surgical resection with total thyroidectomy and lymph node dissection is the primary treatment, with postoperative monitoring of calcitonin and CEA levels to detect recurrence. Targeted kinase inhibitors have shown promise for treating metastatic MTC, but effective systemic therapies remain limited.

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Praveen Kumar Dvm
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48 views17 pages

Medullary Carcinoma Thyroid

Medullary carcinoma of the thyroid (MTC) arises from parafollicular C-cells and produces calcitonin. MTC does not concentrate iodine, making radioiodine therapy ineffective. Approximately 25% of MTC cases are hereditary and associated with familial MEN syndromes caused by RET gene mutations. Surgical resection with total thyroidectomy and lymph node dissection is the primary treatment, with postoperative monitoring of calcitonin and CEA levels to detect recurrence. Targeted kinase inhibitors have shown promise for treating metastatic MTC, but effective systemic therapies remain limited.

Uploaded by

Praveen Kumar Dvm
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© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Medullary carcinoma thyroid

Praveen kumar d v m
MTC
MTC does not arises from follicular epithelial cells, but from the parafollicular C
cells, which are neural crest derieved cells whose function is to produce calcitonin.

Multofocal and bilateral MTCs are usually seen in patients with MEN, but,
otherwise, familial and sporadic MTCs are indistinguishable.

Grossly, well circumscribed and nonencapsulated.


MTC
Microscopically, different appearances.

Solid growth or nests are most common pattern.

amyloid -80% of the cases.

calcitonin -specific for MTC, 20% may not stain for calcitonin.

Chromogranin may be useful.


ABILITY TO CONCENTRATE IODINE

MTC never concentrate iodine to a degree that is


curative.
FAMILIAL MTC
25% of cases are hereditary.

All pts should be screened for familial disease

Genetic counselling is the standard of care for all first degree relatives.

Associated with familial MEN syndromes

Autosomal dominant fashion

Germline point mutation in RET gene on chr 10q11.2

Prophylactic thyroidectomies.
CLINICAL MANIFESTATIONS
Asymptomatic

Hoarseness of voice

Airway compromise, dysphagia, respiratory symptoms

Pain and weight loss

Metastatic cervical lymphadenopathy appears in about 50% of patients at initial


presentation.
Diagnostic evaluation
Serum calcitonin and carcino embryonic antigen.

Neck US with Doppler and US-FNA

Advantages of US-FNA

1) Minimally invasive and outpatient procedure


2) Visually verify that biopsy needle is in the nodule
3) Evaluation of non palpable tumours, and in cystic lesions, lowers the rate of
inadequate specimens.
Diagnostic evaluation
Cervical ultrasound

CT AND MRI

MRI indicated in the presence of hoarseness, stridor, dysphagia or other clinical


signs of locally extensive thyroid cancer that cannot be adequately assessed by
ultrasound.

Before surgery, coexisting pheochromocytoma should be diagnosed and


addressed to avoid hypertensive crisis during surgery.
Outcome and prognostic factors
Age

Positive surgical margins

Number of positive lymph nodes

Extranodal extension

Distant metastasis
Surgical management
Total thyroidectomy

Complications

1) recurrent laryngeal nerve injury(temporary 30% and permanent 2%)

2) hypoparathyroidism(temporary 5% and permanent 0.5%)

Central neck dissection should be performed in all cases of MTC.

Compartment oriented lateral neck dissection is indicated when clinically involved.


Post surgery

Post operative levothyroxine is indicated for all patients.


However, TSH suppression is not appropriate because c cells
lack TSH receptors. Thus, TSH should be kept in the normal
range by adjusting the levothyroxine dose.
MTC Management
No role of adjuvant I-131 therapy.

Serum calcitonin-sensitive and specific marker for the extent of residual disease.

Few studies on the role of EBRT.

Can be considered for grossly incomplete resection when additional attempts at


surgical resection have been ruled out.

Palliate painful or progressing bone metastasis.


Systemic therapy
Cytotoxic systemic therapies have been ineffective in the management of
metastatic or recurrent MTC.

Recent advances in therapies targeting RET-tyrosine kinase receptors have


shown promising pre clinical and early clinical trials.
Persistently increasing calcitonin

Calcitonin and CEA Should be measured 2 or 3 months postoperatively.

Detectable basal calcitonin or elevated CEA Level-neck USG.


Recurrent or persistent disease

1) EBRT/IMRT
2) Vandetanib
3) Cabozantinib

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