This presentation got an brief discussion about the thyroid cancers... based on the SABISTON textbook of surgery 21st edition.., ROBBINS AND COTRAN pathologic basics of disease..., BAILEY & LOVE short practice of surgery 27th edition

1. Dr. NAGARAJAN 1ST YEAR PG..,
Deparment of General Surgery
Government Thiruvannamali
Medical College, Thiruvannamalai

2.  4-10% of thyroid carcinoma
 Originates in the PARAFOLLICULAR CELLS OR C Cells
 Most commonly occur in a sporadic form (80%)
 Less commonly as a AUTOSOMAL DOMINANT inherited disorder such
as MEN2A, MEN2B and familial medullary thyroid carcinoma(FMTC)
 MTC arising in MEN2A usually has a more favorable long term
outcome than MTC arising in MEN2B or sporadic MTC

3.  RET proto oncogene on
CHROMOSOME 10q11.2, encodes
a receptor tyrosine kinase that
binds to glial –derived
neurotrophic factor(GDNF) and
other ligands that transmits
growth and differentiation signal.
 MEN2A as well as MEN2B results
in GERM LINE MUTATIONS which
constitutively activate the RET
receptor

4.  Patients with familial MTC syndromes initially develop C CELL
HYPERPLASIA
(C CELL HYPERPLASIA is a preneoplastic lesion, defined as more than 6 C
cells per follicle or more than 50% per low power field)
 Because of the high penetrance of MTC and early development of C
Cell hyperplasia, family members of MEN2 should be screened at an
early age for RET proto-oncogene
**MEN2B- shortly after birth
**FMTC & MEN2A- before the age of 5

5.  Mass in the neck
 Dysphagia
 Hoarseness
In some instance, the initial manifestation are those of a paraneoplastic
syndrome caused by the secretion of peptide hormone
 Diarrhea- secretion of VIP
 Cushing syndrome – ACTH secretion
SPORADIC CASES

6. Typically has one
of the two
manifestations
A palpable mass
in the thyroid
Elevated
calcitonin level

7.  100% will develop MTC
 50% will have pheochromocytoma
 Diffuse ganglioneuromas of
lips,tongue,eyelids, GI tract
 Marfanoid face
 Megacolon
 Skeletal abnormalities with thick
peripheral nerves
MEN 2B SYNDROME
 MTC seen in 75% of cases
 50% will have pheochromocytoma
 Hyperparathyroidism
 35% will have adrenal medullary
hyperplasia
MEN 2A SYNDROME

8. MICROSCOPICALLY
MACROSCOPICALLY:

9. 1. Ultrasound neck( mass, nodal status)
2. FNAC(amyloid stroma with dispersed malignant cells and C Cell hyperplasia;
calcitonin level of FNAC washout; cytology with immunohistochemistry can also
done)
3. Serum calcitonin level ( normal:<10pg/ml )
4. Serum CEA level (raised in 50% cases of MTC; CEA>30mg/mlincurability of
surgery
CEA>100mg/ml nodal spread
5. CT Abdomen, USG Abdomen, Urinary Metanephrine, Venyl mandelic acid (for
pheochromocytoma)
6. Serum calcium and parathormone( for hyperparathyroidism)
7. CT Neck, CT Chest ( for metastasis)
8. Genetic testing for RET mutations

10.  Surgery is the main therapeutic modality
 MTC can be cured only by complete resection of the primary tumor
and local and regional
 If there is associated phaeochromocytoma, it should be treated
surgically by adrenalectomy first and later only total thyroidectomy is
done
 THYROXINE replacement/maintainence therapy 100mcg in the
morning before food is needed

11. Sporadic MTC Lesion is generally contained within one lobe
MEN2 Lesion involves the upper halves of both lobes
MEN2B RET mutation Prophylactic total thyroidectomy within the first year of life
or at the time of diagnosis
Other germline RET mutations Prophylactic total thyroidectomy before age 5 years or at
the time of diagnosis
<1 year with MEN2B and <5 year with MEN 2A AND
FMTC
Level VI nodal dissection may be omitted
Absence of germline RET mutations, patients with
known or suspected MTC without advanced disease
Prophylactic total thyroidectomy with level VI nodal
dissection
Clinically detectable or ultrasound-detectable
disease in lateral neck
Total thyroidectomy with level VI and lateral compartment
nodal dissection
Distant metastatic disease Less aggressive surgery in the neck
MTC diagnosed post operatively in patient
underwent less than total thyroidectomy
Further operative intervention is needed to complete
theraphy

12.  Serum calcitonin and serum CEA level
 USG/CT NECK
 Recurrence  resurgery  exploration  disease clearance
 PET scan
 Follow up for late onset of pheochromocytoma or hyper
parathyroidism should be assessed regularly

13.  Approximately 1% of all thyroid malignancies
 Undifferentiated tumor of THYROID FOLLICULAR EPITHELIUM
 Most aggressive form of thyroid carcinoma with a disease specific
mortality approaching to 100%
 Patients frequently have a history prior or coexistent differentiated
thyroid carcinoma and upto 50% have a history of a goiter

14.  Can arise de novo, or more
commonly by dedifferentiation of
a well differentiated tumor.
 RAS mutation
 PIK3CA mutations
 TP53 inactivation
 Activating mutations of beta
catenin

15.  MICROSCOPICALLY
1. GIANT CELLS
2. SPINDLE CELLS
3. MIXED GIANT & SPINDLE CELLS
4. EXPRESS EPITHELIAL MARKERS
cytokeratin
5. Foci of papillary or follicular
differentiation, suggesting its
origin from well differentiated
tumor

16.  Elderly females
 A rapidly and progressive growing swelling in the thyroid region
 Stridor and hoarseness of voice (tracheal obstruction)
 Dysphagia
 Fixed to skin
 Swelling is hard
 POSITIVE BERRY’S SIGN: involvement of carotid sheath leads to
absence of carotid pulsation

17.  INVESTIGATIONS
1. FNAC
2. CT NECK
3. MRI NECK
4. PET SCANS

18.  In 90% of patients there will be distant spread at the time of diagnosis
 Tracheostomy has got a role to relieve respiratory obstruction .
 External beam radiotherapy and adjunctive chemotherapy adds little
to prognosis.
 Prognosis is so grim in this disease, end of life planning and
consideration of palliation must be part of very early management and
counseling of the Patients

19.  Primary thyroid lymphoma ,although rare, is being recognized more
frequently
 Approximately half of thyroid lymphomas occur in the setting of pre
existing Hashimoto thyroiditis
 Almost non Hodgkin lymphoma (B Cell origin)
 The diagnosis is considered in patients with a goiter, especially one
that has grown significantly in a shorter period

20.  ULTRASOUND NECK ( pseudocystic
pattern)
 FNAC
 Core needle or open biopsy ( if FNAC is
non diagnostic)
 CT NECK,CHEST,ABDOMEN(
extrathyroidal disease)
 PET
INVESTIGATIONS
1. Swelling in a short duration
2. Hoarseness
3. Dysphagia
4. Fever
5. Diffuse pain
6. O/E : firm, slightly tender,fixed mass
frequently with substernal extension
CLINICAL FEATURES

21.  CHEMOTHERAPYCHOP REGIMEN (cyclophosphamide,
hydroxydaunomycin, oncovin, prednisolone)
 Surgical resection near or total thyroidectomy (MALT LYMPHOMA)
mostly not preferred due to pericapsular edema and loss of normal
plane and most cases reserved for diagnostic biopsies.
 MALT LYMPHOMA 5 year survival 100%
 LARGE CELL&MIXED CELL 5 year survival 71% & 78%