Anterior neck swelling- 2 years

Lt L 3,4 LNs, hard fixed, non tender

USG Neck: B/L Lymphadenopathy
FNAC: s/o neuroendocrine neoplasm
Case 4, 13/ M
Clinical Presentation
FNAC: s/o neuroendocrine neoplasm
CECT Neck
Lobulated supr mediastinum mass 7.2x5x3.8 cm
Hypodense, calcifications, Encasing Left CCA
Multiple left cervical LNs L 2,3,4; extending into Lt
neck, infiltrating left thyroid
Serum calcitonin: >1100 pg/ml
Serum CEA: 43.19 ug/L
RET Proto RET Proto--oncogene: oncogene: Codon Codon 804 / 804 / Exon Exon 14 mutation 14 mutation
(FMTC) (FMTC)
Case 4, 13/ M
Investigative Work-up
(FMTC) (FMTC)
S- calcium, iPTH, 24h Uri-Metanephrine all WNL
DMSA-V scan: DMSA avid tumor neck & mediast.
USG abd: normal
IDL : Left VC Palsy
Doppler neck: Lt IJV thrombus, narrowing of Lt CCA
Case 4, 13/ M
Investigative Work-up. Contd
Case 4, 13/ M
Investigative Work-up . Contd
Left level 3,4 LN formed a conglomerate mass with the left lobe, invading the
Prevertebral fascia, IJV & Carotid. Rt lobe 4x2x1.5 cm
Debulking of the tumor done with considerable residual tissue in the left
paratracheal gutter and level 4 region
Case 4, 13/ M
Operative Management
Near total thyroidectomy (radical debulking)
with gross residual disease along pre-vertebral fascia
Left L 2,3,4 LN mass excision+ trans-sternal mediastinal dissection
paratracheal gutter and level 4 region
FNAC and Histopathology for
discussion discussion
CYTOLOGICAL DIAGNOSIS
NEUROENDOCRINE NEOPLASM
ADVICE HISTOPATHOLOGICAL EXAMINATION
GROSS EXAMINATION
TOTAL THYROIDECTOMY SPECIMEN
Conglomerate mass of thyroid and lymphnodes
Right lobe 3x2x1cm
Left lobe -5x4x2.5cm
Left lobe cut surface showed growth replacing most of the left lobe
Right lobe and isthmus unremarkable
Six lymphnodes identified in fat attached to left lobe largest 2cm. Six lymphnodes identified in fat attached to left lobe largest 2cm.
Right lobe
Left lobe
LYMPHATIC EMBOLI
NERVE
SKELETAL MUSCLE INFILTRATION
LYMPHNODE METASTASIS
THYMIC INFILTRATION
CONGO RED CONGO RED
APPLE GREEN BIREFRINGENCE
UNDER POLARIZING MICROSCOPY
CHROMOGRANIN
DIAGNOSIS
MEDULLARY CARCINOMA THYROID WITH
LYMPHNODE METASTASIS
Medullary carcinoma
Malignant tumor showing parafollicular C-cell
differentiation
Secretes calcitonin can produce a variety of Secretes calcitonin can produce a variety of
hormones
Clinical forms of MTC
SPORADIC MTC HEREDITARY MTC
FAMILIAL
MTC
MEN2A MEN2B
AGE (YRS) 45-50 45-55 25-35 10-20
M:F 1:1.4 1:1.1
INHERITANCE NIL AD
OTHER
COMPONENTS
NIL NIL Phaechromocytoma
(40-60%) parathyroid
hyperplasia ( 10-
Pheochromocytoma
(40-60%)
Mucosal neuromas hyperplasia ( 10-
30%)
Mucosal neuromas
Marfanoid habitus
BILATERALITY 0-32% 92-98%
C-CELL
HYPERPLASIA
Usually absent Usually present
LYMPHNODE
METASTASIS
40-50 10-20 14 38
DISTANT MET. 12 0 0-3 20
BEHAVIOUR Intermediate, tumor
related mortality
30%
Most indolent, tumor
related mortality-0%
Relatively indolent
(0-17%)
Most aggressive
GERMLINE
MUTATION RET
Nil 10,11,13,14
Codon 618, 804
10, 11,Codon 634 16 ( codon 918)
Uneventful recovery, No symptomatic hypocalcemia
External Radiotherapy in view of gross residual disease
RTP CT scan- residual disease in Lt thyroid bed, extending in
anterior mediastinum.
EBRT in 2 phase
Case 4, 13/ M
Post-op Management & Course
EBRT in 2 phase
1
st
phase- 36Gy @ 1.8Gy/20# to cervical node till base of skull
44Gy @ 2Gy/22# to tumor bed followed phase 2
nd
boost to
tumor bed.
6 mo follow-up: asymptomatic, lower s-calcitonin, s-CEA,
free of mets
Residual disease regressed