Etiology
- Radiation – papillary carcinoma (ca)
- Pre-existing multinodular goitre – follicular ca
- Familial – medullary ca
- Hashimoto’s is a predisposing factor for NHL/papillary ca
- Genetic – Cowden syndrome
- differentiated thyroid ca, breast ca, multiple harmatomas
Classification –Dunhill Classification
Differentiated – most common
- Papillary ca
- Follicular ca
- Hurthle ca
Undifferentiated
- Anaplastic ca
Medullary carcinoma
Malignant lymphoma
Secondaries – from colon, kidney, melanoma, breast
Papillary Carcinoma
Etiology
- Most common in females and younger age group
- Radiation – external or internal radio-iodine therapy
- TSH levels are high in these patients – hormone dependent tumour
Pathoanatomy
- Gross – can be soft, firm, hard, cystic, solitary or multiple. Contains brownish-black fluid
- Microscopic – cystic spaces, papillary projections with Psammoma bodies , malignant cells with ‘Orphan Annie eye’
Clinical features
- Soft/hard/firm, solid/cystic, solitary/multinodular thyroid swelling
- Compression features are uncommon
- Neck lymph nodes are palpable – secondaries
Investigations
- FNAC – of nodule and lymph node
- Radioisotope scan – cold nodule
- High TSH level in blood
- X-ray – calcification
- US/CT neck – better to see non-palpable node in neck
Treatment
- Total/near total thyroidectomy with central node compartment dissection
- Level VI (central compartment) – paratracheal and pretracheal nodes
- Suppressive dose of L-thyroxine
- Radioactive 1131 therapy
- Prognosis – good
- AMES scoring assessing prognosis
Follicular Carcinoma
Etiology
- Can occur de novo or in pre-existing multinodular goitre
- More common in females
Pathophysiology
- Thyroglobulin is produced by the follicular cells – it produces T3 and T4
Spread
- Mainly through blood into the bones, lungs, liver
- Bone secondaries are most common in the skull, long bones, ribs
- Only 10% spread to the neck lymph nodes
Types
- Noninvasive – blood spread uncommon
- Invasive – blood spread common
- Capsular invasion and angio-invasion
Clinical features
- Neck swelling – firm or hard nodular
- Tracheal compression/infiltration, stridor
- Dyspnea, haemoptysis, chest pain – lung secondaries
- Recurrent laryngeal nerve involvement – hoarseness
- Berry’s sign – absence of carotid pulse in presence of neck swelling
- Signifies advanced malignancy – infiltration into carotid sheath
Investigations
- FNAC is often inconclusive – unable to detect capsular and angio-invasion
- US abdomen, CXR, XR of bones
- Trucut biopsy – gives tissue biopsy, but danger of haemorrhage and injury to vital structures
- E.g. trachea, recurrent laryngeal nerve, vessels
Treatment
- Total thyroidectomy with central node compartment dissection (level VI)
- Functional neck node dissection – with preservation of sternocleidomastoid, spinal accessory nerve, internal jugular vein
- Lifelong maintenance dose of L-thyroxine
Follow up
- I131 scan at every 6 months – look for secondaries
- Thyroglobulin estimation – >50μg signifies recurrent/metastatic disease
- Normal value – 0.5-50μg
Hurthle Cell Carcinoma
- Variant of follicular carcinoma which contains abundant oxyphill cells
- Spreads more commonly to regional lymph nodes than follicular carcinoma
Anaplastic carcinoma
- Aggressive tumour of short duration, most common in elderly females
- Presents with swelling in thyroid region, rapidly progresses causing
- Stridor and hoarseness due to tracheal obstruction
- Dysphagia
- Fixity to skin
- Berry’s sign
- Swelling is hard, involves isthmus and lateral lobes
- FNAC – diagnostic
- Tracheostomy and isthmectomy – relieve respiratory obstruction temporarily
- Treatment
- External radiotherapy
- Chemo – adriamycin
- Poor prognosis – few weeks to a month
Medullary Carcinoma
- Arises from parafollicular C cells – derived from the neural crest
- Contains amyloid stroma – where malignant cells are dispersed
- Immunohistochemistry – shows calcitonin in amyloid
- Patients have high blood levels of calcitonin – tumour marker
- Tumour secretes serotonin, prostaglandin, ACTH, vasoactive intestinal polypeptide
- Spreads mainly to lymph nodes
- Sipple’s disease – medullary carcinoma associated with MEN IIB (most aggressive)
- Not TSH-dependent – so doesn’t take up radioactive iodine
Clinical features
- Thyroid swelling and enlarged lymph nodes
- Diarrhoea, flushing
- Hypertension, phaeochromocytoma, mucosal neuromas – when associated with MEN II
- Sporadic and familial types – most common in adulthood
Investigations
- FNAC – amyloid deposits with dispersed malignant cells and C cell hyperplasia
- Tumour marker – calcitonin
- US abdomen and neck
Treatment
- Total thyroidectomy with central node dissection (VI)
- No role of suppressive hormone therapy or radioactive iodine
- External radiotherapy – for residual tumour
- Somatostatin/octreoide – for diarrhoea
- Chemotherapy – adriamycin (limited results)
Malignant lymphoma
- Type of non-Hodgkin lymphoma
- Frequently associated with Hashimoto’s thyroiditis
- Diagnosis – FNAC or Tru-cut biopsy
- Treatment – chemotherapy and radiotherapy