Head and neck tumours
Head and neck, salivary, brain and thyroid tumours (FCPS Part 1)
Oral cancer and areca nut, jaw tumours, salivary tumours, brain tumours by age and the four thyroid cancers for FCPS Part 1. Each tumour is explained by its cell of origin, which predicts its spread, marker and treatment.
Oral cavity: why Pakistan sees so much oral cancer
- More than 90% of oral cancers are squamous cell carcinoma. Pakistan has one of the world's highest rates because of areca nut chewing (betel quid or paan, gutka, supari), smokeless tobacco such as naswar, smoking and alcohol. The tumour typically arises in the buccal mucosa where the quid is held, or on the lateral tongue and floor of mouth.
- Areca nut stimulates fibroblasts to lay down collagen and inhibits its breakdown, causing oral submucous fibrosis: blanched, stiff mucosa with progressive trismus. It is premalignant.
| Lesion | Description | Malignant risk |
|---|---|---|
| Leukoplakia | White patch that cannot be rubbed off and is not another disease | Low overall; higher if speckled or on floor of mouth |
| Erythroplakia | Red velvety patch | Highest |
| Oral submucous fibrosis | Areca nut fibrosis with trismus | Significant |
| Erosive lichen planus | Painful erosions | Small |
- Presentation: non-healing ulcer with an indurated rolled edge, pain referred to the ear, cervical nodes. Spread is mainly to cervical lymph nodes.
- HPV-16 oropharyngeal cancer (tonsil, tongue base) affects younger non-smokers, is p16 positive and has a better prognosis.
Jaw tumours
- Ameloblastoma is an odontogenic epithelial tumour of the posterior mandible (molar–ramus region). X-ray shows a multilocular "soap bubble" radiolucency. It is locally aggressive and recurs if curetted, but rarely metastasises, so it is treated by resection with a margin.
- Odontogenic keratocyst (also posterior mandible, recurs, Gorlin syndrome) and dentigerous cyst (around the crown of an unerupted tooth) are the main differentials.
Larynx, nasopharynx and sinuses
- Laryngeal SCC is a smoking cancer. Glottic tumours (commonest) cause early hoarseness and rarely spread to nodes, because the cords have few lymphatics, so prognosis is good. Supraglottic tumours spread early to nodes. Hoarseness lasting more than 3 weeks needs laryngoscopy.
- Nasopharyngeal carcinoma: EBV and salted fish; neck mass, unilateral serous otitis media in an adult, epistaxis, cranial nerve palsies; treated with radiotherapy.
- Sinus adenocarcinoma: hardwood dust; nickel exposure.
Salivary gland tumours
- About 80% arise in the parotid, about 80% of parotid tumours are benign, and most benign ones are pleomorphic adenomas. The smaller the gland, the more likely a tumour is malignant.
| Tumour | Key features |
|---|---|
| Pleomorphic adenoma | Commonest salivary tumour; painless, slow; epithelial cells in chondromyxoid stroma ("mixed tumour"); small risk of carcinoma ex pleomorphic adenoma |
| Warthin tumour | Older male smokers, often bilateral, cystic with lymphoid stroma; hot on technetium scan |
| Mucoepidermoid carcinoma | Commonest malignant salivary tumour (mucous and squamous cells) |
| Adenoid cystic carcinoma | Perineural invasion (pain, facial palsy), cribriform pattern, late recurrence; commonest malignant tumour of minor glands |
- Why pleomorphic adenoma recurs after enucleation: its capsule is incomplete and finger-like projections (pseudopodia) pierce it. Shelling it out leaves these behind, and they regrow years later as multiple nodules. The correct operation is superficial parotidectomy with a cuff of normal tissue.
- Facial nerve palsy with a parotid mass means malignancy until proven otherwise. Parotidectomy complications: facial nerve injury, Frey syndrome (gustatory sweating from misdirected auriculotemporal fibres), great auricular numbness, salivary fistula.
Neck lump in an adult
- A persistent neck mass in an adult over 40 is metastatic SCC from the upper aerodigestive tract until proven otherwise. Investigate with ultrasound and FNAC and panendoscopy; avoid open excision biopsy before this. In Pakistan, TB lymphadenitis (matted nodes, cold or collar-stud abscess) is the main benign alternative.
Brain tumours
| Group | Commonest tumours | Clues |
|---|---|---|
| Adults | Metastases (lung, breast, melanoma, kidney, colon); glioblastoma; meningioma | Metastases are multiple at the grey–white junction |
| Children | Mostly infratentorial: pilocytic astrocytoma, medulloblastoma, ependymoma; craniopharyngioma (suprasellar) | Neonatal tumours are more often supratentorial |
- Glioblastoma: commonest primary malignant brain tumour; butterfly lesion across the corpus callosum, necrosis with pseudopalisading; surgery, radiotherapy and temozolomide; poor prognosis.
- Gliomas (astrocytes, oligodendrocytes, ependymal cells) are GFAP positive, identifying glial origin. Astrocytoma is the commonest glioma.
- Meningioma: commonest benign intracranial tumour; extra-axial with a dural tail, psammoma bodies, women, NF2; it is the commonest brain tumour after cranial irradiation.
- Pilocytic astrocytoma: cerebellar cyst with mural nodule, Rosenthal fibres, excellent prognosis. Medulloblastoma: commonest malignant childhood brain tumour, cerebellar vermis, CSF drop metastases.
- Vestibular schwannoma: cerebellopontine angle, unilateral sensorineural deafness; bilateral in NF2. Oligodendroglioma: calcified, "fried egg" cells, 1p/19q codeletion.
- Craniopharyngioma (Rathke pouch remnant): calcified suprasellar mass, growth failure, field defect. Surgery near the hypothalamus can damage the ventromedial nucleus (satiety centre), causing hyperphagia, and the supraoptic and paraventricular nuclei, causing diabetes insipidus and thirst.
- Pituitary macroadenoma grows up from below and presses the crossing nasal fibres from the inferior retinas first. These fibres serve the upper temporal fields, so the first defect is bitemporal superior quadrantanopia, progressing to bitemporal hemianopia. Craniopharyngioma presses from above and affects the lower temporal fields first.
- Raised ICP: morning headache worse on coughing, vomiting, papilloedema, sixth nerve palsy. Dexamethasone reduces oedema; avoid LP.
Thyroid cancer
- Nodules are common and mostly benign. Assess with TSH, ultrasound (hypoechoic, microcalcification, irregular margin, taller than wide) and FNAC graded by the Bethesda system.
| Type | Share | Cell of origin | Hallmarks | Spread and prognosis |
|---|---|---|---|---|
| Papillary | About 80% | Follicular | Young women, radiation; psammoma bodies, ground-glass (Orphan Annie) nuclei, nuclear grooves; BRAF V600E | Lymphatic to neck nodes; excellent |
| Follicular | 10–15% | Follicular | Iodine deficiency; needs capsular or vascular invasion, so FNAC cannot diagnose it | Blood to bone and lung; good |
| Medullary | About 5% | Parafollicular C cells (neural crest) | Calcitonin and CEA; amyloid stroma; MEN 2A and 2B (RET) | Lymphatic and blood; not iodine-avid |
| Anaplastic | 1–2% | Dedifferentiated follicular | Elderly, rapid growth, airway compression; p53 | Very poor |
| Lymphoma | Rare | Lymphocytes | Arises in Hashimoto thyroiditis | Chemoradiotherapy |
- Differentiated cancer: total thyroidectomy (lobectomy for small low-risk papillary), radioactive iodine, TSH suppression, follow-up with thyroglobulin.
- MEN 2: medullary carcinoma with phaeochromocytoma (the hypertension in "familial medullary carcinoma") plus hyperparathyroidism in 2A, or mucosal neuromas and marfanoid build in 2B. Exclude phaeochromocytoma before thyroid surgery; RET carriers have prophylactic thyroidectomy.
Remember Name the cell, and the spread follows: follicular cells give papillary (lymph) and follicular (blood); C cells give medullary (calcitonin).
Exam trap A thyroid nodule biopsy showing amyloid stroma points to medullary carcinoma, not anaplastic or follicular.
Exam trap Enucleating a pleomorphic adenoma leaves its capsular projections behind; recurrence is multinodular.
How FCPS asks about this
- Habit causing submucous fibrosis and oral SCC in Pakistan: areca nut chewing; erythroplakia carries the highest malignant risk.
- Soap-bubble multilocular radiolucency of posterior mandible: ameloblastoma, locally aggressive, rarely metastasises.
- Recurrence of an enucleated pleomorphic adenoma: projections pierce its incomplete capsule; do superficial parotidectomy.
- Commonest benign and commonest malignant salivary tumours: pleomorphic adenoma; mucoepidermoid carcinoma.
- First field defect with a pituitary macroadenoma: bitemporal superior quadrantanopia.
- Commonest brain tumours: metastases and glioblastoma in adults; infratentorial tumours in children; psammoma bodies in meningioma; GFAP marks glial cells.
- Thyroid cancer spread and markers: papillary lymphatic, follicular blood, medullary calcitonin with RET and MEN 2.
Test yourself on this topic
5 of the 36 questions in our bank on this topic. Choose an answer to see why every option is right or wrong.
A 60-year-old man has headaches and seizures. MRI shows an irregular ring-enhancing mass crossing the corpus callosum. Histology shows pleomorphic astrocytic cells with pseudopalisading around necrosis and vascular proliferation. What is the diagnosis?
Answer: Glioblastoma
Glioblastoma is the commonest and most aggressive primary malignant brain tumour in adults. It infiltrates widely and may cross the corpus callosum ('butterfly glioma'). Diagnostic features: necrosis with pseudopalisading tumour cells and microvascular (glomeruloid) proliferation. Treatment—maximal safe resection, radiotherapy and temozolomide—gives median survival of about 15 months; MGMT promoter methylation predicts better response. Meningiomas are extra-axial, dural-based and benign (psammoma bodies, whorls); pilocytic astrocytoma is a cystic cerebellar tumour of children with Rosenthal fibres; oligodendroglioma has 'fried-egg' cells and calcification.
Brain tumours
- Glioblastoma: pseudopalisading necrosis, vascular proliferation; butterfly across corpus callosum
- Treat: surgery + RT + temozolomide
- Meningioma: dural, psammoma bodies, whorls
- Child: pilocytic astrocytoma (Rosenthal fibres)
Remember Necrosis lined by palisading cells: glioblastoma.
Why the other options are wrong
- Meningioma Extra-axial, dural tail, whorls.
- Pilocytic astrocytoma Children, cerebellum, cyst with nodule.
- Oligodendroglioma Fried-egg cells, calcification, 1p/19q.
- Cerebral abscess Smooth ring, pus, no pseudopalisading.
FNAC of a thyroid nodule shows a follicular neoplasm. Why can cytology not decide whether it is benign or malignant?
Answer: Invasion needs histology
Follicular adenoma and follicular carcinoma look the same cytologically; carcinoma is defined by invasion through the capsule or into blood vessels, which can only be assessed on the whole excised nodule. Hence FNAC reported as 'follicular neoplasm' (Bethesda IV / Thy3f) usually leads to diagnostic hemithyroidectomy. Papillary carcinoma, in contrast, is diagnosed from nuclear features on cytology (Orphan Annie ground-glass nuclei, grooves, pseudoinclusions, psammoma bodies). Follicular carcinoma spreads by blood (bone, lung); papillary by lymphatics.
Follicular vs papillary
- Follicular: needs capsular/vascular invasion → histology
- Papillary: nuclear features on FNAC
- Follicular spreads by blood; papillary by lymph
Remember Follicular cancer is judged by its capsule, not its cells.
Why the other options are wrong
- Cells are not visible They are seen.
- FNAC always over-calls Not true.
- It has nuclear grooves Nuclear grooves suggest papillary carcinoma.
- Calcitonin is needed instead For medullary carcinoma.
A 45-year-old woman has a firm parotid lump removed. Histology shows a mixture of mucus-secreting cells, squamoid (epidermoid) cells and intermediate cells forming cysts and solid nests. What is the tumour?
Answer: Mucoepidermoid carcinoma
Mucoepidermoid carcinoma is the commonest malignant salivary gland tumour and occurs most often in the parotid. It is made of mucous, epidermoid and intermediate cells in varying proportions; low-grade tumours are mostly cystic and mucous, while high-grade tumours are solid and epidermoid. Many carry a MAML2 gene fusion. Grade determines behaviour: low-grade tumours are cured by excision, high-grade ones spread to nodes.
Mucoepidermoid carcinoma
- Commonest malignant salivary tumour
- Mostly in the parotid
- Mucous, epidermoid, intermediate cells
- Behaviour depends on grade
Remember Mucous plus squamoid cells in the parotid: mucoepidermoid.
Exam trap Facial nerve weakness with a parotid lump suggests malignancy until proven otherwise.
Why the other options are wrong
- Pleomorphic adenoma Benign; epithelial and myoepithelial cells in a chondromyxoid stroma.
- Warthin tumour Benign; oncocytic epithelium with lymphoid stroma.
- Adenoid cystic carcinoma Cribriform 'Swiss cheese' pattern with perineural spread.
- Acinic cell carcinoma Cells resemble serous acinar cells with basophilic granules.
A 75-year-old woman has a hard thyroid mass that has grown rapidly over 4 weeks, with hoarseness and stridor. Biopsy shows pleomorphic undifferentiated cells. What is the outlook?
Answer: Very poor; survival usually months
Anaplastic thyroid carcinoma occurs in older people, often in a long-standing goitre, and grows rapidly, invading the trachea, oesophagus and recurrent laryngeal nerves. It does not take up iodine, so radioactive iodine is ineffective. Most patients die within months; treatment focuses on airway protection, palliation and sometimes targeted therapy.
Anaplastic thyroid cancer
- Elderly, rapid growth
- Invades trachea, nerves
- No iodine uptake
- Survival in months
Remember Old woman, fast hard neck mass: anaplastic.
Exam trap Lymphoma also causes rapid thyroid enlargement (with Hashimoto) and is treatable; biopsy matters.
Why the other options are wrong
- Excellent with surgery alone Usually unresectable at presentation.
- Cured by radioactive iodine Does not concentrate iodine.
- Good; spreads only to local nodes That describes papillary carcinoma.
- Usually regresses spontaneously Not true.
A 30-year-old has a thyroid nodule with raised calcitonin. His father had a phaeochromocytoma and hypercalcaemia. Which gene should be tested?
Answer: RET proto-oncogene
Medullary thyroid carcinoma (from calcitonin-producing C cells) with phaeochromocytoma and primary hyperparathyroidism is MEN 2A, caused by gain-of-function mutations of the RET proto-oncogene (autosomal dominant). MEN 2B adds mucosal neuromas and a marfanoid habitus. Phaeochromocytoma must be excluded and treated before thyroid surgery. Carriers have prophylactic thyroidectomy in childhood.
MEN 2
- Gene: RET (gain of function)
- 2A: MTC, phaeo, parathyroid
- 2B: MTC, phaeo, mucosal neuromas
- Treat phaeo first
- Prophylactic thyroidectomy
Remember RET: medullary thyroid in the family.
Exam trap Operating on the thyroid before excluding phaeochromocytoma risks a hypertensive crisis.
Why the other options are wrong
- MEN1 (menin gene) MEN 1: parathyroid, pancreas, pituitary.
- BRAF (V600E) Papillary thyroid cancer.
- VHL tumour suppressor VHL: renal cell carcinoma, phaeochromocytoma, haemangioblastoma.
- APC tumour suppressor Familial adenomatous polyposis.
Where this sits in the CPSP syllabus
- Surgery & Allied, Paper 2: Oncology, Head and neck, brain and thyroid tumours
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