Head and Neck Neoplasia

Basic concepts

Head and neck tumours include a wide spectrum of pathologies with different patterns of behaviour. The complex anatomy of this region makes their management difficult as therapy may result in disruption of speech, swallowing and cosmesis. Patients with head and neck cancer need to be treated in specialist (tertiary) cancer centres by specialist multidisciplinary teams (MDT).

Squamous cell carcinoma of the upper aerodigestive tract accounts for over 90% of tumours in this region.

Management of head and neck cancer is dependent on histological diagnosis, staging and grading of the tumour. The patient’s wishes are equally important factors in determining therapy. Some patients may not be fit for aggressive curative treatment and others may refuse treatment. The head and neck oncologist uses surgery, radiotherapy and chemotherapy as treatment modalities. In general, early cancers are treated with single-modality therapy and advanced cancers require combined modality. A treatment algorithm for head and neck cancer is shown in Figure 4.1.

Fig. 4.1 Treatment principles for head and neck cancer.

Aetiological factors

Cancer develops through a complex multifactorial process (Fig. 4.2). The majority of head and neck cancers result from exposure to carcinogens, mainly via tobacco. Chewing tobacco is also carcinogenic and associated with mouth cancer. Alcohol appears to act synergistically with smoking. Betel nut, which is widely chewed in the Indian subcontinent, is a strong carcinogen for mouth cancer, hence the very high incidence of oral cancer in this region. Exposure to ionizing radiation is implicated in thyroid cancer and sarcomas. Oncogenic viruses such as human papilloma virus (HPV) are known to induce tumours in squamous epithelium. HPV is contracted through oral sex and is thought to be the cause of the increase in incidence of oral and oesophageal cancers in younger patients. Epstein–Barr virus (EBV) is associated with nasopharyngeal cancer and Burkitt’s lymphoma. Heavy metals, such as nickel or chromium, and hardwood dust exposure are important occupational carcinogens. Severe chronic dental caries is thought to predispose to mouth cancer.

Fig. 4.2 Aetiological factors in the development of head and neck neoplasia.

The incidence of head and neck cancer is increasing and the demographics are changing, with more younger men and women being diagnosed. This is due to changes in lifestyle habits such as smoking, alcohol consumption and sexual activity.

Genetic factors also predispose to cancer. Multiple endocrine neoplasia (MEN) is an inherited condition associated with medullary thyroid cancer (MTC) and caused by a specific gene mutation.

Premalignant conditions in head and neck cancer

Head and neck cancer may develop de novo but in a significant proportion of cases premalignant conditions exist long before the tumour develops. These lesions arise as a result of chronic exposure to carcinogens. Dysplasia is the pathological term to describe changes in the cell structure and epithelium architecture which predispose to cancer. Dysplasia ranges from mild, moderate, severe to carcinoma in situ. The management of dysplastic lesions depends upon their size, severity and location. Lifestyle changes are very important: if exposure to the carcinogen is removed, the dysplastic lesion may resolve. Surgery is used to remove localized premalignant lesions. Radiotherapy is useful in diffuse dysplasia. Retinoids have been used in oral dysplasia. New diagnostic screening mouthwashes are also available to detect premalignant lesions.

Clinical manifestations of premalignant conditions are leukoplakia (white patch) or erythroplasia (red patch) which may affect the mucosa (Figs 4.3 and 4.4). These often present as superficial lesions and should be biopsied to determine the grade of dysplasia.

Fig. 4.3 Leukoplakia (thin arrow) and erythroplasia (branched arrow) of the oral tongue. Note the staining from betel nut chewing on the contralateral side of the tongue (thick arrow).

Fig. 4.4 Leukoplakia of the larynx in a heavy smoker. The right vocal cord is affected by dysplastic epithelium (arrow).

Lichen planus

Erosive lichen planus of the oral cavity (Fig. 4.5) may progress to cancer. The common form of lichen planus, which is benign, is located in a symmetrical distribution on the buccal mucosa and tongue. The erosive variety appears in the floor of the mouth. Biopsy is mandatory to identify the type of lichen planus and to distinguish it from leukoplakia.

Fig. 4.5 Erosive lichen planus.

Principles of treatment

The treatment options in head and neck neoplasia include:

radiotherapy ± chemotherapy

surgical excision

terminal care (p. 116).

The preferred modalities, either singularly or in combination, can only be determined by patient assessment, histological information and the extent of the local and regional disease. The majority of head and neck malignancies require primary radiotherapy followed by salvage surgery for recurrent and residual disease (RRD). In advanced disease, treatment will be palliation.

Radiotherapy

Ionizing radiation destroys cancer cells by preventing their division. Irradiation sources include X-rays, gamma and beta rays. More recently, fast neutrons have been employed, but with disappointing results. Since all tissue will absorb radiation, it is important to minimize unacceptable damage. This is achieved by accurate localization and by fractionating the radiation dosage.

Unfortunately, two structures, the lens of the eye and the spinal cord, are highly sensitive to irradiation. Exposure of these two organs is avoided by accurate planning of the radiotherapy fields, based on clinical examination and computed tomography (CT)/magnetic resonance imaging (MRI). A specific mask is constructed to keep the head and neck steady during therapy.

The majority of patients will develop radiation reactions in normal tissue. The skin will invariably show some evidence of treatment (Fig. 4.6). Mucous membrane reactions tend to occur very early, with erythema and ulceration, and may be so severe that nasogastric feeding is required. Fungal infections, particularly by Candida, often compound the mucositis and are not uncommon in the debilitated patient. Prophylactic care with antifungals and anti-inflammatory rinses may be required.

Fig. 4.6 Radiotherapy skin reaction. The mucous membrane of the lip is severely ulcerated.

Neck lumps – introduction

Many neck swellings may be diagnosed after a comprehensive history and a thorough clinical examination of the head and neck. Further evaluation with imaging, endoscopy and biopsy will be required in some instances. It is inappropriate to resort to open biopsy until a full evaluation of local and distant diagnoses has been undertaken. An incorrect biopsy technique of a neck lump may compromise the prognosis of a patient with metastatic neck disease.

It is useful to consider separately the diagnosis of neck lumps in children and adults. The ‘80 : 20 rule’ applies to malignant and benign causes of neck masses (Fig. 4.7). In the adult it must be remembered that metastatic neck disease may occur from structures below the clavicle (Table 4.1).

Fig. 4.7 The ‘80:20’ rule applied to neck lumps.

Table 4.1 Infraclavicular sites of malignancy that may cause neck lumps by metastatic spread

Lung	Kidney
Breast	Prostate
Stomach	Uterus
Pancreas

Clinical history and examination

In addition to a routine history, specific questions must be posed. The ‘20 : 40 rule’ is useful in considering the diagnostic possibilities of a neck lump (Table 4.2).

Table 4.2 The ‘20 : 40 rule’ applied to neck lumps

Age (years)	Possible causes of neck lump
Less than 20	Inflammatory neck nodes (e.g. due to tonsillitis)
	Congenital lesions (e.g. thyroglossal cysts, brachial cyst, midline dermoid, cystic hygroma)
	Lymphoma
20–40	Salivary gland pathology (calculus, infection, tumour)
	Thyroid pathology (tumour, thyroiditis, goitre, lymphoma)
	Chronic infection (tuberculosis, HIV)
Greater than 40	Primary or secondary malignant disease

The presence of pain is a helpful clue to diagnosis. Inflamed tissue, e.g. the lymph nodes, will be tender. Salivary gland calculi may present with recurrent pain and swelling, especially during eating. Congenital lesions such as branchial and thyroglossal cysts may present as painful neck lumps. It is important to establish whether the lump is increasing, decreasing or static in size. The level of tobacco and alcohol intake should be determined.

The primary head and neck sites of malignancy may give rise to very specific symptoms including:

dysphagia (pharyngo-oesophagus)

dysphonia (larynx and hypopharynx).

Earache may be referred from neoplastic lesions in the upper food passages (see Fig. 1.28, p. 13). Referred otalgia is a poor prognostic sign in head and neck neoplasia.

In the presence of a neck lump, any weight loss is significant and implies the presence of malignant disease. If associated with dysphagia, the site is usually in the upper digestive tract, but particularly in the pharyngo-oesophageal region.

Certain systemic symptoms may also give clues to the aetiology of a neck lump. Malaise is a feature of lymphoma and tuberculosis. Nocturnal fevers and pruritus are also common in lymphoma.

It is important to perform a thorough examination of the head and neck, especially the upper aerodigestive tract, as well as looking for other lumps, e.g. in the liver, spleen or axillae. The scalp should be carefully examined, as a primary malignancy in this site is commonly overlooked as a cause of metastatic neck disease. The precise features of the lump should be noted and, if laterally sited, its position in the triangles of the neck accurately described (Fig. 4.8). This approach is useful as an aid to remembering structures located in the triangles which may give rise to pathology. A mass in the midline is most frequently of thyroid origin. Nasopharyngeal carcinoma may present as unilateral or bilateral metastatic nodes in the posterior triangle of the neck. An isolated mass in the supraclavicular region is likely to be metastatic disease from sites below the clavicle (Table 4.1).

Fig. 4.8 The triangle of the neck. The sternocleidomastoid divides the neck into two triangles, the boundaries of which are shown. The anterior triangle contains lymph nodes, submandibular gland, tail of the parotid and the carotid bifurcation. The posterior triangle contains lymph nodes and the spinal accessory nerve.

Multiple neck lumps are most likely to be lymph nodes. There are over 100 lymph nodes on each side of the neck, although they tend to be confined to relatively discrete areas rather than evenly distributed (Fig. 4.9).

Fig. 4.9 The lymph-node distribution in the neck, and the drainage pattern.

Palpation of a neck lump may reveal the presence of pulsation, e.g. carotid body tumours. It is important to determine that this pulsation is not transmitted from arteries in the neck. A pulsatile neck lump should be auscultated to detect the presence of a bruit.

Investigations

The approach to neck lumps is discussed early in this chapter. A proportion of them will be considered to be sinister in origin i.e. either primary or metastatic malignancies. The modern practice is to refer to a “Rapid Access Neck Lump Clinic”, where a multidisciplinary team (MDT) of Head and Neck Surgeon, Radiologist and Cytologist can assess in a one-stop clinic scenario.

An MDT approach will allow clinical examination, a radiological assessment, usually an ultrasound, followed by Fine Needle Aspiration Cytology (FNAC). The cytologist will then identify the specimen and decide its nature (Fig. 4.10):

1. Benign

2. A suspicion of malignancy or

3. Malignant

Fig. 4.10 Fine-needle aspiration cytology smears. (a) From a neck lump showing colloid with a cracking artefact (a1); normal follicular epithelial cells (a2) and a papillary carcinoma of the thyroid (a3) and (b) reactive lymph nodes (b1 and b2) and a high grade non-Hodgkins lymphoma (b3).

(Courtesy of Dr. Tanya Levine, Northwick Park Hospital).

This will direct the management to further and more sophisticated investigations such as CT, MRI or PET scanning (Fig. 4.26, p. 97). In some cases an examination of the upper air and food passages may be required prior to making a definitive diagnosis and formulating a treatment plan.

Biopsy of a neck lump

The diagnosis of a neck lump may be dependent on biopsy. This should only be undertaken after extensive investigations to exclude pathology in potential primary head and neck sites. This would generally include a fine-needle aspiration of the mass for cytological assessment. Examination under general anaesthesia of the upper aerodigestive tract may be required. If both aspiration cytology and a full examination under anaesthesia are negative, an open excisional biopsy should be performed, with a view to proceeding to neck dissection.

With this systematic approach, many neoplasms can be diagnosed without recourse to open biopsy, avoiding the danger of implantation in the neck skin.

Neck lumps – paediatric conditions

In the paediatric age group (less than 20 years of age), the majority of neck lumps encountered are benign. They are commonly located anterior to the sternomastoid muscle in the anterior triangle of the neck. An isolated neck lump located in the posterior triangle has a high likelihood of being malignant. The ‘80 : 20 rule’ is useful in assessing the diagnostic possibilities (see Fig. 4.7, p. 90).

For diagnostic and descriptive purposes, neck lumps can be described by their position: midline or lateral.

Midline neck lumps

Thyroglossal cyst

The most common midline mass (Fig. 4.11) in children is a congenital cyst of the thyroglossal duct. Embryologically, the cyst can arise at any site along the route of the thyroglossal duct, extending from the tongue (foramen caecum) to the thyroid gland.

Fig. 4.11 Some causes of midline neck lumps in the paediatric age group.

The thyroglossal cyst is most commonly located below the hyoid bone, and moves on both swallowing and tongue protrusion (Figs 4.11 and 4.12). Most cysts are asymptomatic, apart from the presence of a lump, but infection will be associated with pain and swelling. Treatment is by excision, which should include the central portion of the body of the hyoid bone to prevent recurrences. A wedge of tongue muscle is resected with the thyroglossal duct behind the hyoid.

Fig. 4.12 A thyroglossal cyst.

Dermoid cyst

Dermoid cysts usually present as submental swellings in the midline. They are dermal remnants occurring along the lines of fusion in the embryo. These cysts are lined by epidermis and may contain hair, teeth and squamous debris. They tend not to move on swallowing or tongue protrusion. Dermoid cysts should be excised.

Miscellaneous lumps

Other midline lumps are rare in children. Chondromas of the cartilaginous structures of the larynx are hard to palpation, and move on swallowing. Treatment is by excision. Occasionally a pyramidal lobe of the thyroid may present as a midline lump. An ultrasound and isotope scan will reveal the underlying cause. Lymph nodes located in the midline of the neck may enlarge secondary to infection or neoplasia.

Lateral neck lumps (Table 4.3)

Inflammatory conditions

Enlarged infected lymph glands of the neck are the most common cause of lateral neck lumps in children. An infective aetiology is accompanied at some stage by tender enlargement. Multiple palpable, non-tender nodes are a normal feature in many children.

Table 4.3 Causes of lateral neck lumps in children

Infective	Cervical lymphadenitis
	Mumps
	Tuberculosis
Congenital	Branchial cysts
	Chemodectoma
	Cystic hygroma
	Haemangioma
Neoplastic
Primary	Lymphoma
	Neuroblastoma
	Parotid malignancy
	Rhabdomyosarcoma
Secondary	Metastases – nasopharyngeal

The primary areas for infection in the head and neck should be carefully inspected and include the skin and scalp in addition to the oral cavity, oropharynx and nasopharynx. The most common infections are of the upper respiratory tract, tonsils and teeth. Resolution of infective lymph nodes is rapid as the primary infection settles. Persistent lymphadenopathy should be re-evaluated.

Mumps

Enlargement of the parotid glands, due to the mumps virus, is extremely common. It is usually a bilateral disease, but unilateral cases can occur (Fig. 4.13). The child has constitutional symptoms of malaise and pyrexia. Rare cases may be complicated by orchitis and encephalitis. Treatment is symptomatic.

Fig. 4.13 Bilateral parotid enlargement due to mumps.

Tuberculosis

Tuberculosis of the cervical lymph nodes is uncommon. Tuberculous nodes are multiple and coalesce, and may form a discharging sinus (Fig. 4.14). Most cases have associated pulmonary tuberculosis. Node biopsy is sometimes required for histological confirmation of diagnosis. Treatment is by combination chemotherapy.

Fig. 4.14 Tuberculous neck nodes about to discharge through the skin.

Congenital conditions

Most solitary lateral neck masses in the paediatric age group are congenital in origin.

Branchial arch cysts

Branchial arch anomalies giving rise to branchial cysts are uncommon. First branchial arch cysts are rare and located anterior to the tragus. True branchial cysts are more frequently encountered and invariably located in the anterior triangle just in front of the sternomastoid (Fig. 4.15). The aetiology is believed to be cystic degeneration in a lymph node. Most of these cysts are lined by lymphoid tissue so that pain and swelling may be experienced with upper respiratory infections. Where a second arch fistula is present a tract may extend to the pharynx, and this must be excised together with the cyst. All brachial arch cysts presenting in patients over 40 years of age should be considered as a possible undiagnosed squamous cell carcinoma.

Fig. 4.15 A lateral neck swelling due to a branchial cyst.

Cystic hygromas

Cystic hygromas are anomalies of the lymph channels and present as lateral neck swellings. They are soft and irregular, and usually present at birth (Fig. 4.16). Typically, the hygroma enlarges during crying and the Valsalva manoeuvre. They transilluminate brilliantly. Most cystic hygromas have to be removed owing to continued enlargement, particularly as they may encroach onto the major airways. Excision is difficult, as this benign lesion encompasses structures such as the carotid arteries and facial nerve.

Fig. 4.16 A large cystic hygroma of the neck in a neonate.

Chemodectomas and haemangiomas

Chemodectomas (glomus tumours) are extremely rare benign tumours arising from the carotid bulb in the region of the carotid bifurcations. They are pulsatile, and a bruit is audible with a stethoscope. Palpation reveals movement in the lateral but not vertical plane. MRI confirms the diagnosis. Surgical treatment will be required in the young age group. In the elderly they may be left untreated as the tumour is extremely slow growing and the risk of metastases is very small.

Haemangiomas are seen in the neonatal period. They are extremely rare and many regress as the child matures. Treatment is required only if the lesion is enlarging and the patient is symptomatic.

Neoplasia

Neoplasia is usually due to primary cancer in the neck, but secondary metastatic disease, particularly from the nasopharynx, can present as an isolated neck lump.

Lymphoma of the Hodgkin’s variety is common. It may present as a unilateral isolated lump in the neck (Fig. 4.17). After histological confirmation, a full evaluation will be required to stage the disease. Treatment is usually radiotherapy in localized disease and chemotherapy with drug combinations in systemic lymphoma.

Fig. 4.17 Swelling of the right neck due to lymphoma.

Rarer primary neoplasia include malignant parotid disease, rhabdomyosarcomas and neuroblastomas.

Neck lumps – adult conditions

The majority of adult neck lumps are malignant in origin, with metastatic squamous cell carcinoma from the upper aerodigestive tract being the most common cause. Benign masses constitute 20% of the total.

Midline neck lumps

Thyroid masses

The thyroid gland is a hormonal gland lying in the midline of the neck at the level of the thyroid cartilage. It consists of a left and right lobe joined by an isthmus. Thyroid masses are common and a systematic approach to managing them should be adopted.

It is important to determine whether there is a goitre (diffuse bilateral thyroid enlargement) or a nodular (single) mass within the thyroid (Figs 4.18 and 4.19). Symptoms and signs of hyper- (overactive) and hypo- (underactive) thyroid disease should be sought (Table 4.4). Clinical examination should determine the size and nature of the thyroid mass. Thyroid enlargement may result in compression of either the trachea, causing stridor, or oesophagus, causing dysphagia. Common disorders to affect the thyroid gland include thyroiditis, multinodular goitre, follicular adenoma, thyroid carcinoma and lymphoma.

Fig. 4.18 Woman with a goitre (diffuse thyroid enlargement).

Fig. 4.19 Woman with single left thyroid nodule.

Table 4.4 Symptoms and signs of thyroid disease

Disease	Symptoms	Signs
Hyperthyroidism	Palpitations	Tachycardia (AF)
	Weight loss	Exophthalmos
	Agitation	Tremor
	Sweating
Hypothyroidism	Tiredness	Bradycardia
	Weight gain	Loss of eyebrow hair
	Poor concentration	Loss of eyebrow hair

Investigations

Blood tests should determine thyroid status; a thyroid-stimulating hormone (TSH) test is the first-line investigation. Autoimmune thyroid antibodies should be measured along with thyroglobulin. FNAC should be undertaken to determine the cellular nature of any thyroid nodule. Ultrasound scanning is the first imaging of choice; it will determine whether the thyroid swelling is cystic or solid and will demonstrate whether multiple nodules are present. Ultrasound and FNAC can be combined to increase diagnostic yield. CT scanning is only indicated when tracheal compression or retrosternal extension is suspected (Fig. 4.20).

Fig. 4.20 CT scan of neck with large goitre compressing the trachea.

Management

Abnormal thyroid status must be controlled and this may involve drug treatment. Nodular thyroid swellings are usually managed by thyroid lobectomy. Goitres may need total thyroidectomy if they cause compressive symptoms.

Thyroid cancer

Thyroid cancer is rare and accounts for only 1–2% of cancers in the UK. The majority present as solitary nodules in the thyroid gland, but only 10% of solitary nodules will turn out to be cancerous.

The common thyroid cancers are papillary, follicular, medullary and anaplastic. Management involves FNAC to diagnose the lesion, imaging and staging. Treatment comprises surgery (a total thyroidectomy) followed by radioiodine ablation. In general, a 90% survival at 10 years can be expected with differentiated thyroid cancer.

Miscellaneous midline lumps

Thyroglossal cysts, midline dermoids and a prominent pyramidal lobe of the thyroid may all be causes of midline neck lumps in adults. These have been discussed elsewhere (see p. 92).

Lateral neck lumps

Neoplasia

Any neck lump appearing for the first time in an adult over 40 years of age should be treated as metastatic cancer until proven otherwise (Table 4.5). Secondary neck disease from malignancy in the upper aerodigestive tract is very common. The patient frequently gives a long history of alcohol and tobacco abuse. The possibility of a supraclavicular neck mass being metastatic disease from sites below the clavicle should not be overlooked (see Table 4.1, p. 90).

Table 4.5 Causes of a lateral neck lump in adults

Type	Condition
Neoplasia	Primary cancer
	Lymphoma
	Neurogenic (schwannoma, chemodectoma)
	Metastatic cancer
	Lymph-node metastasis from head and neck sites
Infection	Glandular fever
	HIV
	Tuberculosis
	Parotitis (mumps)
Autoimmune	Sjögren’s syndrome
Miscellaneous	Sarcoidosis
	Branchial cyst
Normal variants	Transverse process of 2nd cervical vertebra (C2)
	Elongated styloid process
	Normal or cervical rib
	Tortuous, atherosclerotic carotid artery

Unilateral painless parotid masses are likely to be neoplastic, the most common lesion being the benign pleomorphic adenoma. Malignant parotid tumours may cause pain and facial weakness owing to involvement of the facial nerve (p. 113). Hodgkin’s and non-Hodgkin’s lymphoma may initially present as an isolated lateral neck lump. However, disease progression leads to multiple matted neck lumps.

Certain tumours of neural crest origin may present as lateral neck lumps in the adult. These include carotid body tumours, glomus vagale and neurofibromas of the vagus nerve. Multiple neurofibromata (café-au-lait skin pigmentation and cutaneous and neural tumours) may be associated with von Recklinghausen’s disease.

Inflammatory conditions

Glandular fever is a common infection in young adults and invariably presents as a sore throat, similar to an acute streptococcal tonsillitis, with bilateral tender enlargement of cervical nodes. Severe cases may also have hepatosplenomegaly and haematological abnormalities. The diagnosis is confirmed by the presence of atypical monocytes in the peripheral blood and a positive serological test to Epstein–Barr virus antibodies (Paul–Bunnell or Monospot test).

Acute parotitis, either bacterial or viral, may cause neck swelling (Fig. 4.21). The diagnosis is usually straightforward, provided the full anatomical extent of the parotid is appreciated, including the deep lobe which may enlarge into the oropharynx. An infection of the parapharyngeal space of the neck, usually from dental or oropharyngeal infections, may produce a significant neck swelling in association with a mass in the throat (Fig. 4.22).

Fig. 4.21 A unilateral parotid swelling caused by a bacterial parotitis.

Fig. 4.22 An external neck swelling due to infection in the parapharyngeal space.

Tuberculosis in the cervical nodes is uncommon in Europe but very frequent in developing countries. If not associated with pulmonary tuberculosis, an excisional biopsy may be required to confirm the diagnosis.

Sjögren’s syndrome

Sjögren’s syndrome is a triad of xerostomia (dry mouth), keratoconjunctivitis sicca (dry eye) and a systemic autoimmune disease, e.g. rheumatoid arthritis or scleroderma. Parotid enlargement, usually bilateral, is due to lymphocytic infiltration, and the gland shows a reduction in saliva formation. The lacrimal glands are similarly involved, which results in reduced or absent tear formation. Autoantibodies are present in the peripheral blood. A buccal labial biopsy with histological grading is the diagnostic investigation.

Treatment is symptomatic, with the provision of artificial tears and saliva. However, these patients require long-term follow-up, as a small percentage will develop lymphoma in the parotid gland.

Miscellaneous lateral lumps

Sarcoidosis

Sarcoidosis in the neck rarely occurs without mediastinal disease. If hilar involvement is absent, diagnosis is made by biopsy of the neck lump, which reveals the typical non-caseating granulomas. The finding of raised serum angiotensin-converting enzyme levels is diagnostic.

HIV infection

The primary infection with HIV may produce prodromal symptoms similar to glandular fever (p. 86). Persistent lymphadenopathy syndrome (PLS) is seen in the chronic stage of infection with HIV, with lymph nodes in the neck being commonly enlarged. HIV infection may also present as a cystic swelling in the parotid gland, a ‘lymphoepithelial cyst’.

Normal variants

Certain normal bony and cartilaginous structures in the neck may be palpable in some patients and mistaken for lumps (Fig. 4.23). The lateral process of the axis (C2) is often palpable and tender if slight pressure is applied. These features may only be demonstrated on one side of the neck. The styloid process may be elongated and ossified, and therefore palpable as it runs just anterior from the mastoid to the mandible. Normal ribs and, occasionally, an asymptomatic cervical rib may be palpated deep in the supraclavicular fossa. A tortuous atherosclerotic carotid artery in a thin elderly person may be mistaken for a neck mass. It may not be pulsatile, but a bruit is usually audible on auscultation.

Fig. 4.23 Normal variants that can mimic a lateral neck lump in adults.

Neck lumps – adult conditions

Thyroid lesions are the most common cause of midline lumps in adults.

The most common lateral neck lump in adults is metastatic malignant disease, usually squamous cell carcinoma from a primary site in the head and neck.

The symptoms and signs of acute infection with HIV mimic the clinical features of glandular fever.

Neck lumps – management of malignant lumps

In some patients a neck mass may be a lymph node affected by infection, lymphoma or metastatic carcinoma, rather than the specific neck diseases discussed on previous pages.

The general management of such patients is outlined in Figure 4.24. A full ear, nose and throat (ENT) evaluation will include inspection, radiology and possible biopsy of primary sites in the head and neck. If the primary sites are clear, FNAC may assist in the diagnosis. Otherwise, the mass must be biopsied by excision as incisional biopsies carry the risk of implantation of malignant cells in skin.

Fig. 4.24 Management of an isolated neck lump.

The diagnosis of an inflammatory or lymphomatous process in a lymph node will allow appropriate therapy in the former, and staging and eventual treatment strategies in the latter.

Metastatic cervical nodes

Metastatic cervical nodes are clinically assessed and then classified according to the UICC/AJC criteria (Table 4.6). Since the classification is clinically based, it is subject to observer variation. It is also not feasible to decide whether a palpable node contains metastatic cancer or is merely enlarged due to infection. The implication in the classification is that prognosis deteriorates from N1 through to N3 stages. More recently, it appears that the level of metastatic disease in the neck is a better prognostic indicator. Inferiorly placed neck disease has the worst prognosis, with supraclavicular node involvement having the least favourable 5-year survival.

Table 4.6 Classification of regional lymph nodes affected by metastatic carcinoma

Classification (UICC/AJC)	Clinical assessment
N0	No regional nodes palpable
N1	Mobile ipsilateral nodes
N2	Mobile contralateral or bilateral nodes
N3	Fixed nodes

UICC: International Union Against Cancer; AJC: American Joint Committee for Cancer Staging.

The treatment of metastatic cervical nodes depends to a large degree on whether the primary disease in the head or neck, or in distant sites, has been identified. As a rule, surgery in the form of a modified radical neck dissection is advocated for metastatic neck disease (Fig. 4.25). Radiotherapy may be employed in occult and small nodal metastases, and in palliation of fungating lesions.

Fig. 4.25 Principles of the radical neck dissection for metastatic neck disease.

N0: clinically negative neck nodes

Impalpable lymph nodes involved in metastatic disease are called occult nodes. There are certain sites in the head and neck, with a rich and frequently decussating lymphatic supply, from which metastatic nodal disease is highly probable (Table 4.7). Although it would be logical therefore to consider performing an elective modified radical neck dissection in occult neck disease, such a policy shows little benefit. It appears that in selected patients, prophylactic radiotherapy markedly diminishes the incidence of recurrent neck disease with little increase in morbidity.

Table 4.7 Sites of primary carcinoma with a high incidence of occult nodes

N1: palpable ipsilateral neck nodes

N1 metastatic disease is subclassified into whether the primary site is known or unknown.

N1 with known primary site

In these patients the management of the metastatic neck disease must be considered with the primary tumour. Treatment may be primary radiotherapy or primary surgery.

Chemoradiation is chemotherapy combined with radiotherapy and is now used as primary treatment for many advanced head and neck cancers.

If primary irradiation fails to control neck disease, a neck dissection is indicated. Neck nodes over 3 cm in diameter are unlikely to be sterilized by radiotherapy and should be primarily treated by surgery. Metastases from thyroid carcinoma are usually managed by local nodal clearance without formal neck dissection.

N1 with no known primary site (occult primary)

The histological appearance of the lymph node may give a clue to where the primary malignant site may be located (Table 4.8). Metastatic supraclavicular nodes are likely to have been involved from infraclavicular primary malignant sites.

Table 4.8 The occult primary: how the histology of a malignant node may assist in determining the primary site

Histology of metastatic neck node	Probable primary malignant sites
Squamous cell carcinoma	Head and neck sites: nasopharynx, tonsil, tongue base, supraglottic larynx, floor of mouth, piriform fossa, postcricoid region
Adenocarcinoma	Infraclavicular sites: bronchus, stomach, breast, intestine, kidney, prostate, uterus
	Head and neck sites: ethmoid sinuses and thyroid gland
Undifferentiated or anaplastic carcinoma	Exclude lymphoma by immunocytochemistry
	Consider the above sites of carcinoma