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.

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.

Chemotherapy

Chemotherapy is rarely used as the sole modality of treatment. It has traditionally been reserved for patients presenting with advanced disease or in recurrent or residual disease after radiotherapy and surgery. Trials are ongoing to document whether chemotherapy employed in synchrony with radiation confers any increase in survival over radiotherapy alone.

The major side-effect of chemotherapeutic agents is their depressive action on haematopoiesis. This toxicity is increased in the presence of abnormal liver and renal function.

Surgery

Surgical excision will result in some degree of cosmetic and functional deficit. The deficit is related to the extent of ablation and the availability of reconstructive manoeuvres. Resection should be with a 2 cm margin of clearance from the tumour edge. Reconstructive options include local and regional pedicled flaps and microvascular free grafts.

Terminal care

Some patients have no prospect of cure owing to advanced disease or residual or recurrent disease. The dying phase is usually protracted. In order to be able to deal with such situations in a humane manner, the concept of terminal care has evolved. This provides physical and psychological support for patients in the terminal stage of their life (see p. 116).

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.

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.

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

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.

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.

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

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.

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).

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

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

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.

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 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

The occult primary in the head and neck is a rare entity. However, if after thorough clinical examination the primary lesion cannot be identified, positron emission tomography (PET) scanning can be useful in identifying the site (Fig. 4.26).

Fig. 4.26 PET scanning of the head and neck identifying an occult primary in the tonsil. A 53 year old man presented with an enlarged left neck node. Cytology of the node revealed squamous cell carcinoma (SCC) and no primary site was identified on full clinical examination including flexible endoscopy and head and neck MRI. PET CT demonstrates the SCC node (*) and is suggestive that the left palatine tonsil (+) is the primary site. Subsequent biopsies of the tonsil confirmed the primary carcinoma.

N2: bilateral neck nodes

The appearance of bilateral malignant metastatic neck nodes is a very poor prognostic sign. Such an event is more likely in primary tumours of the tongue base and hypopharynx. Serious thought should be given as to whether such patients require active treatment or active palliation. It is feasible to perform bilateral neck dissection as a single rather than staged procedure, preserving one jugular vein. Tying a single internal jugular vein results in a rise in intracranial pressure which is raised even further on tying the second side (Fig. 4.27). Morbidity and mortality related to the huge increase in intracranial pressure may be prevented by modification of the classic radical dissection, and drug intervention.

Fig. 4.27 Severe oedema of head and neck. This occurred after tying the internal jugular vein during a modified radical neck dissection when the other side had been operated on some years previously.