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.

Carcinoma in situ

Carcinoma in situ (TIS) is the stage of laryngeal carcinoma that may precede frank invasive malignant disease (Fig. 4.29). Histologically, the carcinoma does not breach the basement membrane. The affected area is excised under microscopic control using either microinstruments or a carbon dioxide laser. Clearly, all lesions should be biopsied before vaporization with the laser beam.

Fig. 4.29 Endoscopic photograph of a carcinoma arising from the left vocal cord.

Supraglottic laryngeal carcinoma

The sites of tumour in the supraglottic region (Fig. 4.28) include the lower portion of the epiglottis, false cords, ventricles and arytenoids. A very rich decussating lymphatic supply is present, so the frequency of regional nodal metastases is high and may be bilateral.

Clinical features

Malignancy in the supraglottis presents late owing to the potential space available for expansion. Voice changes tend to be late features. The patient may present with dysphagia, respiratory problems or a metastatic neck node.

Modern MRI or CT scanning provides excellent images for assessing the extent of the neoplasia (Fig. 4.30). Endoscopic laryngoscopy allows a full assessment of the extent of the disease, and the precise nature of the disease is confirmed by biopsy. The neck should be painstakingly palpated to detect the presence of any metastatic neck nodes.

Fig. 4.30 Axial CT scan of a supraglottic carcinoma (×). Note erosion of thyroid cartilage (××) and neck node metastasis (+++).

Glottic laryngeal carcinoma

Glottic laryngeal carcinoma is the most common malignant tumour of the larynx. It usually commences on the free vibrating edge of the true vocal cord and can spread in any direction. Anterior spread to the anterior commissure is a poor prognostic sign as this site is close to cartilage and allows further easy spread to the thyroid gland. Lateral spread into muscle will impair the mobility of the cords and may also reach lymphatic channels. The true cord is devoid of any lymphatic supply and, hence, lesions confined to the cord have an excellent prognosis.

Transglottic laryngeal carcinoma

Malignant squamous tumours can involve all three regions of the larynx at the time of presentation. Most have a period of symptomatic growth. Presentation is usually with the onset of respiratory obstruction or the appearance of neck nodes.

Emergency tracheostomy may be required because of respiratory difficulties. Radiotherapy is the preferred treatment in patients without regional nodal disease.

In the presence of neck disease, laryngectomy with radical neck dissection is the primary form of treatment. However, some clinicians regard primary radiotherapy to the larynx and neck as the correct first step in management, with surgery reserved to salvage residual or recurrent disease.

Complications of total laryngectomy

The main problems associated with total laryngectomy are shown in Table 4.10.

Table 4.10 Complications after total laryngectomy

Pharyngocutaneous fistulae

Pharyngocutaneous fistulae are tracts connecting the pharynx to the skin of the neck. They may be multiple and leak saliva (Fig. 4.31). There is an increased risk of developing such fistulae if the surgical technique is poor, preoperative radiotherapy has been given and the patient is poorly nourished. The majority of fistulae will heal spontaneously over a period of weeks. However, those associated with a loss of tissue will require repair with skin or muscle flaps.

Fig. 4.31 Pharyngocutaneous fistulae. The inferiorly placed hole is an end tracheostome after laryngectomy. The superior defect is a pharyngocutaneous fistula: note the evidence of saliva accumulating in the defect.

Recurrence of disease

Recurrence of disease in the end tracheostome has a poor prognosis (Fig. 4.32). It may be due to implantation of tumour cells during the primary laryngectomy, or a new primary cancer at the stomal site. The majority of such patients will be managed as terminal cases. Palliative radiotherapy and surgery may alleviate the distressing symptoms of respiratory distress and fungating skin mass.

Fig. 4.32 An end tracheostome with evidence of florid recurrent carcinoma.

Oesophageal speech

Oesophageal speech is based on a portion of the oesophagus called the pharyngo-oesophageal (PE) segment. It involves the rapid swallowing of air which is trapped in the upper part of the oesophagus (Fig. 4.33). This air reservoir is employed to vibrate the PE segment by controlled contraction of the thoracic and abdominal muscles. The phonatory sound produced is modified in the normal way by the resonators and articulatory mechanisms in the oral cavity and nose. In effect, the oesophagus has replaced the lungs as a small power source for initiating vibration. Only about 20% of laryngectomy patients achieve speech in this way. Oesophageal speech can only be acquired with long-term speech therapy and many patients never achieve a satisfactory quality.

Fig. 4.33 The principles of oesophageal speech after total laryngectomy.

Neoglottic speech

Various surgical techniques employ valved prostheses which are inserted to redirect pulmonary air through the PE segment. The prosthesis is placed in a surgically created fistula connecting the posterior tracheal and anterior oesophageal walls (Fig. 4.34). The one-way valve in the prosthesis allows air to flow into the PE segment when the stoma is occluded during exhalation. More recently, external valve housings have been used to overcome the tedium and inconvenience of finger occlusion. The major complication of surgical prosthetic techniques is the risk of a leak around the tracheo-oesophageal valve, allowing aspiration of food, drink and saliva.

Fig. 4.34 The principles of neoglottic speech after total laryngectomy.

Artificial larynx

Artificial larynxes (Fig. 4.35) work on the principle that air within the vocal tract can be put into vibration by an external battery-powered device. The instrument can be placed either against the skin of the neck or in the oral cavity.

Fig. 4.35 Artificial larynx. This is placed externally in the neck to generate vibration in the pharyngo-oesophageal segment.

Speech production in this technique sounds very mechanical, and it is difficult to reproduce alterations in pitch or loudness.

Aetiological factors

Tobacco smoke and alcohol have been implicated in hypopharyngeal carcinomas. However, despite an increase in tobacco consumption, the incidence of hypopharyngeal cancer does not appear to be rising.

A history of radiation exposure may lead to malignant disease 20–30 years later. This was noted in thyrotoxic patients who were treated by localized radiotherapy to the neck before the development of drug therapy.

Paterson–Brown Kelly syndrome

There is a known association between sideropenic dysphagia and postcricoid carcinoma. Paterson and Brown Kelly described a syndrome (also known as Plummer–Vinson) comprising hypochromic microcytic anaemia, glossitis, koilonychia, splenomegaly and a postcricoid web with dysphagia (Fig. 4.51). It was thought the dysphagia resulted from the presence of the postcricoid web or sometimes a stricture. However, some patients do not demonstrate these obstructive lesions and it is thought likely that the dysphagia is due to muscular incoordination.

Fig. 4.51 Paterson–Brown Kelly (Plummer–Vinson) syndrome. A microcytic anaemia is associated with (a) koilonychia, (b) glossitis and postcricoid web.

Haematological abnormalities include a low serum iron and a raised total iron binding capacity. The haemoglobin is frequently low but may be normal. Some patients demonstrate poor vitamin B₁₂ absorption and a low serum vitamin B₁₂. The postcricoid web or a fibrous stricture can be demonstrated radiologically (Fig. 4.52).

Fig. 4.52 A barium swallow illustrating a postcricoid web.

Treatment of sideropenic dysphagia is directed towards correcting the haematological deficits by administration of iron and sometimes vitamin B complex. The dysphagia may resolve, but if it persists, dilatation will be necessary. All cases should be reviewed regularly as a proportion progress to develop postcricoid carcinoma.

Clinical features

The major clinical features of hypopharyngeal neoplasia are summarized in Figure 4.53. True dysphagia is usually a late feature, as the tumour has considerable space in which to grow before causing actual obstructive symptoms. Initially, the only complaint may be odynophagia (pain or discomfort on swallowing) or a feeling of soreness and pricking as food passes through the pharyngo-oesophagus.

Fig. 4.53 The major clinical features of hypopharyngeal neoplasia.

At the time of presentation, the dysphagia is usually severe and invariably associated with weight loss. Referred otalgia is common. Dysphonia results from either direct invasion of the larynx or vocal cord paralysis caused by involvement of the recurrent laryngeal nerve.

An isolated neck mass due to metastases may be the presenting feature and not accompanied by other symptoms. This is most likely in lesions of the piriform fossa, which has a very rich lymphatic supply.

Mirror examination may reveal obvious tumour if it is located in the mouth of the piriform fossa or the posterior pharyngeal wall. Postcricoid lesions lead to pooling of saliva in the hypopharynx. Vocal cord paralysis may also be present.

Investigations

A barium swallow may show an irregular filling defect of the mucosa (Fig. 4.54). A negative swallow in the presence of persistent feeling of something in the throat requires a formal pharyngo-oesophagoscopy. A chest X-ray may reveal the presence of a second primary cancer or show enlargement of the mediastinum and paratracheal region due to metastatic disease. The lesion should be biopsied and its extent mapped for treatment planning.

Fig. 4.54 A carcinoma of the hypopharynx showing a filling defect on barium swallow.

Management

Advanced hypopharyngeal tumours can be managed with chemoradiation if there are no neck nodes involved. Surgery can be employed for salvage if this fails or when the primary tumour has neck node metastases.

The standard surgical management for advanced hypopharyngeal tumours involves excision of the larynx and pharynx – total laryngopharyngectomy along with a neck dissection, often bilateral (Fig. 4.55). Reconstruction of the pharynx and oesophagus involves microvascular free-tissue transfer with a jejunal (small bowel) segment anastomosed to blood vessels in the neck. This type of surgery requires three surgical teams: one to resect the tumour, one to harvest the bowel from the abdomen and one to perform the reconstruction (Fig. 4.56). This surgery allows the patient to swallow an oral diet.

Fig. 4.55 Total pharyngolaryngectomy and bilateral neck node dissection for advanced hypopharyngeal cancer.

Fig. 4.56 Reconstruction with microvascular free jejunal tissue transfer.

Prognosis

The 5-year survival rates are about 15% with radiotherapy alone, but 30% overall for all forms of treatment.

Aetiology

Many factors have been implicated in the development of nasopharyngeal carcinoma. These include ingestion of salted fish, smoke from burning joss sticks, Chinese herbal medicine, cigarette smoking, and industrial smoke and chemicals.

The Epstein–Barr virus (EBV) may have a major aetiological role in nasopharyngeal carcinoma. The viral genome appears to become incorporated into nasopharyngeal mucosal cells which may then be triggered by some stimulant to initiate malignant change within the cell.

Clinical features

The majority of tumours arise in the fossa of Rosenmüller and can spread in any direction to produce a vast array of potential symptoms and signs (Fig. 4.57). A variety of cranial nerve lesions can occur. The involvement of the trigeminal nerve by superior extension into the foramen ovale manifests as facial pain and altered sensation in the face. Horner’s syndrome (meiosis, ptosis and anhidrosis) may result from involvement of the sympathetic trunk in the carotid sheath.

Fig. 4.57 Clinical features of nasopharyngeal neoplasia.

Invasion of the nasopharyngeal end of the Eustachian tube will give rise to otological symptoms such as hearing loss. In any adult Chinese with secretory otitis media it is essential to assume the presence of nasopharyngeal carcinoma until otherwise proven.

Investigations

The neoplasm may be seen on routine postnasal space mirror examination. A more valuable and reliable view will be obtained with the flexible rhinolaryngoscope or rigid nasoendoscope (Fig. 4.58). It may be feasible to perform a biopsy in outpatients. All other patients should be subjected to formal biopsy under general anaesthesia.

Fig. 4.58 Rigid endoscope used to visualize a mass in the nasopharynx.

MRI scanning is essential to assess the extent of the disease, particularly its potential extension to the skull base and pharyngeal spaces (Fig. 4.59). MRI is superior to CT at depicting involvement of soft tissue, but inferior to CT in showing bony destruction.

Fig. 4.59 MRI scan of a carcinoma of the right nasopharynx. Note involvement around the internal carotid artery (arrow).

EBV antibody titres are useful in assessing the response to treatment, and in detecting subsequent recurrences.

Aetiology

Smoking is the most important risk factor for nasal cancer. Certain other factors are known to be carcinogenic in the nose and paranasal sinuses (Table 4.13). Hardwood dust is a known factor in the development of adenocarcinoma of the ethmoid sinuses. Inhalation of nickel dust is implicated in nasal squamous cell carcinoma. Radiation exposure of the skin of the nose may induce malignant change. About 10% of cases of benign transitional cell papillomata are associated with transitional cell carcinoma.

Table 4.13 Aetiological factors implicated in cancer of the nose and sinuses

Clinical features

Clinical features are dependent on the precise location of the malignant tumour. For example, frontal sinus cancer is likely to cause orbital symptoms early in the disease. Nasal cavity lesions may present with nasal obstruction and epistaxis and cause ocular problems only in advanced cases.

The majority of malignant disease presents in the maxillary antrum, and this serves to illustrate the major symptoms and signs that may occur (Fig. 4.65).

Fig. 4.65 Symptoms and signs in neoplasia of the maxillary antrum.

Antral tumours usually present late, as the mass must grow to fill the sinus space before causing any major symptoms. Epiphora (watering eye) is due to invasion and blockage of the nasolacrimal duct. A denture that becomes ill fitting is not an uncommon presentation if the lesion grows inferiorly. Facial paraesthesia is due to involvement of the infraorbital nerve in the roof of the antrum, or the trigeminal nerve in the retroantral region. Trismus (reduced jaw opening) is a sign that the pterygoid muscles have been invaded by direct posterior extension.

Investigations

Histological confirmation of the malignant process is usually easy owing to the presence of a mass in the nasal cavity. The limits of the disease are most accurately determined by CT scanning, which can be performed in the coronal and axial planes, and provides useful information about soft tissue invasion (Fig. 4.66).

Fig. 4.66 CT scan of carcinoma of the maxillary antrum. The lesion has extended medially into the nasal cavity, anteriorly into the cheek and posteriorly into the retroantral region.

Management

The management of malignant disease of the nose and paranasal sinuses is dependent on the histology and the extent of the neoplasm. The majority of cases require full-dose radiotherapy with subsequent planned surgery. The use of systemic and local chemotherapy has been disappointing. Palliative therapy is extremely valuable, as without some form of treatment the disease is slowly progressive and cosmetically mutilating.

Most malignant disease in the nose and paranasal sinuses may be resected via the lateral rhinotomy approach or by performing a maxillectomy. Any superior extension of the disease into the region of the cribriform plate may be included in the resection by performing a craniofacial resection, i.e. by a combined approach from below and via a craniotomy from above (Fig. 4.67). If the eye is involved, an orbital exenteration will be necessary.

Fig. 4.67 Craniofacial resection. The area of resection includes the cribriform plate. The orbit and maxilla may be included in the resection.

Wegener’s granulomatosis

Wegener’s granulomatosis is a systematic vasculitis of unknown origin. It involves the respiratory tract and kidneys. The nose may be obstructed as a result of thickened mucosa and crusting. The patient is very unwell, despite the minimal nasal signs. Nasal biopsy may be unhelpful. The diagnosis is made by the presence of granulomata on chest X-ray, abnormal renal function and possibly a positive antineutrophil cytoplasmic antibody test (ANCA). Death occurs usually secondary to kidney failure. High-dose steroids improve renal function and relapses may be prevented with cytotoxic therapy such as cyclophosphamide. Renal transplant can be considered if treatment is successful.

Lethal midline granuloma (midfacial lymphoma)

Lethal midline granuloma is a T-cell lymphoma that results in a slow, progressive destruction of the nose and midface (Fig. 4.68). Treatment involves a curative dose of radiotherapy, combined with surgical excision of necrotic tissue.

Fig. 4.68 Lethal midline granuloma.

Benign pleomorphic adenoma

Virtually all benign pleomorphic adenomas occur in the parotid, with a small number arising in the submandibular gland. The patient usually complains of a painless, slowly enlarging lump in the retromandibular region (Fig. 4.69). The presence of facial pain or paralysis is indicative of a malignant neoplasm. Pleomorphic adenomas have a false capsule, so that simple enucleation is liable to leave residual tumour. About 10% of these adenomas show malignant tendencies, particularly if left for many years.

Fig. 4.69 A benign pleomorphic adenoma of the right parotid gland.

Treatment is by parotidectomy. This usually entails resecting the parotid tissue (in which the adenoma is located) superficial to the facial nerve (Fig. 4.70). Rarely, the tumour may arise in the deep lobe of the parotid and present as an intraoral swelling owing to expansion within the parapharyngeal space. In such cases a total parotidectomy with preservation of the facial nerve is performed. A margin of normal tissue should always be excised to ensure that all tumour projections from the main tumour mass are included in the resection.

Fig. 4.70 Principles of parotid surgery. A superficial parotidectomy removes all parotid gland tissue superficial to the facial nerve. The deep lobe of the parotid is located deep to the facial nerve; any mass lesion in this region will tend to expand towards the oropharynx causing medial displacement of the tonsil, as shown by the bold arrow.

Warthin’s tumour (adenolymphoma)

Warthin’s tumour is almost exclusively a disease of the elderly male and is frequently bilateral. It occurs only in the parotid. It presents as an ovoid, mobile, fluctuant mass, usually in the tail of the gland. Painful enlargement may occur in association with upper respiratory tract infections, owing to inflammation of the lymphoid tissue contained within the tumour.

Treatment is by surgical excision via a parotidectomy-type incision.

Mucoepidermoid tumour

Over 90% of mucoepidermoid tumours occur in the parotid gland. If arising from minor salivary glands, the palate is the most common site. This is the most frequently encountered salivary gland tumour in childhood.

The histological structure determines the behaviour of the tumour. Well-differentiated lesions behave as benign tumours, but an undifferentiated appearance is indicative of a high degree of malignancy with a propensity to local and systemic metastases. Intermediate degrees of malignancy are also encountered.

The histological pattern is only confirmed after parotidectomy. If the facial nerve is involved by disease it should be sacrificed. Postoperative radiotherapy is employed in all cases.

Acinic cell tumour

The acinic cell tumour predominantly arises in the parotid gland. It is extremely slow growing, but about 10% of cases give rise to metastases and a very small number of cases are bilateral. Treatment is by surgical excision with preservation of the facial nerve if it is free of tumour.

Adenoid cystic carcinoma

Adenoid cystic carcinoma is the most common of the malignant salivary gland neoplasms and tends to be sited in the submandibular, sublingual and minor salivary glands. The parotid is rarely affected.

The presenting feature is usually a swelling, often with pain of several months’ duration. Latterly, a neck mass may develop. The facial nerve is invariably compromised in tumours arising in the parotid (Fig. 4.71). The nerve involvement is probably due to the spread of cancer by perineural lymphatics, which is a feature of adenoid cystic carcinoma. Local metastases to lymph nodes are not infrequent. Distant metastases in the lung are also not uncommon.

Fig. 4.71 An adenoid cystic carcinoma of the parotid with involvement of the facial nerve.

Treatment is by wide local excision, e.g. excision of the hard palate or total parotidectomy. The facial nerve is sacrificed so as to include cancer cells that will have spread in perineural lymphatics. Grafting the facial nerve is possible by using the sural nerve. A cross-face anastomosis may be offered once the patient is deemed free of tumour. These tumours are radiosensitive but are rarely cured, with recurrences up to 10 years after treatment. The overall 5-year survival rate is about 60%.

Facial weakness

In parotidectomy, with an intact facial nerve, any facial weakness is usually temporary. It is related to pressure and trauma in the vicinity of the facial nerve. Coagulation diathermy may result in heat damage to the neural elements.

Facial weakness after submandibular gland surgery is due to damage of the mandibular branch of the facial nerve. It rarely recovers.

Anaesthesia of the ear

The great auricular nerve is almost always divided in parotidectomy and results in anaesthesia in the inferior half of the pinna and surrounding skin. The area of anaesthesia may diminish with time, but there is always some residual deficit, particularly of the lobule.

Salivary fistula

A salivary fistula is due to the overproduction of saliva from remaining parotid tissue. It invariably settles spontaneously with pressure dressings and suction drainage.

Frey’s syndrome

Frey’s syndrome or gustatory sweating is a result of severed parasympathetic fibres destined for the parotid gland rerouting into the skin. Thus, at meal times, the patient experiences pain, inflammation and sweating of the skin over the parotid region. If the symptoms do not settle spontaneously after 6 months, a tympanic nerve section to divide the secretory parasympathetic fibres as they traverse the middle ear may be beneficial.

Weakness and anaesthesia of the tongue

Weakness and anaesthesia of the tongue are due to trauma to the hypoglossal and lingual nerves, respectively, during submandibular gland excision. These complications are permanent (Fig. 4.72).

Fig. 4.72 Damage to the hypoglossal nerve causes atrophy of the ipsilateral tongue.

Squamous cell carcinoma

The most common tumour is squamous cell carcinoma. It presents with symptoms of otorrhoea, perhaps tinged with blood, and hearing loss. Facial paralysis occurs if the middle ear is invaded and the facial nerve compromised. Otalgia is present and increases with the progression of invasion. Squamous cell carcinoma is treated by mastoidectomy and removal of the parotid gland and temporomandibular joint. Postoperative radiotherapy should be given.

Ceruminomas

The term ceruminomas covers all benign and malignant tumours arising from ceruminous glands of the external meatus. (Tumours of the apocrine sweat glands are incorrectly termed as ceruminomas.) Adenoid cystic carcinoma is the most common type of ceruminoma. Radical wide excision surgery is required with postoperative radiotherapy. The prognosis is poor, with the patient dying from local and systemic metastases.

Pain control

Pain control can be affected by medical or surgical means. Palliative surgery may assist in pain control, e.g. radical neck dissection, where cure is unlikely. Extensive surgery is rarely indicated. The majority of patients will have satisfactory control of pain by medical treatment or by peripheral nerve block.

Respiratory difficulties

Respiratory difficulties may arise from physical obstruction of the airway, parenchymal disease in the lung (most patients have smoked heavily) or a feeling of breathlessness from anxiety.

Tumour invasion of the trachea or bronchi producing obstruction may require local radiotherapy or a bypass tube (tracheostomy, tracheal stent). Corticosteroids can temporarily improve oedema and buy time, and may be used with large doses of opiates if no intervention is planned.

Chest infections are common. Debilitated patients in bed who may have laryngeal overspill and aspiration are particularly at risk. Those with an existing tracheostomy have difficulty in clearing secretions from their chest.

In cases of anxiety, opiates or sedative antidepressant drugs are particularly useful.

Dysphagia

Difficulty in swallowing may be due to local causes such as malignancy or pain from candidal infection. The production of saliva is reduced after radiotherapy and, combined with poor action of the tongue or pharynx, may preclude a solid diet. It is usually possible with the help of a dietitian to discover the type and consistency of food that a patient finds easiest to swallow. Any candidiasis should be treated aggressively, and the addition of benzydamine helps many patients.

Mouth care is essential. This should include regular oral toilet. Sucking ice cubes helps to alleviate the feeling of thirst. Dysphagia may be improved by using a short course of corticosteroids.

With progression of the disease, the nutritional status deteriorates. Support in the form of tube or parenteral feeding may be discussed but is usually deemed inappropriate.

Speech problems

Communication difficulties are frequent and naturally will demoralize the patient. The problem is reduced somewhat by continuity of care, so that the patient is not constantly having to adjust to new carers.

It may be beneficial to seek the advice of a speech therapist. They can provide skills to improve communication and in some cases relevant mechanical or electronic aids to speech.

Miscellaneous problems

In the terminal stage of head and neck cancer, the patient may have a number of problems that should be addressed and managed to improve physical and psychological comfort (Table 4.22).

Table 4.22 Distressing problems in terminal head and neck cancer

Necrotic tissue and anaerobic infections combine to produce a foul odour. Treatment of the infection with metronidazole and regular wound toilet usually help to reduce the offensive smell. Fungating tumour is unsightly, frequently foul smelling due to infection and may bleed. Regular toilet is essential, but often these patients die from massive sudden haemorrhage, e.g. carotid blow-out (Fig. 4.79).

Fig. 4.79 A fungating tumour in the neck. The carotid artery lies in the depth of the mass and may result in a blow-out.

Wound and mouth care is mandatory. The regularity of toilet is more important than the methods or materials used (Fig. 4.80). Sponges soaked with antiseptic are very useful in cleaning dried saliva and food debris, and give a refreshing feel to the mouth.

Fig. 4.80 A poorly cared for mouth in a patient with terminal head and neck neoplasia.

The combination of regular administration of narcotics, recumbency and general cachexia increases the risks of developing constipation. This can be avoided by the use of aperients, e.g. lactulose.

Pressure skin necrosis occurs in cases of terminal cancer, resulting from a combination of poor nutrition, immobility, and loss of control of urinary and bowel sphincters. Good nursing care should prevent problems with pressure areas (Fig. 4.81).

Fig. 4.81 Sacral necrosis in a terminally ill patient.