Summary

Common head and neck tumours include those of the nasopharynx, thyroid, larynx and hypopharynx, oral cavity and oropharynx, nasal cavity and sinuses, and the salivary glands. In the early stages these tumours may present with vague symptoms that are common to a wide variety of infective, inflammatory and other pathologies. Following a careful assessment of symptoms, it is important and essential to perform a thorough examination of the head and neck including careful inspection of the mucosa lined cavities. This may not be straightforward, and cancers that occur here can easily go undetected by those less familiar with the necessary special examination techniques required. While the prognosis for large or advanced cancers is poor, the prognosis for early or small cancers is good. Awareness of the symptoms and signs of common head and neck tumours, and the ability to perform a systematic examination of the head and neck, will improve the outcome of these patients.

摘要

常見的頭頸部腫瘤包括鼻咽部、甲狀腺、喉部、下咽部、口腔、口咽部、鼻腔、鼻竇部以及唾液腺的腫瘤。腫瘤的早期症狀無特異性、不顯著，這些症狀也常常見於感染性、炎性和其他性質的疾病。在對症狀做了認真評價後，對頭頸部做徹底檢查，包括對黏膜腔進行認真探查，是十分重要和必需的。病徵可能不易直接觀察到，其內的腫瘤也很容易被對相應的特殊檢查不熟悉的醫生漏掉。雖然體積大或晚期的腫瘤預後較差，但早期或體積小的腫瘤預後卻較好。了解常見的頭頸部腫瘤的症狀和體徵，以及能夠對頭頸部進行系統檢查，可以改善病人的最終結果。

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Introduction

There has been steady progress in the diagnosis and management of head and neck cancers, allowing cancer patients to survive longer with a better quality of life. Diagnostic techniques have improved with the development of rigid and flexible endoscopes. When coupled to a camera, the capture and processing of images vastly improves the documentation of disease. Advances with colour Doppler sonography, spiral computed tomography (CT) imaging, magnetic resonance (MR) imaging, and positron emission tomography (PET) imaging have revolutionised the way in which tissues can be examined.¹

Treatment options for head and neck cancers include surgery and radiotherapy. In addition to conventional radiotherapy, three-dimensional conformal radiotherapy, intensity modulated radiotherapy, and altered fractionation regimens are now employed. Three-dimensional conformal radiotherapy is a high precision technique that allows the radiotherapist to deliver a dose of radiotherapy that conforms to the three dimensional structure of the tumour while optimally protecting normal tissues. With intensity modulated radiotherapy, the dose intensity can be varied to deliver a higher dose of radiation to all parts of the three-dimensional volume of a tumour while minimising the radiation to healthy surrounding tissues, providing the best chance for cure with the least risk of complications. Altered fractionation regimens such as accelerated fractionation and hyperfractionation schedules offer therapeutic advantages over conventional radiotherapy by decreasing the opportunity for cancer cells to regenerate during treatment.² The role of neo-adjuvant, concurrent and adjuvant chemotherapy is becoming more defined. Surgical resection with complex free-flap reconstruction results in acceptable functional outcomes.

In the year 2000, there were over 21,000 new cases of cancer in Hong Kong.³ The number of new cancer cases is increasing at about 2% per year due to the aging population (age is the single most important risk factor to develop cancer), the expanding population exceeding the natural growth rate, and the westernised life style. Cancer is the leading cause of death in Hong Kong, accounting for over ¹/₃ of all deaths.

About 10% of all cancers occur in the head and neck. Nasopharyngeal malignancies account for 53%, thyroid cancer for 19%, oral cavity and oropharynx malignancies for 12%, larynx and hypopharynx malignancies for 12%, and the remaining 4% is made up of nasal cavity, nasal sinus and salivary gland malignancies (Table 1). The early detection of common head and neck malignancies significantly contributes to the quality of life and long-term survival of these patients.

Table 1: New cases of head and neck cancer in Hong Kong in 2000

Site Male Female Total   n n n (%) Nasopharynx 797 329 1126 (53%) Thyroid 84 313 397 (18.6%) Larynx 172 18 190 (9%) Tongue 68 53 121 (6%) Oral cavity 59 37 96 (4.4%) Hypopharynx 53 7 60 (3%) Nose and sinuses 30 18 48 (2.2%) Major salivary glands 26 46 46 (2.1%) Oropharynx 28 11 39 (1.7%) Total 1317 806 2123 (100%)           n = number

The common head and neck malignancies

New cases of common head and neck malignancies that occurred in Hong Kong in 2000 are listed in Table 1. Of 21,349 new cancer cases, 2123 were head and neck malignancies, accounting for about 10% of all new cancers in that year. Head and neck tumours include those that arise from mucosa in the head and neck, their regional lymph node metastases, and from the thyroid and salivary glands.

Nasopharyngeal carcinoma accounted for roughly 53% of head and neck cancers and for 5.3% of all cancers. It is the sixth most common cancer in Hong Kong. Nasopharyngeal cancer is the commonest cancer in young adult males between 20 and 44 years of age. In young adult women of the same age group, breast cancer is the commonest cancer, followed by thyroid and then nasopharyngeal cancers (Table 2). The crude incidence rate for nasopharyngeal carcinoma in Hong Kong is 24.3 per 100,000 persons for males and 9.7 per 100,000 persons for females. Thyroid cancer accounted for 19% of head and neck cancers and for 1.9% of all cancers. Larynx and hypopharynx cancers accounted for about 12% of head and neck cancers and for 1.2% of all cancers. Oral cavity and oropharynx cancers accounted for 12% of head and neck cancers and 1.2% of all cancers. Nose and sinus cancers accounted for 2.2% and salivary gland cancer for 2.1% of head and neck cancers (Table 1).³

Table 2: The 11 most common cancers in men and women in Hong Kong in 2000

Rank Male   Female   1 Lung (23.0%) Breast (19.9%) 2 Liver (10.4%) Lung (13.1%) 3 Colon (8.9%) Colon (10.3%) 4 Nasopharynx (6.8%) Rectum (4.9%) 5 Rectum (6.0%) Cervix (4.6%) 6 Stomach (5.9%) Stomach (4.0%) 7 Prostate (5.8%) Liver (3.8%) 8 Bladder (4.3%) Uterus (3.6%) 9 Oesophagus (3.7%) Ovary (3.5%) 10 Non-Hodgkinís lymphoma (2.9%) Nasopharynx (3.4%) 11 Non-melanoma skin (1.9%) Thyroid (3.2%)

Aetiology of head and neck malignancies

Carcinogens in contact with mucosa over a long period of time may lead to malignant transformation through stages of hyperplasia, dysplasia, carcinoma-in- situ and invasive carcinoma. Tobacco and alcohol are the two most commonly used substances that do this in the head and neck region. Exposure to radiation, in the form of background radiation, diagnostic x-rays or radiotherapy may cause cancer, especially thyroid cancer. The understanding of the complex role that inherited or acquired genetic abnormalities play in cancer, such as an abnormal p53 gene, is becoming clearer. The p53 protein is encoded by the p53 gene. Activated p53 protein acts as a checkpoint in the cell cycle, either initiating or preventing programmed cell death (apoptosis). The p53 protein has a tumour suppressor function by inducing cell growth arrest and cell death and so guards against cell hyperproliferation. The p53 protein is able to sense DNA damage and mediate the normal cellular response to DNA damage. Disruption of p53 activity allows diseased cells to multiply and in this way a tissue progresses from being benign to malignant.⁴

There is a strong association between Epstein-Barr virus infection of nasopharyngeal mucosa and nasopharyngeal carcinoma.⁵ Similarly, human papilloma virus may be associated with laryngeal carcinoma. There may be an association between the low consumption of fresh fruit and vegetables, a western diet, and cancer.⁶

Symptoms of common head and neck malignancies

Common symptoms of head and neck cancer include an ulcer that does not heal, a lump, difficulty in swallowing and persistent hoarseness.⁷ Pain may indicate a cancer and its source should always be identified. Symptoms in the mouth include an area of thickened or ulcerated mucosa, a lump or mass, and blood stained sputum and pain. Larynx and hypopharynx cancers present with hoarseness, difficulty in swallowing or choking, referred pain to the ear and, later, with airway narrowing. Nasal obstruction or blood stained nasal secretions may indicate a nasal, sinus or nasopharyngeal cancer. Any lump in a salivary gland or in the neck needs further investigation, as does a nerve palsy. Unfortunately, no symptom or symptom complex is strongly correlated with early head and neck cancer.⁸

Basic examination of the head and neck

The family physician should be able to thoroughly inspect readily accessible mucosal surfaces of the head and neck with basic equipment. The oral cavity and oropharynx are inspected with a headlight, to free up both hands, and two tongue depressors, which allow the mucosa to be stretched and examined. The tongue is palpated, as is the floor of mouth, with a bimanual technique. The anterior nasal cavity is examined with a headlight and Thudicum nasal speculum. The nasopharynx can be inspected with a nasopharyngeal mirror and tongue depressor, and the hypopharynx and larynx with a laryngeal mirror. A flexible or rigid endoscope will greatly augment the examination, especially of the nasal cavities, pharynx and larynx. The salivary glands are usually soft, a firm mass within them may be malignant. All regions of the neck must be carefully palpated for lymph nodes, whose number, size, consistency and position should be noted. An enlarged lymph node is often the presenting feature of a head and neck cancer.

Signs of common head and neck malignancies

Changes in the mucosa, such as the presence of white or red patches (leukoplakia or erythroplakia), thickening, ulceration or a growth may indicate malignancy. More advanced tumours present with induration, ulceration and fixation. Look for asymmetry and swellings. Identify the source of blood the patient may have produced. Note any loss of function. The source of pain should be sought. Palpate for masses and lumps. Clinical signs may be insufficient for the detection of early head and neck cancer because they are only weakly predictive.⁷

Investigating an abnormal finding

As the diagnosis of a malignancy can only be made histologically, a biopsy of any abnormal mucosa should be done. Bleeding disorders should be excluded and then accessible lesions biopsied under topical or local anaesthetic. Other lesions may require a general anaesthetic. Depending on the site of the malignancy, appropriate radiological imaging should be done to stage the malignancy and to plan therapy. Advances with colour Doppler sonography, CT imaging, MR imaging, and PET imaging have revolutionised the way in which tissues can be examined.¹ Colour Doppler sonography increases the ability of the sonographer to differentiate reactive from malignant lymphadenopathy based on the nodal vascular architecture.⁹ The advantages of spiral CT scanning include faster scanning with reduced examination time, greater accuracy especially for small lesions, and better images. The data set is three-dimensional, allowing multiplanar and 3D reconstructions. PET imaging is very accurate in differentiating benign from malignant tissues, including primary, metastatic and recurrent lesions, because it quantitatively measures the biochemical and functional activity in living tissue. Malignant tissue is metabolically more active than non-malignant tissue.

Most small or early head and neck cancers are usually curable with either surgery or radiotherapy. This emphasises the need for their early detection and referral to a specialist or specialist team.

Nasopharyngeal tumours

The early symptoms of nasopharyngeal carcinoma (NPC) include blood stained nasal secretions and saliva, and unilateral hearing loss and tinnitus. The commonest presenting symptoms of nasopharyngeal carcinoma are a neck mass (43% of cases), nasal symptoms (30% of cases), and ear symptoms (17% of cases).¹⁰ With special training and specific instruments, the nasopharynx can be inspected with a nasopharyngeal mirror or with a nasal endoscope. Examination of the nasopharynx will usually reveal an exophytic, ulcerating or asymmetrical mass. The mass can be biopsied through the nasal cavity under topical anaesthesia to confirm the diagnosis. For less obvious cases or as a screening procedure, blood can be taken for the EBV IgA-VCA titre, and if positive, the EBV IgA-EA titre or serum EBV DNA concentration is determined. The sensitivity of an IgA-VCA titre of 1/10 for diagnosing NPC is 81% and costs about HK$80. In order to improve the accuracy of screening, the IgA-EA titre is determined in cases with a positive IgA-VAC result. Although IgA-EA is used to eliminate the false positive IgA-VCA results, its sensitivity in detecting NPC is only 70%. Thus the combination of IgA-VCA and IgA-EA in a screening setting may still lead to a proportion of true cases being missed. This limitation is overcome by using EBV DNA, which has a 95% sensitivity for detecting NPC. Although the cost of the EBV DNA test is high, about HK$600, it can be used in those cases with a positive IgA-VCA result, and will eliminate about three quarters of the false positive IgA-VCA results. The combination of IgA-VCA titre and EBV DNA concentration has a sensitivity of 99% and a specificity of 96-98% for the detection of NPC.¹¹

Once the diagnosis is suspected or confirmed, the patient should be referred for specialist management.

Thyroid tumours

Masses in the thyroid gland usually have no presenting symptoms. They are often diagnosed on routine examination of the neck although, when they get bigger, they can present with hoarseness, dysphagia, and stridor or as an enlarged lower midline neck swelling which moves when the patient swallows. Most of the lesions are benign, but the main problem is to identify malignant masses which will require surgery. About 5% of adults have palpable thyroid nodules that can be detected by their doctor. Although fewer than 10% of these palpable thyroid nodules will prove to be malignant, all thyroid nodules should undergo an ultrasound examination and a fine needle aspiration for cytology (FNAC).¹² An ultrasound examination will determine if the nodule is solitary or multiple, if it has characteristics of malignancy, if there are any abnormal regional lymph nodes, and will guide the fine needle aspiration. There is an increased risk of malignancy if there is a family history, the patient is male, younger than 20 or older than 60 and if the nodule is solitary. Patients with a thyroid nodule should be referred for definitive assessment and management.

Laryngeal and hypopharyngeal tumours

Early tumours of the vocal cords present with hoarseness, and so usually the patient detects the symptom. Early tumours of the supraglottis and hypopharynx, unfortunately, are symptom free. When they are more advanced they present with blood stained saliva, haemoptysis, dysphagia, odynophagia, aspiration, choking, stridor, referred pain to the ear or a lump in the neck.

The hypopharynx and larynx can be inspected with a laryngeal mirror and headlight, but a flexible or 70 rigid endoscope gives a magnified view. Mucosal irregularity, vocal cord movement, symmetry and fullness of the larynx or pyriform fossae are noted. If an abnormality is suspected the patient should be referred for specialist management.

Oral cavity and oropharynx tumours

Symptoms in the mouth include an area of discoloured, thickened or ulcerated mucosa, a lump or mass, blood stained sputum and pain. The oral cavity and oropharynx are inspected with a headlight, to free up both hands, and two tongue depressors, which allow the mucosa to be stretched and examined for any abnormalities. The tongue is palpated and the floor of mouth is palpated with a bimanual technique. Look for changes of the mucosa such as the presence of white or red patches (leukoplakia or erythroplakia), thickening, ulceration or a growth. Early lesions have a favourable prognosis compared to more advanced tumours that present with induration, ulceration and later fixation to underlying deeper structures.¹³

Nasal cavity and sinus tumours

Common presenting symptoms of early nasal cavity or sinus tumours include nasal obstruction, epistaxis, and nasal discharge either purulent or blood stained. Symptoms of a more advanced tumour include unilateral swelling of the face, cheek or nose, diplopia or blurred vision, proptosis, nasal and cheek pain, headache and cranial nerve palsies.

The anterior nasal cavity can be inspected with a Thudicum nasal speculum and head light, revealing the anterior portion of the nasal septum, the anterior end of the inferior and middle turbinates and possibly the presence of large polyps or pus in the middle meatus. An adequate examination of the nasal cavity can be achieved with an endoscope, either flexible or rigid, when a topical vasoconstrictor is applied to the nasal mucosa. Familiarity with the anatomy of the nasal cavity will allow for abnormalities to be detected and a further plan of management formulated. This may include imaging and biopsy of any suspicious tissue.

About 55% of nasal and sinus tumours originate from the maxillary sinus, 35% originate from the nasal cavity, 9% originate from the ethmoid sinus, and 1% from the other sinuses or the septum.

Salivary gland tumours

The salivary glands are usually soft and not easily palpable when normal. The parotid gland lies in front of the ear and tragus, with the tail filling the angle between the mastoid process and ramus of the mandible. The submandibular gland is palpated bimanually, with one finger in the oral cavity palpating the gland through the floor of the mouth and the other hand palpating the gland through the skin. As a generalisation, the bigger the salivary gland, the smaller the chance is that a lump within it is malignant. About 80% of salivary gland tumours occur in the parotid gland, of which 20% will be malignant; 15% of salivary gland tumours occur in the submandibular gland, of which 50% will be malignant; and 5% of salivary gland tumours occur in the sublingual or minor salivary glands, of which 60% 80% will be malignant.¹⁴ A lump in a salivary gland needs further investigation by a specialist, including imaging and FNAC, before excision.

The special case of the lateral neck lump

An upper lateral neck lump in an adult patient is usually malignant and will usually be an enlarged lymph node due to metastatic carcinoma, often from a primary in the head and neck region that will normally be evident on examination. When a patient presents with a lateral neck lump, a thorough examination of all the mucosa of the head and neck region must be done to look for a primary tumour. If none is found, a fine needle aspiration of the lump should be done to confirm its origin. If carcinoma is found in the lump, the patient should be examined with imaging and under anaesthesia to look for a primary tumour. Until these investigations have been completed, neither an incisional nor an excisional biopsy of the neck lump should be done. Once the investigations have been completed and the nature of the lumps remains unknown then a focused approach is used to obtain tissue from the lump for histology. In general, the lump should be entirely excised with a margin of normal tissue if this can safely be done, taking into consideration potential further surgery when planning the skin incision. If the mass is determined to be unresectable clinically or radiologically, and there is little chance that the patient will undergo surgical resection or excision of the mass, then an incisional biopsy of the mass is performed to obtain a tissue diagnosis, taking into consideration the placement of the incision.

The role of the family physician

Cancer prevention

The family physician has a role in educating patients regarding cancer. The family physician should inform patients of the benefits of a healthy diet and lifestyle, of the dangers of tobacco and alcohol use, and of the early symptoms of cancer. Literature on this topic should be available to patients. Continuing medical education will keep the family physician up to date with current strategies. The family physician as well as the patient should know how to prevent and detect cancer.

Cancer detection

The detection of early oral cancer will lead to a 90% 5-year survival rate. Unfortunately 60% of oral tumours are detected when they are advanced, and have a 5-year survival of about 20%.¹⁵ There is much to be achieved in detecting these cancers earlier, with significant benefit for patients. During a patientís routine check-up visit, the family physician should ask about symptoms and look for signs that may indicate cancer. Any suspicious symptom or sign will need further evaluation until a diagnosis is reached, so that the correct treatment can be given or the patient referred appropriately. Timing of referral is important to avoid delayed detection. Patients under medical supervision are more likely to have their cancers detected early, supporting the value of surveillance by the primary care physician. The absence of definite early warning signs for most head and neck cancers suggests the need to develop essential screening criteria. Defining the population that is at high risk for head and neck cancer and subjecting it to an aggressive screening protocol is essentia.^l7 Currently, there is no evidence for the effectiveness of screening for oral cancer, or to recommend population screening for oral cancer.^l6 Despite this, many institutions and organisations recommend that all patients over 40 who are heavy smokers or drinkers should have the mucosa of their mouths examined yearly and be encouraged to reduce or stop tobacco and alcohol use.^l7

Cancer treatment

The family physician should maintain good lines of communication with both the patient and the treating specialist in order to ensure good management of the patient. The family physician should have a role in the counselling of these patients and in the provision of further psychosocial support if necessary. The patient should be cared for at all stages of their treatment.

Follow-up

Collaboration between the family physician and the specialist is essential for the rehabilitation and follow-up of the patient. The family physician should be involved in restoring the patientís quality of life, self-confidence and psychosocial well-being. In general, once a patient has had cancer, there remains a chance of a recurrence of the cancer or of a new cancer developing. For these reasons the patient is closely followed-up, at least for the first 5 years, and usually for life. If the patient has received radiotherapy, side effects and/or early and late complications may occur which will need recognition and management. Early complications include mucositis, an inflammation of the mucosa that can be severe and debilitating but which will eventually resolve. Longer-term complications include fibrosis of soft tissues and muscles, dryness of the mouth due to the sensitivity of glandular tissue to radiation, and maybe cranial nerve palsies. The patient may experience difficulties in mastication and swallowing. Management of the early and late sequelae of radiotherapy is usually done by the radiotherapist who treated the patient initially.

Palliation

When cancer is no longer curable, then treatment offered is palliative. There is a wide range of support that can be offered to patients with an incurable cancer. These patients can be offered a reasonable quality of life. The main symptom to control is often pain, with both pharmacological agents and psychological support. Depression is another important condition to manage well in cancer patients. The family physician should offer good emotional support and be compassionate.

Conclusion

The family physician needs skills in the prevention of head and neck malignancies through patient education and in their early detection. Once a cancer is suspected or diagnosed, the family physician must refer the patient to an appropriate specialist or specialist centre. The family physician should remain actively involved in all aspects of the patientís management.

Key messages

1. Cancer is the leading cause of death in Hong Kong. Ten percent of cancer occurs in the head and neck.
2. Head and neck cancer includes nasopharyngeal cancer (50%), thyroid cancer (20%), oral cavity and oropharynx cancer (10%), hypopharynx and larynx cancer (10%), and nasal cavity and salivary gland cancer (5%).
3. Tobacco and alcohol cause cancer. Always educate patients about these common carcinogens.
4. Head and neck cancer usually arises from the mucosa of the head and neck cavities. The salivary glands and thyroid gland can also become malignant.
5. Symptoms of head and neck cancer include pain, abnormal mucosa, an ulcer, a lump, difficulty in swallowing, hoarseness, nasal obstruction, blood stained saliva and blood stained nasal secretions.
6. Head and neck cancer often presents as a lump in the neck.
7. Carefully inspect the mucosa of all the head and neck cavities and biopsy any mucosa that looks abnormal.
8. Any lumps found during palpation of the neck should be investigated with ultrasound imaging and fine needle aspiration cytology.
9. Inspect the oral cavity and palpate the neck for lumps of patients who smoke or drink during their annual check-up.

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References