Abstract
Neck lumps are among the most common presentations to primary care. The majority of cases are of a benign aetiology, however, the morbidity associated with delayed referral or incorrect diagnosis can be catastrophic in cases of malignancy. It is, therefore, vital that clinicians have a sound approach to this important presentation. The aim of this article is to equip clinicians with an understanding of regional neck anatomy and pathology, a systematic approach to neck masses, and guidance to follow for appropriate investigations and referrals.
The GP curriculum and neck masses
Understand how to recognise rarer but potentially serious conditions such as oral, head and neck cancer Know the epidemiology of head and neck cancers, including the risk factors, and identify unhealthy behaviour Identify symptoms that are within the range of normal and require no treatment such as small neck lymph nodes in healthy children and ‘geographic tongue’
Neck lumps may be incidental on examination or reported by a concerned patient. The list of differentials for neck lumps appears broad, but can be skilfully narrowed down based on the anatomical site and features of the mass. Invariably, this knowledge, along with that of differing neck pathologies, can be used to underpin a diagnosis. The succeeding step would be to utilise laboratory, radiological or invasive investigations to clarify the diagnosis.
The clinical approach will differ based on certain factors, including the age of the patient. Neck lumps in children are usually of a reactive nature. However, an adult with lateral neck lumps should not be subjected to prolonged observational periods or trials of antibiotics. The diagnostic approach in such instances must be tailored to exclude malignancy. If there are any doubts in diagnosis, or if malignancy is suspected, a 2-week fast track referral to a head and neck surgeon is warranted. There is strong evidence to suggest that delayed referral, and thus diagnosis and therapeutic involvement, is associated with poorer outcomes in head and neck cancers (O'Brien, Smith, Soong, Urist, & Maddox, 1986). The subsequent treatment of any confirmed malignant process requires a multidisciplinary approach.
Relevant anatomy
An in-depth understanding of the anatomy of the neck and subsequent pathologies that may arise in such locations is imperative to making a provisional diagnosis. Consideration of benign anatomical variations would also be necessary in carrying out a thorough assessment.
Triangles of the neck
The borders of the anterior triangle consist of three structures: inferior aspect of the mandible, the anterior aspect of sternocleidomastoid, and the midline (Fig. 1). Palpable structures within the midline (moving inferiorly) include the hyoid bone, thyroid cartilage, cricothyroid membrane, cricoid cartilage and the trachea. Although a normal thyroid gland is usually impalpable, the isthmus lies over the first two tracheal rings, with the right and left lobes overlying the thyroid and cricoid cartilages. The posterior aspect of the sternocleidomastoid, anterior aspect of the trapezius and clavicle form the borders of the posterior triangle.
Triangles of the neck.
Lymph nodes
As well as having an understanding of the anatomy of the neck, it is important to be able to recognise where lymph nodes may be located in this area. Levels at which lymph nodes sit can be categorised and later inform any staging and treatment planning of malignancy (Fig. 2).
Illustration of anatomic lymph node levels.
Lymph node metastasis reduces survival from squamous cell carcinoma (SCC) of the head and neck by around 50%, and is thereby the most significant prognostic factor (O’Brien et al., 1986). The number of levels containing nodal metastasis and the size and number of nodes has been shown to correlate with distant metastasis in head and neck cancer (Li et al., 2009).
Assessment
The following factors are extremely important when eliciting a history of a neck lump:
The age of the patient Duration of symptoms The anatomical location
Reactive lymphadenopathy resolution can take up to 6 weeks, and thus only a node persisting beyond 2 weeks should require further evaluation (National Institute for Health and Care Excellence (NICE), 2015). A benign reactive lymph node is a commonly encountered phenomenon in paediatric practice, but less so in adults. Other suspicious features requiring urgent attention include masses more than 2 cm in diameter, firm or rubbery in consistency and with a fixed mobility (NICE, 2015). Solitary small lymph nodes less than 1 cm in diameter can be located within the neck, axillary and inguinal regions in adults, however, any progressive enlargement should raise suspicion.
Lymphadenopathy in children is mostly due to infective causes, with ENT malignancies being rare in this age group (Coughlin, 2009). Children commonly present with a brief history of painful adenopathy alongside symptoms of an infective process. An important detail, other than the short duration, is that the lump is painful. This suggests infective processes such as acute infective lymphadenitis, infected branchial cyst or a haemorrhagic thyroglossal cyst, but steers away from a diagnosis of malignancy. Non-tender nodes can also be suggestive of subclinical viral pathology, and can also be remnants of previous infections.
The main malignancy that serves as a caveat in paediatric patients is Hodgkin’s lymphoma. A non-sinister diagnosis should be made tentatively in a child presenting with multiple nodes. In such instances, the axillary and inguinal areas must also be palpated, as well as the abdomen to elicit organomegaly. Congenital masses, are often, but not always, present from birth or in the infantile period. Branchial cysts usually present in young adults in their twenties (Thandar & Jonas, 2004). Thus, a history of coughs, colds, recent travel, dental work, cat scratches or insect bites in a child should be sought, combined with any systemic signs (NICE, 2016). In children, systemic diseases that may cause cervical lymphadenopathy include Kawasaki’s disease, Juvenile Kukichi–Fujimoto disease, Rosai–Dorfman disease and Langerhans Cell Histiocytosis (Coughlin, 2009).
Clinical evaluation in adults is tailored to exclude malignancy, and middle-aged individuals with a history of alcohol consumption and smoking should be presumed to have a mucosal malignancy of the oral cavity, oropharynx or laryngopharyngeal region unless otherwise proven. The risk of such individuals is 35-fold that of a teetotal non-smoker, 10-fold in smokers and six-fold in drinkers consuming more than two units of alcohol a day (Mashberg & Samit, 1995). Head and neck cancers may cause localised obstruction or infiltrate surrounding nerves such as the vagus, for example, causing referred ear pain.
Red flag signs and symptoms.
Adapted from NICE (2015).
Examination
Likely diagnosis of neck lumps from examination findings.
Investigations
Investigations are usually tailored based on the findings of the history and examination, coupled with the age of the patient. Thyroid, salivary masses or persistent lumps existing beyond a 4–6 week period warrant further investigation. Routine bloods provide marginal benefit, with the exception lymphocytosis on a full blood count in addition to viral serology: Paul Bunnell Monospot for Epstein–Bar Virus (EBV) and serology for toxoplasma/cytomegalovirus (CMV) or HIV (Thandar & Jonas, 2004). Thyroid function tests are routinely indicated in any individual presenting with a thyroid lump. An unexplained neck lump in a patient older than 45 years, a persistent and unexplained neck lump or thyroid mass should be referred for further assessment on the suspected cancer pathway (2-week wait) (NICE, 2015).
Within a community environment, an ultrasound scan of neck lumps may be requested prior to referral. Upon referral to secondary care, a computed tomography (CT) scan, fine needle aspiration and cytology (FNAC) and any further relevant imaging can be organised. CT and FNAC are two of the most important investigations in the evaluation of neck lumps (Thandar & Jonas, 2004). A contrast-enhanced soft tissue CT scan clearly defines any neck lumps, while also providing information on local structures/primary sites in the instances where nodes are metastatic (Som, 1992). This may also be utilised as an adjunct to FNAC when difficulties arise in localising the mass.
Ultrasonography is the imaging modality of choice for assessment of thyroid masses. It determines whether the mass is a solitary nodule, or part of a multi-nodular goitre. It is also useful in performing FNAC of the thyroid. Its ease of access, low cost and absence of ionising radiation are among its benefits (Haugen et al., 2015).
Chest X-rays are an integral aspect of a diagnostic work-up in patients presenting with neck lumps, and in particular when malignancy is suspected. It is also useful in instances where an inflammatory cause is suspected (tuberculosis (TB) and sarcoidosis, presenting with bilateral hilar lymphadenopathy) (Thandar & Jonas, 2004).
Magnetic resonance imaging (MRI) is utilised when accurate information about soft tissue pathology is required, with enhancement further provided by gadolinium contrast. It is particularly beneficial in assessing vascular malformations (SIGN, 2006). It demonstrates none of the risks associated with ionising radiation, but its use is limited by cost and limitations in patients with pacemakers, aneurismal clips and metallic debris within the eye.
Positron emission tomography is particularly useful in evaluating metastatic disease with an unknown primary, or tumour recurrence (Bailet et al., 1992). It is not a first line investigation.
Whether history and examination are highly suggestive of a diagnosis or not, the information obtained from a FNAC should not be underestimated. The procedure reliably distinguishes between cystic and solid lesions, and is often diagnostic in malignancy. It is a relatively safe/accurate procedure with a high sensitivity and specificity, but it should only be undertaken if the results are likely to influence treatment (Thandar & Jonas, 2004). Complications include tumour-seeding (extremely rare, minimised by utilising a 21G needle), and a localised haematoma (Thandar & Jonas, 2004). The presence of squamous cells in both branchial cysts and necrotic metastases makes differentiation difficult (Stanley, 2002). An associated false negative rate occurs with FNAC, and thus suspicious masses should always be referred for comprehensive work-up. In children, the false negative rate with FNAC can be as high as 55% (Hoperia, Larin, Jensen, Bauer, & Vasko, 2010).
Excision biopsy is a procedure where the entire abnormality or area of interest is removed. This is sometimes used when assessing abnormalities of the lymph nodes.
Congenital neck lumps
Thyroglossal cysts
Thyroglossal cysts are remnants of the tract via which the thyroid gland descends into the neck from the foramen caecum. This usually involutes in utero, but can present as a painless cystic swelling below or at the level of the hyoid bone within the midline. It is not unusual for such masses to be found as superior as the tongue base, or as inferior as the suprasternal notch. During examination, its classic movement is superiorly on protrusion of the tongue or swallowing. They account for three-quarters of all congenital abnormalities within the neck, and although found in all age ranges, are more common within the first decade of life (Bychkov, 2016). They have a tendency to present following an upper respiratory tract infection. Surgical intervention is usually always indicated to prevent future infections.
Soft tissue tumours (lipomas, dermoid cysts)
Superficial intra- or subcutaneous masses may be either dermoid cysts or lipomas. The classic occurrence of dermoid cysts is within the midline, at points of fusion or locations of trauma/surgery. Diagnosis and treatment of both pathologies is predominantly by means of surgical intervention under local anaesthesia (Salam, 2002). In the instances where such lesions are localised within the vicinity of the eyes, nose or forehead, a CT/MRI is usually indicated to exclude connections with a meningocele or encephalocoele.
Cystic hygromas
A cystic hygroma is a lymphangioma, arising from lymphatic channels within the neck. They present in most cases as a smooth, painless cyst within the posterior cervical triangle or supraclavicularly. They are usually soft, fluctuant and transilluminable masses lying deep to the skin. They are almost always first noted within the second year of life, and rarely in adulthood. It is the commonest lymphatic malformation in children. They may expand rapidly in size due to haemorrhage or upper respiratory tract infection. In the event where they do not regress spontaneously, complete surgical excision is indicated, which can be problematic due to numerous satellite extensions and association with cranial nerves (Acevedo, 2015).
Branchial cysts
Branchial cysts are vestigial remnants of the branchial apparatus, most commonly the second branchial cleft. They present as smooth, fluctuant and non-tender masses within the lateral neck, anterior to the sternocleidomastoid muscle. They can become symptomatic at any age, but a diagnosis is usually made within the first two decades of life. Diagnosis is more apparent in the presence of a sinus tract, associated with intermittent discharge (Hong, 2015). Treatment is surgical, with any inflammation treated prior to intervention.
Vascular malformations
Hemangiomas are considered congenital, due to their presence from birth (10% of all infants) (Hoornweg, Smeulders, Ubbink, & van der Horst, 2012), or development within the first decade of life. They are easily distinguishable from other masses by their venous tinge, warmth, refilling ability post compression and thrill. Diagnosis is by means of angiography, but this is rarely warranted. An observational approach is taken with such masses, given their ability to spontaneously involute. Features warranting further intervention include rapid enlargement, vital organ involvement, thrombocytopaenia, anaemia, microangiopathic haemolytic anaemia, and coagulopathy. Treatment is limited to those patients with life-threatening malformations or those that will have a detrimental effect to vital functions. It is comprised of steroids, Interferon α and surgery.
Plunging ranula
Ranulas (named after their blue translucent appearance) typically present as fluctuating cystic swellings on the floor of the mouth, usually as a result of sublingual gland mucous extravasations (Gupta & Karjodkar, 2011). These can rarely rupture through the musculature in the floor of the mouth, and deposit within the submandibular triangle or pierce through the mylohyoid muscle – thus a plunging ranula. Surgery is usually curative, and consists of excision of the sublingual gland via transoral excision.
Inflammatory or infective
Lymphadenopathy
Cervical lymphadenopathy is very common in children and young adults. It is usually related to a self-limiting upper respiratory tract infection and can persist for several months.
Acute lymphadenitis
Acute lymphadenitis can be bacterial (e.g. Staphylococcus and Streptococcus) or viral (e.g. Epstein–Barr virus, mumps, toxoplasmosis, CMV). If there was recent exposure to cats then Bartonella henselae titres should be taken for cat-scratch disease.
Mycobacterial lymphadenitis
Mycobacterial lymphadenitis typically has a more indolent onset and does not fully respond to antibiotic therapy. Atypical infection (usually Mycobacterium avium-intracellulare) is more commonly seen in children. Typical mycobacterium infection (e.g. TB) is more commonly seen in adults. It can be associated with systemic and respiratory symptoms.
HIV infection
One of the initial stages of HIV infection is lymphadenopathy and this commonly occurs in the cervical region. HIV should be tested for in patients with relevant risk factors.
Acute sialadenitis
Acute infection of the salivary glands can be bacterial or viral. It is more common in the elderly and most frequently seen in the parotid glands (Fig. 3). Bacterial sialadenitis may cause significant swelling and can lead to airway compromise. Most cases resolve with symptomatic management with hydration, analgesia and gland massage. Empirical broad-spectrum antibiotics should be given if infection persists or if the patient develops a high fever. Surgical drainage is required if an abscess develops.
Eighty-four-year-old lady presenting with submandibular swelling and diagnosed with acute sialoadenitis.
Viral sialadenitis is commonly due to mumps infection, typically seen in children and its classic presentation is with bilateral parotid swelling. Autoimmune sialadenitis is associated with Sjogren’s syndrome, in which features include parotid enlargement, dry eyes and mouth (Fazio & Emerick, 2013).
Sialolithiasis
A calculus in the parotid or submandibular gland can cause blockage of the draining duct, resulting in rapid swelling. Wharton’s duct, located adjacent to the frenulum, is affected in 80–92% of cases (Fazio & Emerick, 2013). The onset of swelling is typically associated with eating and drinking.
Sialadenosis
Sialadenosis is a non-neoplastic, non-inflammatory asymptomatic swelling of the salivary glands. It primarily affects the parotids and may be idiopathic or associated with systemic illness, e.g. diabetes, liver disease, beriberi disease, chronic malnutrition (Fazio & Emerick, 2013).
Thyroiditis
Hashimoto’s thyroiditis is the most common cause of an inflammatory goitre. Autoantibodies to thyroid peroxidase and/ or thyroglobulin cause gradual destruction of the follicles of the gland, due to infiltration of lymphocytes. There is usually an initial period of hyperthyroidism before the subsequent hypothyroidism.
Inflammatory masses usually tend to resolve spontaneously, although a single course of broad-spectrum antibiotics with subsequent reassessment within a fortnight is also a reasonable management plan.
Neoplastic: Benign
Lipomas, haeamangiomas, neuromas and fibromas are benign neck lumps. These are characterised by slow growth and lack of invasion.
Thyroid nodule
The majority of thyroid nodules are benign. Features suggesting a more sinister nodule include extremes of age, exposure to ionising radiation, family history of thyroid cancer, large nodule, hard nodule, fixed nodule, hoarse voice and enlarged cervical lymph nodes (Haugen et al., 2015). These patients should be referred urgently to an ENT surgeon for further investigation.
Salivary gland tumours
A pleomorphic adenoma is the most common type of benign salivary gland tumour. The majority of salivary gland tumours (70%) occur in the parotid gland (Speight and Barrett, 2002). Surgical excision is the treatment of choice. Facial nerve involvement is suggestive of malignancy and should be investigated immediately.
Carotid body tumours
Carotid body tumours are rare benign tumours of the carotid body neural plexus. They develop within the adventitia of the medial aspect of the carotid bifurcation. A carotid body tumour usually presents with a painless pulsatile mass and can only be moved side to side, as it is vertically fixed. A bruit can be felt; however, absence of a bruit does not rule it out. Due to the close proximity of the tumour to lower cranial nerves (such as the hypoglossal, glossopharyngeal, recurrent laryngeal, spinal accessory or sympathetic chain), growth of the tumour can lead to invasion of these nerves (Gad et al., 2014).
Neoplastic: Malignant
SCC
SCC is the most common cause of a malignant neck lump (Fig. 4) (Sanderson & Ironside, 2002). A malignant neoplasm in the neck can arise as a primary (e.g. adenocarcinomas, lymphoma, sarcoma), or more commonly, from metastatic spread from the upper aerodigestive tract.
Eighty-eight-year-old man with a malignant neck lymph node.
Lymphoma
The enlarged lymph nodes are typically ‘rubbery’ in consistency and the patient typically experiences constitutional symptoms such as weight loss, night sweats and malaise. Lymphoma is the most common cause of a malignant neck lump in children and therefore should always be considered (Coughlin, 2009). Generalised lympadenopathy in children should be assessed with an urgent full blood count and unexplained lymphadenopathy should be considered for referral (NICE, 2017). A history of constitutional symptoms or other nodal involvement should be taken.
Unexplained lymphadenopathy in adults should raise suspicion of lymphoma. A history of constitutional symptoms should be taken and referral should be considered (NICE, 2017). Adults with generalised lymphadenopathy should have an urgent full blood count and those with supraclavicular or persistent cervical lymphadenopathy should have an urgent chest X-ray (NICE, 2017).
Adenocarcinoma
Adenocarcinoma is a less common cause of a malignant neck lump. These can arise as a primary tumour from glandular epithelium present in the salivary glands.
Thyroid cancer
Papillary carcinomas are the most common type of thyroid cancers (88%) followed by follicular carcinoma at 9% (Davies & Welch, 2006). The incidence of cancer increases with exposure to ionising radiation and a positive family history.
Salivary gland tumours
Salivary tumours include mucoepidermoid, adenoid cystic, adenocarcinoma and SCC. Features suggestive of malignancy include pain, facial nerve palsy, rapid growth in size of mass and a fixed mass (Mehanna, McQueen, Robinson, & Paleri, 2012). Treatment is with surgical excision.
Post-traumatic
Haematomas can develop following trauma or surgical intervention (Fig. 5). Other possible causes that should be considered are seromas and abscess formation.
Haematoma following hemi-thyroidectomy.
Conclusion
It is vital for clinicians to have good understanding of neck mass pathologies and the implications that these can have on their patients. The anatomy, history and examination of a neck lump can provide sufficient information to either narrow down options or make a diagnosis. An amalgamation of this information is presented in Table 2. From these differentials, further imaging or referral to a head and neck surgeon can be carried out.
KEY POINTS
Diagnosis can be made in most cases through a careful history and examination of the head, neck and skin Neck lumps are categorised into three distinct groups: congenital/developmental, inflammatory and neoplastic Red flag signs and symptoms for cancer of the head and neck include persistent unexplained hoarseness, an unexplained lump in the neck, unexplained ulceration in the oral cavity lasting for more than 3 weeks, unexplained thyroid lump and unexplained lymphadenopathy Red flag symptoms of systemic illness include fever, night sweats, weight loss, persistent fatigue, breathlessness and pruritis Delay in referral, diagnosis and treatment is associated with a poor outcome FNAC and CT scanning of the head and neck are the two most useful investigations in neck masses and ultrasonography is the modality of choice in assessment of thyroid masses
