Neck SwellingsBMJ 2014; 348 doi: http://dx.doi.org/10.1136/bmj.g1078 (Published 23 January 2014) Cite this as: BMJ 2014;348:bmj.g1078
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Neck swellings are common findings that present in all age groups from many causes, ranging from congenital to acquired, from cysts, inflammatory, infective to neoplastic disease, encompassing any neck structures
In the community, the inflammatory lymph node is most common, whereas in hospital thyroid swelling or goitre is most frequently seen
It behoves all clinicians to understand the embryology and anatomy to aid with making the correct diagnosis thus allowing for appropriate management
There are more than 100 lymph nodes in each neck, and other organs or glands are singular!
Knowledge of patient's age, associated symptoms and anatomical location of the lump, is key to proceeding to treatment in General Practice, or is an indication for referral for further investigations, including imaging and surgery
Neck lumps in adults (over 40 years) should be considered malignant or at least have malignancy excluded by examination of the mucosal surfaces of the head and neck region, and a needle sample of lump be examined by a pathologist
Neck swellings are commonly encountered and present at all ages. The differential diagnosis of a neck mass is extensive. In the community inflammatory lymph nodes are most common, while in the hospital environment the thyroid swelling or goiter is most frequently seen. It therefore behoves all clinicians to understand the embryology and anatomy to aid with making the correct diagnosis and following an appropriate management algorithm.
Anatomy of the Normal Neck
The old surgical aphorism,
consider the anatomical structures and then the pathology that can arise from these
is never more appropriate than when one contemplates the causes of a lump in the neck.
Clinicians are taught to examine a patients' neck, its landmarks and contents, early in their undergraduate careers, and for the remainder of their clinical life should never have to reconsider what they have been taught. Patients are untaught, and hence when they get a symptom located to their neck, naturally palpate their own neck and that of others, and consider possible diseases diagnoses and become alarmed with what they can feel!
The normal glandular structures are consistent in their location; the thyroid gland is a bilobed structure located along the midline of the neck on either side of the trachea, above the sternal notch, and below the cricoid cartilage. The parotid gland is found in front of the ear or pinna, and extends from the cheek bone (zygoma) above, down and behind to the mastoid tip, below into the upper neck near the hyoid bone and forward onto the cheek for about 2–3 cms. The submandibular gland, is, located below the posterior half of the mandible, but not extending beyond the angle of the mandible, above the hyoid bone (Figure 1) (see Chapter 1).
Within each side of the neck there are located more than 100 lymph nodes, usually impalpable, and distributed mainly along the jugular chain, to be found within each of the 5 clinically and anatomically described levels in the anterior neck and in the single but divided level in the posterior neck. The posterior border of the SCM muscle separates the anterior neck from the posterior neck (Figure 2). While there are other major structures within the neck, such as nerves, blood vessels, muscles, cartilages and bones, knowledge of their anatomic outline should allow the examiner to consider any such abnormal enlargement or swellings in certain anatomic locations to be included within a differential diagnosis.
Reaching a Diagnosis
Reaching a diagnosis obviously requires some knowledge of the potential pathology. It is difficult to present an exhaustive list of the potential causes of a neck swelling, but a simple classification is tabulated below (Box 1).
More Common Causes of Neck Swellings
Congenital: lymphangiomas, dermoids, thyroglossal cysts
Developmental: branchial cysts, laryngoceles, pharyngeal pouches
Skin and subcutaneous tissue: sebaceous cyst, lipoma
Thyroid swellings: multinodular goitre, solitary thyroid nodule
Salivary gland tumours: pleomorphic adenoma, Warthins tumour
Tumours of the parapharyngeal space: deep lobe parotid, chemodectoma
Reactive neck lymphadenopathy: tonsillitis, glandular fever, HIV
Malignant neck node: carcinoma metastases (unknown primary), lymphoma
In practical terms, the diagnosis is reached from the patients' age, the history, location and physical examination of the neck (Box 2), followed by a thorough examination of the upper aerodigestive tract and the results of appropriate tests and investigations.
Reaching a Diagnosis
Examination of the lump
Examination of the head and neck
The first consideration should be the patient's age group (Table 1). In general, neck masses in children and young adults are more commonly inflammatory than congenital and only occasionally neoplastic. However, the first consideration in the older adult should be that the mass is neoplastic. The “rule of 80” is often applied as a useful guide. In adults 80% of non-thyroid neck masses are probably neoplastic and 80% of these masses are malignant. This statement probably refers to masses over 2 cm in diameter, in patients over 35 years of age, and for clinicians who are not regularly seeing patients with neck masses. A neck mass in a child, on the other hand, has a 90% probability of being a benign condition of which 55% are congenital.
Onset and duration of symptoms is one of the most important historical points. Inflammatory disorders are usually acute in onset and resolve within 2–6 weeks. Cervical lymphadenitis is often associated with a recent upper respiratory tract infection. In contrast, congenital masses are often present since birth as a small, asymptomatic mass which enlarges rapidly after a mild upper respiratory tract infection. Metastatic carcinoma tends to have a short history with progressive enlargement. Transient post-prandial (after meals) swelling in the submandibular or parotid area is suggestive of salivary gland duct obstruction—a stone or a stenosis. Bilateral diffuse tender parotid enlargement is suggestive of parotitis, most commonly mumps, usually school children, and manifests in local epidemics and can only get the disease once!
One must also be mindful that associated symptoms both specifically to the mass and symptoms suggestive of a systemic process such as fever, night sweats, fatigue or weight loss (consider lymphoma) must be sought and documented (Figure 3). Symptoms of sore throat or upper respiratory tract infection may suggest an inflammatory cervical lymphadenopathy. Persistent hoarseness or sore throat, pain on swallowing, cough and sensation of a lump in the throat are risk symptoms of an upper aerodigestive tract malignancy. The symptoms are particularly relevant in patients who are over the age of 40 years and smoke cigarettes. These are the patients who should be referred via the ‘Two week proforma’ to the local ENT service.
While congenital and organ masses are more consistent in their locations, metastatic nodes follow a predictive pattern and help in identifying the primary malignancies (see Chapter 2).
A full head and neck examination (see Chapter 3) including mucosal surfaces is important, especially when suspecting malignancies (Table 2).
Congenital masses may be tender when infected or inflamed, but are generally soft, smooth and mobile. A tender, mobile mass or a high suspicion of inflammatory adenopathy with an otherwise negative examination may warrant a clinical trial of a broad-spectrum antibiotic and a review after 2 weeks. Chronic inflammatory masses and lymphomas are often non-tender and rubbery and may be mobile or feel like a “matted mass” of nodules. In older age groups, the submandibular and parotid glands may become ptotic (droopy) and mimic a neck mass, and can cause concern to patients.
Full blood count and ESR.
Viral serology: Epstein–Barr Virus, cytomegalovirus and toxoplasmosis.
Throat swab: occasionally helpful (but must be sent immediately in the proper medium).
Thyroid function tests and ultrasound in all cases of thyroid enlargement.
Chest X-ray in smokers with persistent neck lump.
Ultrasound scan (USS) can delineate the position, size, and sometimes the nature of a neck lump. It may delineate impalpable nodes and thyroid nodules. The shape of a lymph node (normally oval with a fatty hilum) can be altered by malignant disease (round shape with irregular margins and altered hilum). Although USS is performed with a view to guiding a fine needle aspiration biopsy (USSgFNAB) it should be noted that a biopsy may not be indicated if the size and nature of the lump is obviously benign.
Fine needle aspiration biopsy (FNAB) is helpful for the diagnosis for neck masses and is indicated in any neck mass that is not an obvious abscess and persists following prescribed antibiotic therapies. A negative result may require a repeat FNAB, USSgFNAB or even an open biopsy, correlating with other clinical information.
Radionucleotide scanning: for suspected parathyroid and thyroid gland masses.
Computed Tomography (CT) scanning can distinguish cystic from solid lesions, define the origin and full extent of deep, ill-defined masses, and when used with contrast can delineate vascularity or blood flow.
Magnetic Resonance Imaging (MRI) is useful for parapharyngeal and skull base masses and for assessment for unknown primary carcinomas. With contrast it is good for vascular delineation and MRI angiography may substitute for arteriography in the pulsatile mass or mass with a bruit or thrill.
See Table 3 for differential diagnosis according to position.
Benign Neck Lesions
Haemangiomas and Lymphangiomas
These are congenital lesions usually presenting within the first year of life. Lymphangiomas usually remain unchanged into adulthood, whereas haemangiomas often resolve spontaneously within the first decade (Figure 4). A lymphangioma mass is soft, doughy, ill-defined, and may present with pressure effects. Haemangiomas often appear bluish on the overlying skin and are compressible. CT or MRI may help define the extent of the neoplasm, especially when involving the airway. Treatment of lymphangioma includes injection of picibanil or excision. For haemangiomas, treatment is generally non-surgical and to await spontaneous resolution. The aggressive proliferative types are treated using propranolol +/− steroids orally, but this must be given under specialist hospital care because of its side-effects. The duration of treatment remains controversial, but should be given until the expected proliferative phase has elapsed.
These are common masses presenting in older patients. They are slow growing, but may become fluctuant and painful when infected. Diagnosis is made clinically; the overlying skin is adherent to the underlying mass and a punctum is often seen. Excisional biopsy confirms the diagnosis and is curative.
Lipomas or fatty lumps are the most common benign soft tissue tumour in the neck (Figure 5). They present as a poorly defined, soft mass usually presenting after the fourth decade. They are usually asymptomatic, soft to touch and deep to the skin. Occasionally they can grow very large and be deeply seated between muscles and nerves. FNAC or MRI scan will confirm the diagnosis and extent of the lesion. Surgery is generally for cosmetic reasons, but may be indicated when there appears to increasing in size or doubt about the accuracy of diagnosis.
Branchial Cleft Cyst
Most often presents in young adults following an upper respiratory tract infection, as a sudden onset of a tender oval mass, which is described as being 4–6 cms in size, located in the upper neck, junction of upper and middle third anterior border of the SCM muscle (Figure 6). If presentation is as an acute infected process then the lesion should be treated with antibiotics followed by elective interval surgery. The differential diagnosis is between a solid mass—most likely to be a lymphoma in this age group. In patients over 40 years of age, there is a possibility that such a presentation could represent a cystic metastasis from a papillary thyroid carcinoma or a cystic metastasis from a primary pharyngeal squamous cell carcinoma. Treatment is surgical excision of the mass with histological confirmation of the true nature of the cystic process.
Thyroglossal Duct Cyst
This is a common congenital midline neck mass, but it may lay off-centre at the lateral edge of the thyroid cartilage (Figure 7). On occasions it can be found along a tract from the hyoid bone to the anterior mediastinum. The lesion elevates on tongue protrusion as it is attached to the hyoid bone. This movement distinguishes a thyroglossal cyst from a dermoid cyst or an enlarged lymph node. Should presentation be as an infected cyst then treatment should be with board-spectrum antibiotic followed by interval surgery. Surgery must include excising the mid-portion of the hyoid bone (Sistrunk's procedure) as not to do so is likely to result in incomplete excision resulting in a discharging thyroglossal fistula. This will require to be treated subsequently by further surgery.
Inflammatory Cervical Adenitis
Benign cervical adenopathy is the most common cause of a neck mass in children. Palpable nodes are present in 40% of infants and approximately 55% of children. Thus all paediatric age groups have lymph nodes that are palpable but not necessarily associated with an underlying systemic infection or illness. Cervical lymph nodes that are asymptomatic and <1 cm in diameter may be considered normal in children under 12 years of age. Lymphadenitis in children may have a viral, bacterial, fungal, parasitic or non-infectious aetiology. The most common cause of bacterial cervical adenitis in children is staphylococcus aureus and Group A streptococci. Anaerobic bacteria also may be involved. Suspected cases of bacterial adenitis will usually respond to a 10 day course of a beta-lactamase- resistant agent, with complete resolution of the mass in 4–6 weeks. However in cases of progressive symptoms, enlargement of nodes, or the development of fluctuation or an abscess, aspiration or incision and drainage should be performed and the pus sent for culture.
Granulomatous Cervical Adenitis
This is another common cause of infectious cervical adenitis in children. Aetiology may be tuberculous or non-tuberculous mycobacteria. Atypical or non-tuberculous mycobcateria are the most common causes arising from Mycobacterium avium-intracellulare, or Mycobacterium scrofulacerum. Typically the skin overlying the involved area undergoes violaceous colour change, with skin breakdown and drainage (Figure 8). Surgery using complete nodal excision is the preferred treatment, but when involving areas such as the parotid gland curettage has been equally effective, all cases should have post-operative clarithomycin.
In general, if a presumed cervical adenopathy mass does not respond to conventional antibiotics, located in the supraclavicular fossa, or posterior triangle, or accompanied by other symptoms such as pain, fever or weight loss, a biopsy should be performed after a complete head and neck work-up to rule out malignancy or granulomatous disease. The rule of thumb is if the neck mass in an infant or child is bigger than a “golf-ball” after 3–4 weeks of observation or a “course of antibiotics”, then a serious underlying disease needs to be excluded—lymphoma or sarcoma (Figure 9a and b).
Salivary Gland Enlargement
Salivary gland enlargement is the common denominator of systemic metabolic, endocrine and autoimmune disorders. Diseases such as obesity, starvation, diabetes and hypothyroidism are associated with fatty infiltration of the salivary glands most noticeable in the parotid glands. Of course the commonest cause of parotid enlargement seen in the community is viral sialadenitis which can be caused by a variety of viruses: coxsackie virus A, echoviruses, influenza A, cytomegalovirus and most commonly the mumps (paramyxovirus). Mumps is usually a self limiting disease requiring supportive measures and pain relief. Other causes include sialadenitis or stone formation which can block the duct and present as a swelling of the major glands—parotid and submandibular gland.
Salivary gland enlargement due to neoplasms accounts for < 5% in children. The most common benign tumour of the salivary glands in children is the haemangioma, followed by pleomorphic adenoma and lymphangiomas. Benign pleomorphic adenoma is treated similarly as in adults, with FNAC to confirm the diagnosis followed by complete surgical excision. In adults pleomorphic adenoma and adenolymphoma (Warthin's Tumour) are the commonest tumours (Figure 10). Salivary gland malignancy in children is uncommon and usually presents in children over 10 years of age. The most common malignant salivary gland tumours in children and adults are the mucoepidermoid carcinoma, followed by acinic cell carcinoma and adenoid cystic carcinoma. Diagnosis is by FNAC, evaluation by CT scan and treatment is by surgery excision, with or without post-operative radiotherapy.
Thyroid neoplasms are a common cause of anterior compartment neck masses in all age groups with a female predominance and are mostly benign (Figure 11). All children with a thyroid swelling or mass should be investigated for the possibility of a malignancy. Fine-needle aspiration of thyroid masses has become the standard of care and ultrasound may help to determine if the mass is cystic or solid. Unsatisfactory aspirates should be repeated, and negative aspirates should be followed up with a repeat FNAC and examination in 3 months time.
Paraganglioma (Carotid Body Tumour, Glomus Vagale)
These are rare tumours usually found in adults (Figure 12). They are slow growing painless lumps which have an average presentation at the fifth decade. Sometimes they present as a parapharyngeal mass pushing the tonsil medially and anteriorly or as a firm mass in the anterior triangle of the neck. Biopsy is contraindicated and MRI angiography is the investigation of choice. Surgical removal is based on patient factors and presenting symptoms.
Malignant Neck Masses
The incidence of neoplastic cervical lymphadenopathy increases with age and approximately 75% of lateral neck masses in patients older than 40 years are caused by malignant tumours. In a large reported series from the UK it was reported that 74% of enlarged cervical nodes had developed from head and neck primary sites and only 11% had come from primary sites outside that region. The finding of a mass in the left supraclavicular fossa also called a Virchow's node maybe an indication of an infraclavicular metastatic malignancy—most commonly an upper GIT, lung of GUT system (Figure 13).
Thorough examination of the upper aerodigestive tract (to include the oral cavity, postnasal space, pharynx and larynx) and the thyroid gland is therefore mandatory.
Primary of Unknown Origin is a term is applied to patients with a metastatic carcinoma in cervical lymph nodes with an “occult primary”. A careful search will usually reveal the primary tumour in the skin or mucosal surfaces of the head and neck, or rarely, in an area below the clavicles, such as the lungs. It is important to thoroughly search for the primary tumour by all available diagnostic methods. A thorough history and endoscopic examination will reveal most cases, but imaging (MRI and PET CT) and panendoscopy with selective targeted biopsies from high risk sites (nasopharynx, ipsilateral tonsil excision, base of tongue and piriform fossa) may be required. In approximately 3–11% of cases the primary tumour remains elusive and it is these that the term “primary of unknown origin” should be reserved.
Indications for Specialist Referrals
In the primary care setting, neck lumps are mostly caused by inflammatory conditions that are self limiting, resolving within 2–6 weeks. A course of appropriate antibiotics with a 2-week follow-up assessment is an appropriate first line of management. Failure to resolve requires hospital referral, especially if any presenting signs or symptoms suggest a possible underlying malignancy. In a high risk patient for malignancy with a neck lump, immediate referral is recommended, usually adults who have a history of smoking or chewing tobacco and indulging in excessive drinking of alcohol. Lumps associated with weight loss or dysphonia (hoarseness), dysphagia (swallowing), or dyspnoea (breathing) for 3 weeks or more should be referred urgently for a head and neck assessment.