Chapter 122: Neck Masses in Infants and Children

Neck masses are common in childhood, and although most are benign, malignancy is always a primary consideration. Correct diagnosis is challenging, but differentiating neck masses into inflammatory, congenital, or neoplastic categories is the first step toward diagnosis.

In patients referred to tertiary centers for surgical excision of a cervical lesion, 90% to 96% of lesions are benign and are predominantly congenital.^1,2

CLINICAL FEATURES

A thorough history and physical examination will narrow the broad differential for cervical lymphadenopathy. The acuity and laterality of node swelling are helpful for diagnosis. Typically, acute bilateral lymph nodes are due to a viral cause, acute unilateral nodes are due to a bacterial cause, and subacute/chronic nodes (>4 to 6 weeks) are due to granulomatous bacteria or noninfectious causes. This framework is most effective for diagnosis when combined with the general and specific features of different causes (Tables 122-1 and 122-2). Carefully record the features of all head and neck masses for future comparison. Lymph node location and characteristics give clues based on lymphatic drainage patterns. Look for systemic disease by assessing for generalized lymphadenopathy, hepatosplenomegaly, testicular masses and/or enlargement in males, and the child's overall condition.

GENERAL MANAGEMENT

Ideally, the treatment of a childhood neck mass is directed at the specific cause; however, due to the many etiologies, this is not always obvious at first presentation. The majority of presentations are benign; however, the priority of childhood neck masses is to correctly identify those that are neoplastic. The algorithm in Figure 122-1 provides an approach to management based on suspected etiology and should be adapted to the individual patient. Follow-up of patients who are treated with antibiotics is necessary at 48 to 72 hours; if improvement of symptoms is not seen, then expanded coverage to include periodontal flora or MRSA is encouraged, and aspiration of fluctuant nodes for culture may be considered. Most lymphadenopathy will improve after 2 weeks of observation or antibiotics; however, all cases require 6-week follow-up to ensure resolution.

The most common pediatric neck mass is an enlarged lymph node caused by infection. Be careful to consider masquerading lesions such as salivary gland infections, acutely infected congenital lesions, and, most importantly, malignancies.

Cervical lymph nodes drain the skin of the head and neck as well as the entire nasal, oral, and pharyngeal mucosa (Figure 122-2). Submandibular and cervical nodes are most common because they drain much of the oropharynx, including the adenoids and tonsils.^3,4 Supraclavicular lymphadenopathy is suspicious for metastasis because it drains the abdomen and thorax.⁴

Palpable cervical lymph nodes are found in about 28% to 44% of healthy infants and children, with the incidence peaking in early childhood.^3,5,6 Lymph nodes ≤1 cm in children <12 years old are considered normal.^7,8 Most lymphadenopathy represents nonspecific reactive hyperplasia, often due to a viral upper respiratory tract infection.⁹ Lymphadenitis is lymph node inflammation (swelling, tenderness, warmth, erythema) and is most commonly due to viral or bacterial causes. Infection with a pyogenic organism may lead to liquefactive necrosis (suppuration) and abscess formation (Figure 122-3). If the immune system is unable to eradicate a particular organism, macrophages will attempt to contain it, forming a chronic granulomatous lymphadenitis.

ACUTE BILATERAL LYMPHADENOPATHY

Acute bilateral lymphadenopathy is usually due to viral infection and is self-limited. Common viruses include rhinovirus, parainfluenza, influenza, respiratory syncytial virus, coronavirus, reovirus, and adenovirus.¹⁰ Treatment is symptomatic and expectant.

Alternative viral causes include infectious mononucleosis, characterized by fever, exudative pharyngitis, and significant lymphadenopathy (Figure 122-4). Heterophile antibodies or Epstein-Barr virus–specific immunoglobulin M confirms the diagnosis, and in immunocompetent patients, treatment is symptomatic. The classic viral exanthems associated with lymphadenopathy are measles (Koplik spots, conjunctivitis, and a descending rash) and rubella (associated with Forchheimer spots, rash, and polyarthritis). Acute bilateral lymphadenopathy and oral lesions may also be due to herpes simplex virus (gingivostomatitis) or coxsackie virus (herpangina). Pharyngitis caused by group A Streptococcus is accompanied by cervical lymphadenopathy.¹¹ Bilateral swelling that extends over the jaw suggests parotid gland involvement due to mumps and may be associated with orchitis and a rash.

ACUTE UNILATERAL LYMPHADENOPATHY

Acute unilateral lymphadenopathy is most often due to bacterial lymphadenitis (Figure 122-5) caused by Staphylococcus aureus and group A Streptococcus.¹² Lymph nodes will likely have signs of inflammation, and if an abscess has developed, fluctuance may be appreciated. Often the source of group A Streptococcus is the pharynx, whereas S. aureus originates from a break in the skin. Careful examination of the head, neck, throat, skin, and ears may identify a source that can be cultured.

Generally, lymph nodes <1 cm are normal and do not need treatment. If lymphadenitis measures between 1 and 3 cm, appropriate antibiotics treating group A Streptococcus and Staphylococcus for up to 2 weeks should lead to complete resolution.¹³ Nodes >3 cm raise suspicion for malignancy, but if the nodes are acute and inflammatory, a course of antibiotics and observation is reasonable.

Due to increasing β-lactamase–resistant Staphylococcus, first-line oral antibiotic choices are first-generation cephalosporins, cloxacillin/dicloxacillin/oxacillin, or clindamycin.^12,14,15 If the child is unwell or immunocompromised, treat with IV cefazolin, nafcillin, or clindamycin. Reassess in 48 hours, and if no improvement is appreciated, consider changing antibiotics to treat methicillin-resistant S. aureus.^16,17

Fluctuance of the node suggests abscess formation due to pyogenic bacteria, with the source often from the tonsils.¹⁵ Fluctuant nodes typically respond to antibiotics alone, but needle aspiration after local anesthesia may be helpful to avoid incision in cosmetically important areas.¹⁵ If a superficial abscess is pointing or not resolving within 2 weeks of antibiotics, then evaluation by US and incision and drainage may be necessary.¹⁸ When associated with torticollis or trismus, suspect a retropharyngeal abscess, and obtain imaging and/or surgical consultation (see chapter 123, "Stridor and Drooling in Infants and Children").

Infants have a higher incidence of infection with group B Streptococcus rather than S. aureus or group A Streptococcus. Group B streptococcal infection can be associated with bacteremia, pneumonia, and meningitis.^19,20 Infections originating from the oral mucosa, seen most commonly in children age 5 to 15 years with periodontal disease, are more likely to contain anaerobic bacteria requiring coverage with penicillin V, amoxicillin-clavulanic acid, or clindamycin.¹⁴ Sialoadenitis is characterized by unilateral swelling crossing the jaw and tenderness after meals, and can be associated with purulent discharge from Wharton's and Stensen's ducts (Figure 122-6). Sialolithiasis can cause recurrent infections due to outflow obstruction of the gland.

SUBACUTE/CHRONIC LYMPHADENOPATHY

Chronic lymphadenopathy is defined as persistence for 6 weeks without resolution. Persistent lymphadenopathy, generalized lymphadenopathy, or failure to respond to 2 to 4 weeks of antibiotics requires evaluation for uncommon infectious, congenital, and neoplastic causes. Persistent infectious agents evade eradication by granuloma formation. Generally, the course of evolution is slower than a typical inflammatory lymphadenitis and may be characterized by a violaceous overlying skin change. Refer to otolaryngology for definitive diagnosis and management.^21,22,23 Refer immunocompromised patients to an infectious disease specialist. Some of the more common causes of granulomatous lymphadenitis are covered below.

Mycobacterial Lymphadenitis

Chronic cervical lymphadenitis may be due to tuberculous (Mycobacterium tuberculosis) or nontuberculous mycobacterial strains. Clinically, both varieties of lymphadenitis are characterized by a chronic, minimally tender, "cold abscess," with overlying violaceous skin. Spontaneous drainage can transform into a chronic draining sinus.²² It is important to differentiate between tuberculous and nontuberculous strains because treatment is different. Differential Mantoux testing with a combination of antigens can identify strains in about 93% of patients.²⁴ Consider M. tuberculosis lymphadenitis in children with risk of tuberculosis exposure, in those exhibiting constitutional signs, and in those with an abnormal chest x-ray and with a strongly reactive purified protein derivative skin test,²⁵ and treat for 8 to 12 months. Treatment for nontuberculous lymphadenitis is surgical excision.²⁶

Cat-Scratch Disease

Cat-scratch disease is caused by Bartonella henselae, with inoculation by a scratch or a bite from a kitten. The inoculation site develops into a painless, erythematous vesicle or pustule after 3 to 10 days, which may disappear before other symptoms develop. After 1 to 3 weeks, regional lymphadenopathy typically develops. Suppuration is uncommon. Diagnosis involves serologic testing, but because antibody development may be delayed and the primary lesion may have resolved, rely on a strong history of cat exposure and an inoculating lesion. No treatment is needed. Serious complications (encephalitis) can develop in immunodeficient patients, with treatment options based primarily on case reports.

Toxoplasmosis

Toxoplasma gondii is a protozoan parasite with a complex life cycle that can infect humans through ingestion of undercooked meat, exposure to oocysts in cat feces, or through maternal-fetal transmission (congenital toxoplasmosis). Infection is widespread; an estimated 22.5% of children in the United States are infected by the age of 12 years old; in some countries, up to 95% of the population has been infected. The lymphatic system is the most common organ system involved. Diagnosis is usually made serologically with antibody titers. Treatment is not typically needed in healthy children.

Tularemia

Tularemia is caused by the bacteria Francisella tularensis, which is transmitted by arthropod bites, handling infected animals (classically rabbits), and contaminated food and water. The most common forms are ulceroglandular and glandular tularemia. In the ulceroglandular form, the inoculation site develops from an erythematous tender papule into an exudative ulcer within days. Regionally draining lymphadenopathy subsequently develops that may spontaneously suppurate and drain if not treated. Tularemia is diagnosed serologically with microagglutination testing. Gentamicin is the treatment of choice.

Other Causes

Other causes of cervical lymphadenopathy include recent immunizations (diphtheria, pertussis, and tetanus; polio; typhoid), blood transfusions (causing Epstein-Barr virus, cytomegalovirus, or human immunodeficiency virus exposure), various medications (including phenytoin, carbamazepine, isoniazid, and hydralazine) and systemic disease (Kawasaki's disease; sarcoid; periodic fever, aphthous stomatitis, pharyngitis, and adenitis; and autoimmune) (Tables 122-1 and 122-2).

In patients referred to tertiary centers for surgical excision of a cervical lesion, 90% to 96% of lesions are benign, and most are congenital.^1,2 Congenital lesions are the most common cause of noninflammatory pediatric neck masses,¹ but a mass may not be recognized until infection develops. The sternocleidomastoid muscle divides the neck into anterior and posterior triangles, and congenital neck masses can be organized into midline, anterior, and posterior groups (Table 122-3). The most common congenital neck masses and their characteristic features are discussed below.

THYROGLOSSAL DUCT CYSTS

Thyroglossal duct cysts (Figure 122-7A) account for approximately 70% of the congenital neck masses and are the second most common benign neck mass after lymphadenopathy.²⁷ Thyroglossal duct cysts result from the persistence of any segment of the thyroglossal duct along its course from the foramen cecum of the tongue to the pyramidal lobe of the thyroid and are located in the midline of the neck within the anterior triangle.²⁸ Most of the lesions are infrahyoid (65%), whereas suprahyoid cysts account for 20%, and another 15% of lesions are at the level of the hyoid bone.^29,30 The pathognomonic feature is a painless fluctuant mass that moves with swallowing or protrusion of the tongue. Acutely infected cysts require antibiotics. Further investigations and surgical excision are undertaken after infection subsides because they carry a very small risk of malignant transformation.³¹

DERMOID CYSTS

Dermoid cysts are developmental anomalies involving pluripotent embryonal stem cells and occur most often in children <3 years old. Dermoid cysts are usually suprahyoid³² and midline and are often misdiagnosed as thyroglossal duct cysts.^33,34 Dermoid cysts are mobile, but they do not move with tongue protrusion. Management is surgical excision.

BRANCHIAL CLEFT CYSTS

Incomplete obliteration of the branchial apparatus, predominately the cleft, leads to branchial cleft anomalies: cysts, sinuses, or fistulae.²⁸ Up to 95% of branchial cleft cysts arise from remnants of the second branchial cleft,³⁵ which are most often anterior to the sternocleidomastoid muscle near the angle of the mandible (Figure 122-7B). The cysts are round, smooth, and mobile, but are not tender unless they become infected. There may be a history of recurrent swelling or infection in the same area. Cysts may spontaneously rupture, forming an external sinus or fistula. Oral antibiotics are indicated for infected cysts prior to definitive surgery.

LYMPHANGIOMAS

Lymphangiomas (also known as cystic hygromas) result from sequestration of lymphatic channels that fail to communicate with the internal jugular vein leading to a blockage of the lymphatic system.³⁶ They can occur anywhere in the body, but nearly 75% arise within the neck, most commonly along the jugular chain of lymphatics. Cervical lymphangiomas are soft, painless, and compressible, and can be very large (Figure 122-7C). Morbidity is usually secondary to compression of surrounding structures, and large neck lesions may cause serious airway and feeding problems. Sixty-five percent present at birth, and 90% are clinically detected by the end of the second year.^29,36 Sudden enlargement can occur from infection or hemorrhage into the lesion. Careful inspection of the oral cavity and palpation of the trachea for deviation are important in assessing the airway of patients with large cystic hygromas. Injection of sclerosing agents can be used in some lesions, although surgical excision is the definitive treatment.³⁶

Malignancy should always be considered as a potential etiology in the evaluation of a child with a neck mass. Of childhood malignancies, 5% to 12% present as a head or neck mass, of which 99% are primary tumors.^{1,37,38,39,40} Diagnosing malignancies is made challenging by their rarity; only 1% of pediatric superficial lumps are malignant;⁴¹ accordingly, a high index of suspicion and attention to worrisome features from history and physical examination are required (Table 122-4).^{12,25,39,40,41,42,43} One review demonstrated that in 80% of superficial masses, malignancy can be ruled out with 99.7% accuracy based on the absence of five clinical features (Table 122-4).⁴¹ The presence of any worrying features should prompt urgent referral for further diagnostic workup that may include fine-needle aspiration cytology, imaging, or excisional biopsy.

BENIGN NEOPLASMS

Benign noninflammatory pediatric neck masses comprise 5% of presentations.⁴⁴ An exhaustive review is not included in this chapter; instead, benign masses with specific identifying features are highlighted.

Hemangiomas

Hemangiomas are congenital vascular tumors that present at 2 to 4 weeks, usually grow rapidly until 9 to 10 months of age, and typically regress thereafter. When palpable, they are soft, mobile, and frequently have a bluish hue.⁴⁵ Almost 90% of hemangiomas resolve spontaneously without the need for therapy. Lesions showing unusually rapid growth, hemorrhage, recurrent infection, or compression of adjacent structures may require treatment by a specialist, including β-blockers, steroids, and laser or surgical excision.⁴⁶ Hemangiomas compromising the airway result in biphasic stridor not responsive to nebulized epinephrine and may be associated with cutaneous lesions in a beard distribution.

Neurofibromas and Schwannomas

Neurofibromas and schwannomas are rare usually large tumors often involving the orbits, the skull base, or the parotid region, and are frequently associated with neurofibromatosis type I, which may present with café-au-lait spots and other classic features.⁴⁴

Fibromatosis Colli

Fibromatosis colli (also known as pseudotumor of infancy) presents in the neonatal period as a mass in the sternocleidomastoid muscle (Figure 122-8). Often due to birth trauma with forceps delivery, part of the involved muscle is replaced by dense fibrous tissue.^44,47 Parents will often notice a mass or limited range of motion of the neonate's neck in the first weeks of life. Physical examination reveals a firm, solid, immobile mass located within the sternocleidomastoid muscle that is horizontally mobile, vertically fixed, and moves with the muscle when the head is turned. It is often associated with some degree of sternocleidomastoid contracture (torticollis). The diagnosis can often be made clinically, and imaging is not necessary. Treatment involves stretching and physical therapy, resulting in spontaneous resolution over a period of 4 to 8 months.^30,44,47

MALIGNANT NEOPLASMS

Malignant head and neck masses are rare, accounting for approximately 1% of all etiologies. However, assessment for worrying features is important in the ED (Table 122-4); management of a suspected malignant lesion should include urgent referral for definitive treatment. The more common childhood malignancies presenting as a neck mass are highlighted briefly (see also chapter 143, "Oncologic and Hematologic Emergencies in Children").

Lymphoma

Lymphoma is the most common childhood malignancy of the head and neck.^28,39,44 Most lymphomas present as a large, firm mass that is often mobile but can be fixed and is commonly located in the anterior triangle or the supraclavicular area.^{1,37,39,48,49} One study found that 35% of childhood head and neck lymphomas presented as a supraclavicular mass.¹ Hodgkin's lymphoma is more likely nodal, found in the supraclavicular area, and seen more often in teenagers, whereas non-Hodgkin's lymphoma is primarily extranodal and has increasing incidence with age. Diagnosis requires excisional biopsy. Do not give steroids before biopsy because steroids interfere with staging.

Rhabdomyosarcoma

Rhabdomyosarcoma is a frequent malignancy of the head and neck in children.⁴⁴ This tumor presents in the neck in 40% of cases, often as a large painless mass, rarely causing compression or infiltration of adjacent structures, most notably the airway.^37,38 Peak incidence is at 2 to 5 years old and again at 15 to 19 years old. Neck tumors may present with brachial plexus palsy.

Neuroblastoma

Neuroblastoma is a malignancy of the sympathetic chain, and the adrenal glands are the most common primary site.^30,50 Most present before the age of 5 years, and those arising in the neck (5%) have a better prognosis than tumors of adrenal origin.^28,51 Neuroblastoma may present with local compressive signs such as hoarseness, dysphagia, airway obstruction, Horner's syndrome, or cranial nerve palsies.

Thyroid Cancer

Thyroid cancer accounts for 21% of pediatric head and neck cancers; however, its incidence is decreasing due to declining radiation exposure.⁴⁰ Childhood thyroid cancer is usually papillary, presenting at a more advanced stage, with node, muscle, and metastatic involvement in 90% of cases; despite its advanced presentation, the prognosis is better than adult thyroid cancer.⁵² Benign causes of diffuse thyroid enlargement include symptomatic thyrotoxicosis or Hashimoto's thyroiditis,⁵³ although goiter is seen in some parts of the world.

Metastatic Disease

Metastatic disease may also present as cervical lymphadenopathy, accounting for 1% of childhood head and neck cancers. Tumors that metastasize to the neck include nasopharyngeal carcinoma and some thoracic/GI tumors. The location of the metastasis may give a hint to the origin of the primary tumor: posterior triangle nodes are often seen in nasopharyngeal carcinoma, whereas isolated supraclavicular nodes suggest a mediastinal or abdominal mass.