CHAPTER 94

Neck Masses

Casey Buitenhuys, MD, FACEP, and Stanley H. Inkelis, MD, FAAP

A neck mass is any swelling or enlargement of the structures in the area between the inferior mandible and the clavicle. Normal variants, such as the angle of the mandible or tip of the mastoid bone, may occasionally appear as swellings, and the parent or guardian sometimes confuses these with neck masses. If the swelling is not a normal structure, a well-directed history and physical examination usually determine the etiology.

Lymphadenopathy from viral or bacterial throat infections is the most common cause of neck masses in children. Therefore, neck masses are common because children frequently have sore throats. Most parents and guardians know about swollen lymph glands, and they usually do not seek medical advice unless the glands become quite large or do not recede in a few days. Neck masses in children may have many other causes besides lymphadenopathy. Most of these masses may be categorized as inflammatory, neoplastic, traumatic, or congenital in origin. A well-described mnemonic in the adult literature, KITTENS (congenital/developmental anomalies, infectious/inflammatory, trauma, toxic, endocrine, neoplasms, systemic disease), can summate many of the causes of neck masses in children as well (Box 94.1).

Epidemiology

Most neck masses are benign. Almost 50% of all children 2 years of age and up to 90% of children between 4 and 8 years of age have palpable cervical lymph nodes. Although more than 25% of malignant tumors in children are found in the head and neck region (this is the primary site in only 5%), less than 2% of suspicious head and neck masses are malignant.

The epidemiology of neck masses of infectious origin depends on the infectious agent itself, geographic location of the child, and the child’s immediate environment. Neck masses of viral origin may be related to focal infection of the oropharynx or respiratory tract but often are associated with generalized adenopathy. Neck masses of bacterial origin typically occur from normal bacterial flora of the nose, mouth, pharynx, and skin that secondarily spreads to lymph nodes. These organisms are not usually transmitted from person to person. Pathologic flora, such as group A streptococcus and Mycobacterium tuberculosis, that result in neck masses can spread by human-to-human contact, however. Additionally, cat-scratch disease is caused by Bartonella henselae, a vector-borne pathogen.

Clinical Presentation

Children with neck masses present in a variety of ways depending on the etiology of the mass. Typically, a swelling or enlargement in the neck, which a parent or guardian often notices more than the child, is evident. Associated signs and symptoms include fever, upper respiratory tract infection, sore throat, ear pain, pain or tenderness over the mass, changes in skin color over the mass, skin lesions of the head or neck, and dental caries or infections (Box 94.2). Malignant tumors are usually slow-growing, firm, fixed, nontender masses. Congenital neck masses and benign tumors, which have frequently been present since birth or early infancy, are soft, smooth, and cyst-like and may be recurrent. Neck masses associated with trauma are often rapidly evolving and may result in airway obstruction. Temporal development of neck masses is a helpful predictor of mass etiology. The “rule of 7” proposed by Skandalakis may be applied and adapted to pediatric neck masses (Table 94.1).

Pathophysiology

The pathophysiology of neck masses in children is dependent on etiology. Most neck masses are related to inflammation or infection of lymph nodes. Enlargement of lymph nodes usually results from proliferation of intrinsic lymphocytes or macrophages already present in the lymph node (eg, lymphadenopathy caused by a viral infection) or from infiltration of extrinsic cells (eg, lymphadenitis, metastatic tumor). Neck masses from trauma occur from leakage of fluid into the neck, and congenital anatomic abnormalities become apparent because of fluid collection or infection of the defect. The parotid gland may be enlarged from inflammation (eg, blocked salivary gland duct), infection (eg, mumps), or tumor (eg, pleomorphic adenoma), but the swelling primarily involves the face rather than the neck and obscures the angle of the jaw. Other salivary glands may be infected or obstructed and may cause submandibular swelling, erythema, and tenderness.

Adapted with permission from Skandalakis JE. Neck. In: Skandalakis LJ, Skandalakis JE, Skandalakis PN, eds. Surgical Anatomy and Technique: A Pocket Manual. 3rd ed. New York, NY: Springer; 2009:17–91.

Differential Diagnosis

Neck masses in children are usually the result of inflammation or infection of lymph nodes, tumors of lymph nodes and other neck structures, trauma, and congenital lesions. The location of the mass is often a clue to its etiology (Figure 94.1).

Figure 94.1. Differential diagnosis of neck mass by location. 1, Parotid—cystic hygroma, hemangioma, lymphadenitis, parotitis, Sjögren syndrome, infantile cortical hyperostosis (Caffey-Silverman syndrome), lymphoma. 2, Postauricular—lymphadenitis, branchial cleft cyst (first), squamous epithelial cyst. 3, Submental—lymphadenitis, cystic hygroma, thyroglossal duct cyst, dermoid, sialadenitis. 4, Submandibular—lymphadenitis, cystic hygroma, sialadenitis, tumor, cystic fibrosis. 5, Jugulodigastric—lymphadenitis, squamous epithelial cyst, branchial cleft cyst (first), parotid tumor, normal-transverse process C2, styloid process. 6, Midline neck—lymphadenitis, thyroglossal duct cyst, dermoid, laryngocele, normal hyoid, thyroid. 7, Sternomastoid (anterior)—lymphadenitis, branchial cleft cyst (second, third) pilomatricoma, rare tumors. 8, Spinal accessory—lymphadenitis, lymphoma, metastasis from nasopharynx. 9, Paratracheal—thyroid, parathyroid, esophageal diverticulum. 10, Supraclavicular—cystic hygroma, lipoma, lymphoma, metastasis, normal fat pad, pneumatocele of upper lobe. 11, Suprasternal—thyroid, lipoma, dermoid, thymus, mediastinal mass.
Adapted with permission from May M. Neck masses in children: diagnosis and treatment. Pediatr Ann. 1976;5[8]:517–535, with permission from SLACK Inc.

Lymphadenopathy/Lymphadenitis

Lymphadenopathy is lymph node enlargement or hyperplasia secondary to localized infection or antigenic stimulation proximal to the involved node or nodes. Because lymphoid tissue steadily increases until puberty, palpable lymph nodes, including those in the cervical area, are a common, normal finding in children. Palpable cervical lymph nodes are present in up to 90% of children between 4 and 8 years of age. Any lymph node in the neck larger than 10 mm qualifies as cervical lymphadenopathy.

The most common cause of bilateral cervical lymphadenopathy is a viral infection of the upper respiratory tract. Lymphadenopathy usually begins and resolves with the acute infection. Occasionally, the swelling remains for several days or months, however, and a child presents because of parental or guardian concern. Bacterial pharyngitis, usually from infection with group A β-hemolytic streptococcus, is often associated with cervical lymphadenopathy. Cervical as well as generalized lymphadenopathy may occur in the child with systemic illness (eg, Kawasaki disease, sarcoid, HIV, mononucleosis). Cat-scratch disease, toxoplasmosis, fungal infection, and mycobacterial and atypical mycobacterial infections may be associated with lymphadenopathy that may later progress to lymphadenitis. Viral illnesses, including Epstein-Barr virus (ie, mononucleosis); adenovirus; enterovirus; human herpesvirus, 1, 2, and 6; and cytomegalovirus commonly cause lymphadenopathy and lymphadenitis.

Lymphadenitis is infection or inflammation of the lymph node that occurs when microorganisms and neutrophils infiltrate the node, resulting in necrosis and abscess formation. This condition usually is associated with proximal bacterial infection that drains to the affected nodes by connecting afferent lymphatic channels. Lymphadenitis often is a progression of disease, resulting in enlarged nodes that measure 2 to 6 cm. These nodes, which are typical of bacterial disease, are often termed “hot nodes,” and they are erythematous, warm, tender, and sometimes fluctuant. Subacute or chronic inflammation of lymph nodes typical in illnesses such as cat-scratch disease, mycobacterial infection, or toxoplasmosis are not warm to the touch and are usually not as tender. They are typically not suppurative and may be difficult to distinguish from nodes that are simply enlarged (Figure 94.2).

Staphylococcus aureus and group A β-hemolytic streptococcus are responsible for 50% to 90% of cases of acute unilateral cervical lymphadenitis. These organisms spread from a primary site to the lymph nodes, draining those sites. Common primary sites of infection are the throat, teeth and gums, and skin (lesions), particularly on the scalp or ears. Infections at these sites may result from trauma, such as scratches or scabs, or from primary infection, such as impetigo. Anaerobic oral flora constitute a small portion of causes for bacterial cervical adenitis, especially in older children with poor dentition. Staphylococcus aureus or group B streptococcus usually causes cervical lymphadenitis in neonates or very young infants. Such staphylococcal infections, which often are nosocomially spread from contact in the newborn nursery, present as discrete masses. Group B streptococcus causes the “cellulitis-adenitis” syndrome, which presents as cervical adenitis associated with a facial cellulitis. Pseudomonas aeruginosa is an unusual cause of cervical adenitis in newborns.

Mycobacterial disease must always be considered in children of all ages who present with cervical adenitis particularly involving posterior cervical nodes. The child with M tuberculosis cervical adenitis often has multiple nodes, sometimes bilateral, usually nontender, and usually not erythematous or warm. These children are typically older, commonly reside in an urban setting, have a history of tuberculosis exposure, and often have an abnormal finding or findings on chest radiography. Intradermal placement of a purified protein derivative (PPD) often produces more than 15 mm of induration in most children with typical mycobacterial infection. A reaction of 5 to 14 mm may be caused by tuberculous or nontuberculous mycobacterial infection. Atypical mycobacterial infection usually occurs in children between 1 and 6 years of age with unilateral rather than bilateral lymph node enlargement and normal findings on chest radiography.

Cat-scratch disease should be considered in children who have cats or kittens or who play with them. The infection may result from a cat scratch or from a cat licking a child’s broken skin. If the inoculum is near the head and neck area, cervical adenitis manifests. Contact with the hand may result in axillary lymphadenitis. Occasionally, generalized lymphadenopathy is present. An associated nonpainful papule or papules where the cat scratch or lick occurred may be apparent. Bartonella henselae has been identified as the organism causing cat-scratch disease.

Figure 94.2. The lymphatic drainage and lymph nodes involved in infants and children with cervical lymphadenitis.

Adapted with permission from Feigin RD, Cherry JD, eds. Textbook of Pediatric Infectious Diseases. 5th ed. Philadelphia, PA: Saunders; 2004:186.

Toxoplasmosis may be accompanied by adenitis, usually in the posterior cervical area. Nodes are painless and may fluctuate in size, and children often are asymptomatic. Multiple lymph nodes are involved in approximately one-third to one-half of cases.

The child who presents with recurrent cervical adenitis and recurrent fever may have PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis). This entity usually occurs in children younger than 5 years and can be aborted with steroids.

Kikuchi disease, or histiocytic necrotizing lymphadenitis, has an Asian and female predilection and is characterized by fever, leukopenia, and cervical lymphadenopathy. The illness is self-limited, and follow-up is recommended because of a possible association with systemic lupus erythematosus.

Less common bacterial, viral, and fungal causes of cervical adenitis are listed in Box 94.3.

Tumors

Compared with other neck masses, malignant neck tumors occur rarely; nevertheless, they should be considered in any child with an enlarging or persistent neck mass. Hodgkin disease and non-Hodgkin lymphoma are the most frequent cause of head and neck malignancies in children, accounting for almost 60% of cases. Rhabdomyosarcoma is the next most frequent head and neck malignancy, followed by thyroid tumor, neuroblastoma, and nasopharyngeal carcinoma. Age is an important factor in determining the likelihood of specific tumors. Neuroblastoma, leukemia, and rhabdomyosarcoma are the most common tumor types in children younger than 6 years. Non-Hodgkin lymphoma typically occurs in preadolescence, and Hodgkin disease and thyroid carcinoma are the most common malignancies in adolescents.

Benign neck tumors, with the exception of those mentioned later in the discussion of congenital lesions, are uncommon. They include epidermoid inclusion cysts, lipomas, fibromas, neurofi-bromas, pilomatricomas (ie, benign skin neoplasms of hair follicle origin), keloids, goiters, and ranulas (ie, intraoral mucocele) and plunging ranula (ie, an extension of the oral ranula into the neck).

Trauma

Trauma to the neck may be associated with bleeding and edema. Large hematomas that affect vital structures are potentially life-threatening. Significant trauma and structural injury usually accompany expanding neck hematomas. Neurologic deficits and stroke after neck trauma should alert the physician to consider cervical arterial dissection. In the child with mild injury and neck hematoma, bleeding disorders are a possible cause of the hematoma. Twisting injury to the neck may result in muscle spasm of the sternocleidomastoid muscle (ie, torticollis) and an apparent mass that is the contracted muscle. Additionally, intramuscular hematoma or bleeding from vaginal delivery may cause torticollis in the neonatal period. The neck is bent toward the side of the affected sternocleidomastoid muscle. Child abuse should also be considered in children who have neck injuries that are not consistent with their histories.

Atlantoaxial rotary subluxation may result in a torticollis-like syndrome in the patient with minimal to no trauma. Because of the relatively flat nature of the facets, rotation and subluxation of C1 on C2 can occur. The patient generally presents with unilateral neck pain and inability to turn the head. The head is tilted to 1 side with the chin rotated the opposite direction from the subluxation.

A foreign body in the neck may present as a mass because of the foreign body itself (eg, piece of glass or metal, bullet) or surrounding inflammation. A crepitant neck mass following trauma to the neck or chest is suggestive of subcutaneous emphysema from tracheal injury or a pneumomediastinum. Crepitant neck masses may also occur secondary to pneumomediastinum in the child with obstructive lung disease, such as asthma or cystic fibrosis.

Congenital Lesions

The child with a congenital neck lesion can present with a neck mass in early infancy or later in childhood. Some congenital lesions are not discovered until adulthood. The most common of these benign lesions are thyroglossal duct cysts, branchial cleft cysts, lymphatic malformations (ie, cystic hygromas/lymphangiomas), and hemangiomas (Figure 94.3).

Thyroglossal duct cysts are almost always midline in the neck and inferior to the hyoid bone. They usually move upward with tongue protrusion or swallowing. Most branchial cleft cysts occur anterior to the middle third of the sternocleidomastoid muscle. Less commonly, branchial cleft cysts may appear in the posterior triangle of the neck and the preauricular area. Branchial cleft sinus tracts appear as slit-like openings anterior to the lower third of the sternocleidomastoid muscle and may present as neck masses if they become infected. Thyroglossal duct cysts and branchial cleft cysts may also present for the first time as infected neck masses.

Cystic hygromas are usually large, soft, easily compressible masses found in the posterior triangle behind the sternocleidomastoid muscle in the supraclavicular fossa. They transilluminate well. Two-thirds of cystic hygromas are present at birth, and 80% to 90% are identified before 3 years of age. They are more common on the left side of the neck. Cystic hygromas occasionally become secondarily infected, with findings of erythema, warmth, and tenderness.

Hemangiomas are usually not present at birth but appear in early infancy and may enlarge rapidly. In most cases, they recede spontaneously by 9 years of age. They are usually much smaller than cystic hygromas, do not transilluminate, and may be recognized by their reddish color (eg, capillary or strawberry hemangioma) or by a bluish hue of the overlying skin (eg, cavernous hemangioma).

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