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Neck Masses

Potsic (1997)reported that in children, 95% of neck masses are enlargedlymph nodes (hyperplasia constituting 90% and cervicaladenitis 10%), 3% are congenital masses, and 2% areother masses.

A lymph node located in the neck regionthat is soft, mobile, nontender, and <1 cm in diameteris normal. Presence of larger lymph nodes or supraclavicular nodesis abnormal.

Principal Causes of Neck Masses

1. Congenitalmasses
   1. Branchialcleft anomalies
   2. Preauricular pits, sinuses, and cysts
   3. Thyroglossal duct cyst
   4. Dermoid cyst
   5. Congenital muscular torticollis (fibromatosiscolli)
   6. Lymphatic malformation
   7. Laryngocele
   8. Thymic cyst
   9. Vascular anomalies
2. Enlarged lymph nodes
   1. Infection
      1. Acuteunilateral adenitis
      2. Acute bilateral cervical adenitis
      3. Chronic unilateral cervical adenitis
         1. Cat scratchdisease
         2. Mycobacterial infection
         3. Toxoplasmosis
   2. Noninfection
      1. Kawasaki disease
      2. Sarcoidosis
      3. Histiocytic necrotizing lymphadenitis(Kikuchi-Fujimoto disease)
      4. Other
3. Trauma
   1. Hematoma
   2. Subcutaneous emphysema
4. Thyroid gland enlargement
   1. Congenitalenlarged thyroid gland
   2. Acquired enlarged thyroid gland
      1. Chroniclymphocytic thyroiditis (Hashimoto thyroiditis)
      2. Colloid goiter
      3. Graves disease
      4. Acute thyroiditis
      5. Subacute thyroiditis
      6. Thyroid neoplasm
5. Neoplasm

Clinical Features and Diagnosis

Congenital Masses

Branchial Cleft Anomalies

Anomaliesfrom first branchial cleft can include draining sinus anterior toear or small cyst overlying parotid gland.

Second branchial cleft anomalies aremost common: ostium of sinus tract or fistula may be seen alonganterior border of lower aspect of sternocleidomastoid muscle.

Less common are anomalies of thirdand fourth branchial clefts, which occur in lower neck area.

Any of these anomalies may become infected.

Diagnosis is usually clinical, althoughCT may be useful in identification of deep cysts.

Preauricular Pits, Sinuses, and Cysts

Abnormal development of auditory tuberclesis responsible for preauricular pits, sinuses, and cysts, whichare located anterior to tragus of the ear. Drainage of sebaceousmaterial as well as infection may occur.

Thyroglossal Duct Cyst

May occuranywhere along the line of descent of thyroid from base of tongueto pyramidal lobe of thyroid gland. Usually located in midline ofneck in the area of hyoid bone and may move with swallowing or protrusionof tongue.

Some cysts may not be recognized untilthey become infected.

Thyroid scan can help distinguish thyroglossalcyst from ectopic thyroid tissue.

Diagnosis is confirmed by histologicexam.

Dermoid Cyst

May occurin midline of neck overlying hyoid bone.

Its spherical shape and yellow castunder the skin when observed in reflected light help distinguishit from thyroglossal duct cyst. Excisional biopsy confirms the diagnosis.

Congenital Muscular Torticollis (Fibromatosis Colli)

1- to 2-cmfirm mass in sternocleidomastoid muscle is noted in neonatal period.Indicative of fibrosis of muscle, which occurred in utero. Headis turned away from involved muscle, resulting in torticollis.

Diagnosis is usually clinical and canbe confirmed by U/S.

Muscular torticollis also may occurwithout a mass; the muscle may be shortened and firm.

Lymphatic Malformation

The termlymphatic malformation is now used to describe cystic hygroma. Thissoft, discrete, nontender, usually compressible, cystic mass occursmost commonly in posterior triangle of neck but also can occur inaxilla or mediastinum. May transilluminate.

Hemorrhage or infection can cause rapidenlargement that may compromise the airway.

Although U/S can determineits cystic nature, CT is valuable in determining its extent and involvementof other structures.

Laryngocele

Air-filled mass arising from laryngeal ventriclethat can cause airway obstruction during infancy. Respiratory distresscan worsen during crying with increased distension of air in laryngocele(see Chap. 63, Stertor, Stridor,and Airway Obstruction).

Thymic Cyst

Can presentin midline or lateral neck areas but is rare.

U/S demonstrates cyst, butdiagnosis is confirmed histologically.

Vascular Anomalies

2 rare vascularanomalies may occur as neck masses.

Dilatation of jugular vein (jugularvein phlebectasia) appears as cystic swelling anterior to sternocleidomastoidmuscle and increases in size with straining and crying. Diagnosismay be confirmed by CT with IV contrast enhancement, MRI, or digitalsubtraction angiography.

Aneurysm of internal or common carotidartery may present as pulsatile cervical neck mass, and magneticresonance angiography or conventional angiography is diagnostic.

Enlarged Lymph Nodes

Infection

Lymph nodes of the neck include superficialand deep cervical chains as well as submandibular and submentalnodes. Most cases of cervical adenitis can be classified as acuteunilateral, acute bilateral, or chronic bilateral.

Acute Unilateral Cervical Adenitis

Common causein infants 2–6 wks of age is group B streptococcal cellulitis-adenitis syndrome.In children 1–4 yrs of age, usual pathogens are S. aureusor group A Streptococcus.

Acute onset of fever, irritability,and facial or submandibular inflammation characterize this syndrome,which is often associated with bacteremia. In some cases an abscessmay form.

Acute Bilateral Cervical Adenitis

Most commoncauses are viral upper respiratory infections and group A streptococcal pharyngitis.Other causes include herpes gingivostomatitis, human herpesvirus6 (roseola), enteroviruses, adenoviruses, Epstein-Barr virus, cytomegalovirus,and M. pneumoniae. Periodontal infections and dental abscesses mayresult in adenitis secondary to anaerobic organisms. Much less commonare infections caused by F. tularensis (tularemia), P. multocida(from animal bite or scratch), and Y. pestis (plague).

Lymph nodes are enlarged and may betender.

Chronic Unilateral Cervical Adenitis

Cat scratch disease, mycobacterial infections,and toxoplasmosis are common causes.

Cat Scratch Disease

B. henselaeis the only Bartonella species known to be associated with cat scratchdisease.

Scratches to face or shoulder by cator kitten may lead to infection of cervical nodes, which often swellto >4 cm in diameter. Spontaneous drainage sometimes occurs.Constitutional symptoms are usually mild.

History is often diagnostic, althoughserologic tests are available. Excisional biopsy may be necessaryfor chronically draining node.

Mycobacterial Infection

M. tuberculosisinfection may involve superficial or deep cervical nodes as extension fromprimary lesion in upper lung fields. Onset is usually 6–9mos after initial infection. Involvement is usually unilateral,with discrete, firm, nontender nodes. Other than low-grade fever,individuals are usually asymptomatic. Tuberculin test is usuallyreactive, and abnormal chest radiograph is common.

Infection with nontuberculous mycobacteriausually occurs in children 1–4 yrs of age. Most commonpathogen is M. avium complex, whereas other species include M. scrofulaceum,M. kansasii, and M. haemophilum. Pain, tenderness, and constitutionalillness are minimal, yet fluctuance and spontaneous drainage maydevelop. Mantoux test may show induration of 5–9 mm. Chestradiograph is normal.

Sometimes distinguishing M. tuberculosisinfection from nontuberculous mycobacteria infection is difficult.

Chronic nontenderadenopathy with tissue breakdown and sinus tracts may occur in both.

Chest radiograph is always normal innontuberculous infection and may be normal in tuberculous infection.

Results of tuberculin test usuallyare positive in tuberculous infection and may be negative in somenontuberculous infections.

Important diagnostic clue is that nontuberculousinfection usually occurs in children <3 yrs of age withcomplete absence of illness. Often excisional biopsy and cultureof lymph node are required for definitive diagnosis.

Toxoplasmosis

Acquiredtoxoplasmosis often presents as cervical node enlargement, usuallyin posterior cervical triangle. Node may or may not be tender, andit does not suppurate. Low-grade fever, malaise, anorexia, and coughalso may occur.

Diagnosis is usually made by serology.

Noninfection

Kawasaki Disease

Cervical lymph node enlargement is leastcommon principal diagnostic criterion of Kawasaki disease (see Chap. 21, Fever).

Sarcoidosis

Enlarged cervical or supraclavicular lymphnodes may be found in sarcoidosis. Other findings can include enlargedmediastinal lymph nodes, pulmonary disease, and uveitis (see Chap. 38, Lymphadenopathy).

Histiocytic Necrotizing Lymphadenitis (Kikuchi-Fujimoto Disease)

Rare disorderof unknown cause.

Characteristic findings include bilateral,enlarged, tender cervical nodes; fever; vomiting; hepatosplenomegaly;night sweats; weight loss; and leukopenia.

Lymph node biopsy confirms diagnosis.

Other

Other causes of cervical adenopathy includelocal hypersensitivity reactions to stings or insect bites, drugs(phenytoin), collagen vascular disease, reactive hyperplasia, andunknown causes.

Trauma

Hematoma

Trauma from falls, contact sports, or motorvehicle accidents may cause hematoma formation, and vital structures(e.g., trachea and carotid artery) may be compromised.

Subcutaneous Emphysema

Air in mediastinum produced by trauma tobronchopulmonary tree may dissect into neck and produce subcutaneousemphysema with crepitance. History, physical exam, and chest andneck radiographs are diagnostic.

Thyroid Gland Enlargement

Generally, thyroid is enlarged if laterallobes can be distinctly defined by palpation. Normal thyroid function,hypothyroidism, or hyperthyroidism can exist with enlarged thyroidgland.

Congenital Enlarged Thyroid Gland

Maternalingestion of antithyroid drugs (propylthiouracil, methimazole) duringpregnancy inhibits thyroid hormone synthesis, which triggers increasein TSH secretion and fetal thyroid enlargement.

If a mother has Graves disease, transplacentalpassage of TSH receptor-stimulating antibodies causes neonatal Gravesdisease with thyroid enlargement.

Enlarged thyroid gland at birth orearly infancy also may be caused by defective thyroid hormone synthesisfrom metabolic defect or iodine deficiency, although the latteris rare in U.S.

Low serum thyroxine (T₄)stimulates production of TSH and subsequent enlargement of the gland.In neonates, primary hypothyroidism must be treated as soon as possible inattempt to prevent mental retardation. Precise metabolic defectcan be diagnosed, if necessary, when child is older.

Acquired Enlarged Thyroid Gland

Chronic Lymphocytic Thyroiditis (Hashimoto Thyroiditis)

Most commoncause of acquired thyroid enlargement in pediatric population.

Children usually present with enlargednontender thyroid gland and normal thyroid function or with hypothyroidismwith or without thyroid enlargement.

Most individuals with lymphocytic thyroiditishave positive thyroid peroxidase antibodies. Antithyroglobulin antibodiesare often positive as well.

Although diagnosis may be confirmedby biopsy, one is rarely needed for clinical management.

Colloid Goiter

Second mostcommon cause of acquired thyroid gland enlargement. Occurs almost exclusivelyin girls, with onset usually in adolescence.

Test results of thyroid function arenormal, and antithyroid antibodies are nondetectable.

This is a diagnosis of exclusion.

There is no association between colloidgoiter and cancer.

Graves Disease

Hyperthyroidismin children is almost exclusively due to Graves disease, which isan autoimmune disorder.

Besides enlarged thyroid gland, otherfindings include tachycardia, palpitations, tremor, heat intolerance,fatigue, emotional lability, worsening school performance, systolichypertension, prominence of eyes (exophthalmus), and increased appetitewith weight loss.

Elevated serum T₄ orfree T₄ and low serum TSH signify hyperthyroidism.Measurement of serum TSH receptor-stimulating antibodies helps confirm diagnosis.

Acute Thyroiditis

Commonlycaused by group A Streptococcus, S. aureus, S. pneumoniae, or anaerobic organisms.

Thyroid gland is enlarged, tender,warm, and occasionally fluctuant. Aspiration may yield pus.

Gram-stained smear and culture usuallyconfirm diagnosis.

Thyroid function is usually normal,although transient elevations in T₄ can occur.

Subacute Thyroiditis

Usually follows viral illness (enteroviruses,adenoviruses, Epstein-Barr virus, influenza viruses, mumps virus)and is insidious in onset. Usual findings are low-grade fever andenlarged, tender gland.

Thyroid Neoplasm

Benign tumorsof thyroid gland include adenoma and teratoma. Malignant tumorsinclude various types of thyroid carcinoma and metastatic tumors.

Enlarged thyroid gland, lobe, or masswithin the gland may be palpable.

History of neck radiation; family historyof multiple endocrine neoplasia; or presence of hard, fixed, rapidlyenlarging, solid mass suggests malignancy. Enlarged metastatic cervicallymph nodes commonly occur in children with thyroid cancer.

Histologic diagnosis is definitive.

Neoplasm

Benign Tumors

Hemangiomasare commonly found in head and neck regions. They are soft, mobile, nontenderlesions with reddish-blue skin discoloration that can be seen atbirth. They usually enlarge during first year of life and regressin next few years. Diagnosis is usually obvious on physical exam.

Typical presentation of lipoma is painless,mobile, slowing enlarging, subcutaneous cervical mass.

Other tumors (e.g., neurofibromas andteratomas) also may arise in cervical area.

Calcification on plain radiographyor mixed echogenicity with solid and cystic components on U/Ssuggests presence of teratoma, which is rarely malignant.

With other tumors besides hemangioma,histologic diagnosis is confirmatory.

Malignant Tumors

Malignancyshould be considered with rapid enlargement of painless, firm, fixedcervical mass.

In children <6 yrs of age,most common malignant neck masses are neuroblastoma, non-Hodgkinlymphoma, rhabdomyosarcoma, and Hodgkin disease.

Between 7 and 13 yrs of age, Hodgkindisease and non-Hodgkin lymphoma occur with equal frequency, whereasrhabdomyosarcoma and thyroid carcinoma are less common.

Adolescents are usually affected withHodgkin disease.

Other tumors include nasopharyngealcarcinoma, thyroid tumors (discussed above), and metastatic leukemia.

Complete physical exam is imperative,with careful exam of ears, nose, mouth, pharynx, and larynx becauseprimary tumor may be in these locations.

Neck and chest radiographs, CT, andMRI help locate and define extent of tumor. Histologic diagnosisis definitive.

Neuroblastoma

Neuroblastoma in neck region is usually dueto metastasis from other sites; however, primary cervical neuroblastomacan present as firm mass in lateral part of neck.

Hodgkin Disease

Usuallyoccurs in adolescents.

Commonly presents as painless enlargednode or nodes in neck or supraclavicular area. Other nonspecificsymptoms include fever, night sweats, weight loss, anorexia, andpruritus.

Non-Hodgkin Lymphoma

In contrastto Hodgkin disease, usually occurs in children 2–12 yrsof age.

Generalized lymphadenopathy is morecommon than in Hodgkin disease, but enlarged nodes can occur inneck area.

Rhabdomyosarcoma

Firm enlarging mass in cervical region mayoccur with rhabdomyosarcoma and other sarcomas.

Nasopharyngeal Carcinoma

Rare tumor in pediatric population. Mostchildren present with nasopharyngeal obstruction. Deep cervicallymph nodes may be enlarged.

Diagnostic Approach

Congenital vs Acquired Masses

Congenitalmasses (e.g., branchial cleft anomalies or thyroglossal duct cysts)may not be recognized until infancy or childhood when they enlargeor become infected.

Acquired neck masses can be enlargedlymph nodes, hematomas, enlarged thyroid gland, or neoplastic lesion.Most common are enlarged lymph nodes due to reactive hyperplasiaor cervical adenitis.

Cervical Adenitis

Importantto determine whether adenitis is acute or chronic and unilateralor bilateral.

In infants, most common pathogen causingacute unilateral adenitis is S. aureus, whereas in children, mostcommon pathogens are S. aureus and group A Streptococcus.

Usual causes of acute bilateral cervicaladenitis are a viral syndrome or group A Streptococcus. Infectiousmononucleosis should also be considered.

Cat scratch disease and nontuberculousmycobacterial infection are most common causes of chronic unilateralcervical adenitis.

When tuberculosis is suspected, a Mantouxtuberculin test (5TU) and a chest radiograph should be performed.

Persistent Lymph Node

Diagnosticdilemma is persistence of lymph nodes in asymptomatic children.Observation over ensuing 6–8 wks for spontaneous resolutionis reasonable.

If node enlarges, is hard, or is fixedto underlying tissue, biopsy should be performed.

If biopsy shows nonspecific hyperplasia,patient must be followed carefully because a small number may developlymphoreticular malignancies.

References

1. Bamji M, et al. Palpable lymph nodesin healthy newborns and infants. Pediatrics 1986;78:573–575.
2. Behrman RE, et al., eds. Nelson textbook of pediatrics,16th ed. Philadelphia: WB Saunders, 2000.
3. Bergman KS, Harris BH. Scalp and neck masses. PediatrClin North Am 1993;40:1151–1160.
4. Bluestone CD, et al., eds. Pediatric otolaryngology,3rd ed. Philadelphia: WB Saunders, 1996.
5. Brown RL, Azizkhan RG. Pediatric head and neck lesions.Pediatr Clin North Am 1998;45:889–905.
6. Chesney PJ. Cervical lymphadenitis and neck infections.In: Long SS, et al., eds. Principles and practice of pediatric infectiousdiseases. New York: Churchill Livingstone, 1997:186–197.
7. Cotton RT, Myer CM III, eds. Practical pediatric otolaryngology.Philadelphia: Lippincott-Raven, 1999.
8. Feigin RD, Cherry JD, eds. Textbook of pediatric infectiousdiseases, 4th ed. Philadelphia: WB Saunders, 1998.
9. Hopwood NJ, Kelch RP. Thyroid masses: approach to diagnosisand management in childhood and adolescence. Pediatr Rev 1993;14:481–487.
10. Lake AM, Oski FA. Peripheral lymphadenopathy in childhood:ten-year experience with excisional biopsy. Am J Dis Child 1978;132:357–359.
11. Long SS, et al., eds. Principles and practice of pediatricinfectious diseases. New York: Churchill Livingstone, 1997.
12. McAneney CM, Ruddy RM. Neck mass. In: Fleisher GR,Ludwig S, eds. Textbook of pediatric emergency medicine, 4th ed.Philadelphia: Lippincott Williams & Wilkins, 2000:383–389.
13. Potsic WP. Neck masses. In: Schwartz MW, ed. Pediatricprimary care: a problem-oriented approach, 3rd ed. St. Louis: Mosby-YearBook, 1997:324–327.
14. Rudolph AM, ed. Rudolph's pediatrics, 20thed. Stamford, CT: Appleton & Lange, 1996.
15. Zitelli BJ. Lymphadenopathy. In: Gartner JC Jr, ZitelliBJ, eds. Common and chronic symptoms in pediatrics. St. Louis: Mosby-YearBook, 1997:365–380.

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Book Source Details

• Book Title: The Diagnostic Approach to Symptoms and Signs in Pediatrics
• Author(s): Paul S. Bellet
• Year of Publication: 2006
• Copyright Details: The Diagnostic Approach to Symptoms and Signs in Pediatrics, Copyright © 2006 Lippincott Williams & Wilkins.

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