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Neck Masses, Pediatric

Basics

Description

- CBC - Infections: leukocytosis - Mononucleosis: atypical lymphocytosis - Kawasaki: thrombocytosis after 1st week - Neck malignancies: usually normal initially

- Complete metabolic panel with lactate dehydrogenase (LDH) and uric acid when malignancy is suspected - "Mono spot " ¯ (mononucleosis) test: less reliable in children <4 years old; EBV titers more useful - Indirect fluorescent antibody titers for Bartonella: confirms cat-scratch disease - Purified protein derivative: negative or weakly positive in atypical mycobacterial infections - Chest radiograph: important in all evaluations when malignancy is a possibility; adenopathy seen in malignancies and tuberculosis; cavitary lesions and infiltrates in tuberculosis - Lateral neck radiograph: prevertebral soft tissue space at C2 " “C3 abnormally wide (>1/2 adjacent vertebral body diameter) in cases of retropharyngeal abscess - Ultrasound - 1st-line imaging modality in neck masses - provides immediate, noninvasive information on location, size, and composition of mass (cystic vs. solid) - Doppler adds information about vascularity.

- Cat-scratch disease - A usually self-limited, although sometimes protracted, illness (2 " “4 months) - Caused by the gram-negative bacillus Bartonella henselae - Starts as a papule at a cat-scratch site; progresses to regional adenopathy, 5 " “50 days later (median, 12 days) - Axillary adenopathy is most common; cervical nodes 2nd most common. - Adenopathy for weeks to months

- Infectious mononucleosis: EBV infection most commonly seen in older children who present with fever, exudative pharyngitis, adenopathy, and hepatosplenomegaly. - Toxoplasmosis - Parasitic disease caused by Toxoplasma gondii which presents with cervical adenopathy, rash, fever, malaise, and hepatosplenomegaly - Acquired from contact with cat feces or inadequately cooked meat

- Peritonsillar abscess: suppurative sequela of a severe tonsillopharyngitis, usually caused by group A ˇ ²-hemolytic Streptococcus (GABHS); commonly presents in older children and adolescents with trismus, "hot potato " ¯ voice, and uvular deviation from a bulging palatal abscess - Ludwig angina - Rapidly expanding, diffuse inflammation of the submandibular/sublingual spaces - May compromise the airway - Often occurs with dental infections

- Congenital - Branchial cleft cyst: common congenital neck lesion (usually a remnant of the 2nd branchial cleft) which presents as a nontender (unless infected) cyst at the anterior border of the sternocleidomastoid - Thyroglossal duct cyst: common congenital neck mass which is a remnant of the embryonic thyroglossal sinus and presents as a nontender (unless infected), mobile, anterior midline mass near the hyoid bone - Cystic hygroma (lymphangioma): complex, multiloculated mass of lymphatic tissue, which presents in the 1st year of life as a large, soft, compressible mass in the posterior triangle of the neck; may cause airway obstruction - Dermoid cyst: small, firm, nontender mass, usually high in the midline - Hemangioma: bluish purple, blanching mass characterized by rapid growth in the 1st year of life, then slow regression - Sternocleidomastoid (pseudo) tumor of infancy (congenital muscular torticollis): benign perinatal fibromatosis, often associated with difficult deliveries or abnormal uterine positioning, that results in a hard, immobile, fusiform mass in the sternocleidomastoid - Laryngocele: cystic dilation of the laryngeal saccule; presents as an air-filled cyst or as a foreign body sensation with coughing - Cervical wattle: benign pedunculated congenital anomaly on lateral neck with a core of elastic cartilage - Cervical bronchogenic cyst: cervical neck mass in the anteromedial neck (superior to the sternal notch), resulting from abnormal development of the tracheobronchial tree - Thymic cyst: ectopic thymic mass resulting from abnormal development of pharyngeal pouches and branchial clefts - Teratoma: malformation of all three germ layers that can cause significant airway obstruction as well as feeding dysfunction - Ranula: a mucocele created by obstruction of the sublingual salivary glands; usually a painless, slowly accumulating mass

- Malignant - Hodgkin lymphoma: slowly enlarging, unilateral, firm, nontender neck malignancy; usually presents in previously well adolescents - Non-Hodgkin lymphoma: presents in young adolescents as a painless, rapidly growing, firm collection of lymph nodes - Leukemia: most common tumor associated with cervical adenitis in first 6 years of life - Neuroblastoma: commonly presents in infants/young children as a large, nontender abdominal mass; associated with a myriad of signs and symptoms due to its propensity for metastasis - Rhabdomyosarcoma: head and neck malignancy that usually presents as a rapidly enlarging mass - Melanoma: an increasingly identified cause of neck malignancy in pediatrics

- Thyroid - Chronic lymphocytic thyroiditis (Hashimoto thyroiditis): autoimmune childhood goiter that may be eu-, hypo-, or hyperthyroid - Thyrotoxicosis (Graves disease): clinically hyperfunctioning thyroid caused by circulating thyroid cell " “stimulating antibodies - Thyroiditis: painful bacterial infection of the thyroid caused by Staphylococcus or Streptococcus

- Miscellaneous - Kawasaki disease - Idiopathic vasculitis distinguished by prolonged fever, conjunctivitis, oral involvement, extremity changes, rash, and unilateral cervical node >1.5 cm - Cervical node: least common feature

- Sinus histiocytosis with massive lymphadenopathy (Rosai " “Dorfman disease); benign form of histiocytosis that presents as massive, painless enlargement of cervical nodes - Hematoma: secondary to trauma - Hypersensitivity reaction: secondary to bites, stings, or other allergens - Drugs: Notably, phenytoin and isoniazid may be associated with lymphadenopathy. - Immunization: Adenopathy may follow DPT or polio immunization.

A mass in the tissues of the neck; cervical adenopathy is defined as a neck node >1 cm. ‚

Etiology

Varies depending on underlying condition. ‚

Diagnosis

To diagnose and appropriately manage neck masses, one must combine the history with a careful examination of the mass. The major task of the differential diagnosis is to distinguish infections from congenital and malignant causes. ‚

History

  • Fever: infection, Kawasaki disease, malignancy, "PFAPA " ¯ (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome
  • Intercurrent infection: reactive hyperplasia, mononucleosis, abscess, congenital cyst
  • Subacute or chronic cervical lymphadenitis: cat-scratch disease, toxoplasmosis, Epstein-Barr virus (EBV), and mycobacterial infection
  • Increasing size: infection, newly infected congenital lesion, malignancy (less common)
  • Sore throat: mononucleosis, peritonsillar or retropharyngeal abscess
  • Swallowing problems: retropharyngeal or peritonsillar abscess, thyroglossal duct cyst
  • Cat contact: cat-scratch disease, toxoplasmosis
  • Recurrently infected mass: infected congenital cyst (thyroglossal duct, branchial cleft)
  • Mass noticed neonatally: cystic hygroma, hemangioma, sternocleidomastoid tumor of infancy
  • Weight loss, cough, chronic constitutional symptoms: malignancy, tuberculosis
  • Hypothyroid or hyperthyroid symptoms: thyroglossal duct cyst, thyroidal diseases

Physical Exam

  • Tender, erythematous, indurated mass may indicate cervical adenitis, infected congenital lesion, or cat-scratch disease
  • Nontender, enlarged lymph nodes(s) suggest reactive hyperplasia or malignancy.
  • Fluctuant mass may indicate adenitis with abscess or cystic hygroma.
  • Drainage suggests adenitis with abscess, atypical mycobacterial disease, infected thyroglossal duct, or branchial cleft cyst.
  • Regional adenopathy: reactive hyperplasia, cat-scratch disease, or malignancy
  • Exudative pharyngitis: mononucleosis
  • Asymmetric soft palate with uvular deviation suggests peritonsillar abscess.
  • Pulmonary findings: tuberculosis, malignancy
  • Midline mass suggests thyroglossal duct or dermoid cyst or thyroidal disease.
  • If mass moves with tongue protrusion, thyroglossal duct cyst may be present.
  • Sinus opening may indicate thyroglossal duct, branchial cleft, or dermoid cyst.
  • Multiloculated mass that transilluminates suggests cystic hygroma.
  • Matted-down mass may indicate malignancy.
  • Mass posterior to sternocleidomastoid muscle may be malignancy or infection.
  • Inferior deep cervical nodes (scalene and supraclavicular) suggest malignancy.
  • Generalized adenopathy suggests malignancy.
  • Hepatosplenomegaly may indicate malignancy or infectious mononucleosis.
  • Skin discoloration suggests trauma, abscess, or atypical mycobacterial disease.
  • A skin papule is a clue to cat-scratch disease.
  • Conjunctivitis, oral involvement, extremity changes, rash, and adenopathy, in the context of fever: Suspect Kawasaki disease.
  • Torticollis in a neonate suggests sternocleidomastoid (pseudo) tumor of infancy.

Diagnostic Tests & Interpretation

  • CBCInfections: leukocytosisMononucleosis: atypical lymphocytosisKawasaki: thrombocytosis after 1st weekNeck malignancies: usually normal initially
  • Complete metabolic panel with lactate dehydrogenase (LDH) and uric acid when malignancy is suspected
  • "Mono spot " ¯ (mononucleosis) test: less reliable in children <4 years old; EBV titers more useful
  • Indirect fluorescent antibody titers for Bartonella: confirms cat-scratch disease
  • Purified protein derivative: negative or weakly positive in atypical mycobacterial infections
  • Chest radiograph: important in all evaluations when malignancy is a possibility; adenopathy seen in malignancies and tuberculosis; cavitary lesions and infiltrates in tuberculosis
  • Lateral neck radiograph: prevertebral soft tissue space at C2 " “C3 abnormally wide (>1/2 adjacent vertebral body diameter) in cases of retropharyngeal abscess
  • Ultrasound1st-line imaging modality in neck massesprovides immediate, noninvasive information on location, size, and composition of mass (cystic vs. solid)Doppler adds information about vascularity.
  • CT or MRI scan: useful in evaluating deep neck infections and complex neck massesCT advantages: more readily available; shorter study; less need for sedationMRI advantages: no ionizing radiation; better soft tissue resolution
  • Thyroid scintigraphy: useful when malignancy is a concern
  • Gram stain and culture of specimen after needle aspiration or incision and drainage: diagnostic and therapeutic with infections
  • Histologic evaluation after fine-needle aspiration or biopsy: diagnostic for malignant versus congenital versus infectious causes

Differential Diagnosis

  • InfectiousReactive hyperplasia: self-limited, enlargement of bilateral minimally tender nodes; usually viralBacterial lymphadenitisUsually staphylococcal or streptococcal infection of unilateral, tender, swollen, warm, erythematous nodeCellulitis " “adenitis syndrome in neonates caused by group B StreptococcusCat-scratch diseaseA usually self-limited, although sometimes protracted, illness (2 " “4 months)Caused by the gram-negative bacillus Bartonella henselaeStarts as a papule at a cat-scratch site; progresses to regional adenopathy, 5 " “50 days later (median, 12 days)Axillary adenopathy is most common; cervical nodes 2nd most common.Adenopathy for weeks to monthsTuberculosis: acute or insidious onset of fever and firm, nontender adenopathy in children exposed to adult infected with acid-fast bacillus Mycobacterium tuberculosisAtypical mycobacterial diseaseInfection usually caused by Mycobacterium avium complex or Mycobacterium scrofulaceum (ubiquitous agents found in the soil)Rapidly enlarging mass of firm, nontender nodes in young children with no known exposure to tuberculosisNodes often occur with overlying skin discoloration and thinning; some spontaneously drain.Infectious mononucleosis: EBV infection most commonly seen in older children who present with fever, exudative pharyngitis, adenopathy, and hepatosplenomegaly.ToxoplasmosisParasitic disease caused by Toxoplasma gondii which presents with cervical adenopathy, rash, fever, malaise, and hepatosplenomegalyAcquired from contact with cat feces or inadequately cooked meatRetropharyngeal abscessSuppurative adenitis of the retropharyngeal nodes that presents in children <5 years of ageThese children often have fever, neck stiffness, dysphagia, respiratory distress, drooling, and stridor.Peritonsillar abscess: suppurative sequela of a severe tonsillopharyngitis, usually caused by group A ˇ ²-hemolytic Streptococcus (GABHS); commonly presents in older children and adolescents with trismus, "hot potato " ¯ voice, and uvular deviation from a bulging palatal abscessLudwig anginaRapidly expanding, diffuse inflammation of the submandibular/sublingual spacesMay compromise the airwayOften occurs with dental infections
  • CongenitalBranchial cleft cyst: common congenital neck lesion (usually a remnant of the 2nd branchial cleft) which presents as a nontender (unless infected) cyst at the anterior border of the sternocleidomastoidThyroglossal duct cyst: common congenital neck mass which is a remnant of the embryonic thyroglossal sinus and presents as a nontender (unless infected), mobile, anterior midline mass near the hyoid boneCystic hygroma (lymphangioma): complex, multiloculated mass of lymphatic tissue, which presents in the 1st year of life as a large, soft, compressible mass in the posterior triangle of the neck; may cause airway obstructionDermoid cyst: small, firm, nontender mass, usually high in the midlineHemangioma: bluish purple, blanching mass characterized by rapid growth in the 1st year of life, then slow regressionSternocleidomastoid (pseudo) tumor of infancy (congenital muscular torticollis): benign perinatal fibromatosis, often associated with difficult deliveries or abnormal uterine positioning, that results in a hard, immobile, fusiform mass in the sternocleidomastoidLaryngocele: cystic dilation of the laryngeal saccule; presents as an air-filled cyst or as a foreign body sensation with coughingCervical wattle: benign pedunculated congenital anomaly on lateral neck with a core of elastic cartilageCervical bronchogenic cyst: cervical neck mass in the anteromedial neck (superior to the sternal notch), resulting from abnormal development of the tracheobronchial treeThymic cyst: ectopic thymic mass resulting from abnormal development of pharyngeal pouches and branchial cleftsTeratoma: malformation of all three germ layers that can cause significant airway obstruction as well as feeding dysfunctionRanula: a mucocele created by obstruction of the sublingual salivary glands; usually a painless, slowly accumulating mass
  • MalignantHodgkin lymphoma: slowly enlarging, unilateral, firm, nontender neck malignancy; usually presents in previously well adolescentsNon-Hodgkin lymphoma: presents in young adolescents as a painless, rapidly growing, firm collection of lymph nodesLeukemia: most common tumor associated with cervical adenitis in first 6 years of lifeNeuroblastoma: commonly presents in infants/young children as a large, nontender abdominal mass; associated with a myriad of signs and symptoms due to its propensity for metastasisRhabdomyosarcoma: head and neck malignancy that usually presents as a rapidly enlarging massMelanoma: an increasingly identified cause of neck malignancy in pediatrics
  • ThyroidChronic lymphocytic thyroiditis (Hashimoto thyroiditis): autoimmune childhood goiter that may be eu-, hypo-, or hyperthyroidThyrotoxicosis (Graves disease): clinically hyperfunctioning thyroid caused by circulating thyroid cell " “stimulating antibodiesThyroiditis: painful bacterial infection of the thyroid caused by Staphylococcus or Streptococcus
  • MiscellaneousKawasaki diseaseIdiopathic vasculitis distinguished by prolonged fever, conjunctivitis, oral involvement, extremity changes, rash, and unilateral cervical node >1.5 cmCervical node: least common featurePFAPA syndromePeriodic fever, aphthous stomatitis, pharyngitis, and cervical adenitisIdiopathic, periodic, febrile syndrome most commonly seen in young childrenSinus histiocytosis with massive lymphadenopathy (Rosai " “Dorfman disease); benign form of histiocytosis that presents as massive, painless enlargement of cervical nodesHematoma: secondary to traumaHypersensitivity reaction: secondary to bites, stings, or other allergensDrugs: Notably, phenytoin and isoniazid may be associated with lymphadenopathy.Immunization: Adenopathy may follow DPT or polio immunization.

Treatment

General Measures

  • InfectiousAntibioticsIncision and drainage (I&D) of abscesses
  • CongenitalAntibiotics if infectedENT referral for surgical excision
  • Malignancy: oncology referral for chemotherapy/radiation/excision
  • Thyroidal: endocrine referral for pharmacotherapy
  • MiscellaneousKawasaki disease: IVIG and aspirin therapy to prevent coronary artery aneurysms; cardiology referral for echocardiographyPFAPA syndrome: Steroids (a single dose) are efficacious in aborting fever attacks.Sternocleidomastoid tumor of infancy: massage, range of motion, and stretching

Alert

Corticosteroids should not be given to neck mass patients until malignancy has been excluded, except in dire conditions of airway compromise. ‚

Ongoing Care

Close follow-up is essential for all neck masses; consider referral for biopsy in the following cases: ‚

  • Nodes not responding to antibiotics
  • Toxic illness/systemic symptoms
  • Clinical signs of malignancy (weight loss, peripheral adenopathy, hepatosplenomegaly)
  • Firm, nontender nodes fixed to deep tissues
  • Nodes posterior to the sternocleidomastoid or in the lower cervical/supraclavicular regions
  • Bilateral nodes >2 cm

Additional Reading

  • Al-Dajani ‚ N, Wootton ‚ SH. Cervical lymphadenitis, suppurative parotitis, thyroiditis, and infected cysts. Infect Dis Clin North Am. 2007;21(2):523 " “541, viii. ‚ [View Abstract]
  • Dulin ‚ MF, Kennard ‚ TP, Leach ‚ L, et al. Management of cervical lymphadenitis in children. Am Fam Physician. 2008;78(9):1097 " “1098. ‚ [View Abstract]
  • Friedman ‚ ER, John ‚ SD. Imaging of pediatric neck masses. Radiol Clin North Am. 2011;49(4):617 " “632. ‚ [View Abstract]
  • Geddes ‚ G, Butterly ‚ MM, Patel ‚ SM, et al. Pediatric neck masses. Pediatr Rev. 2013;34(3):115 " “124. ‚ [View Abstract]

Codes

ICD09

  • 784.2 Swelling, mass, or lump in head and neck
  • 785.6 Enlargement of lymph nodes
  • 682.1 Cellulitis and abscess of neck
  • 075 Infectious mononucleosis
  • 759.2 Anomalies of other endocrine glands

ICD10

  • R22.1 Localized swelling, mass and lump, neck
  • R59.0 Localized enlarged lymph nodes
  • L02.11 Cutaneous abscess of neck
  • B27.90 Infectious mononucleosis, unspecified without complication
  • Q89.2 Congenital malformations of other endocrine glands

SNOMED

  • 274751001 Mass in head or neck (finding)
  • 127086001 cervical lymphadenopathy (disorder)
  • 6284004 abscess of neck (disorder)
  • 271558008 Infectious mononucleosis (disorder)
  • 39462005 Thyroglossal duct cyst

FAQ

  • Q: How should nodes respond to therapy?
  • A: Consider referral for biopsy if increasing size after 2 weeks, no decrease in size >2 " “4 weeks, or not back to normal >8 " “12 weeks.
  • Q: Do all external neck abscesses need antibiotic therapy after drainage?
  • A: Many experts believe that antibiotics are not always necessary if I&D is done appropriately.
  • Q: Is antibiotic coverage for community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) necessary?
  • A: As CA-MRSA is increasingly common, an antibiotic agent with MRSA coverage is indicated; clindamycin is a common choice.