Neck Masses

Introduction

A neck mass is a common reason for presentation of pediatric patients to a pediatrician. Some 80-90% of cases are benign, with infectious etiologies predominating. However, many classes of pathology can present as a palpable neck mass. Accurate diagnosis often depends upon knowledge of local anatomy.

Neck Anatomy 

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https://en.wikipedia.org/wiki/Triangles_of_the_neck

https://en.wikipedia.org/wiki/Triangles_of_the_neck

Evaluation

Evaluation of a patient with a neck mass should include a thorough history and physical since many diagnoses can be made clinically. In the outpatient pediatric office, the clinician need not always confirm a diagnosis, but he or she should arrive at a rational plan to observe, treat, or refer. Outlined below is an approach to the differential diagnosis followed by an algorithm for management.

Differential Diagnosis

Although some causes of masses in the neck can present in almost any anatomical location, the localization of the mass can narrow the diagnostic possibilities:

Other historical, physical, lab, and imaging findings can assist in diagnosis of common neck masses. Salient features and basic details of treatment are provided below.

CONGENITAL

Thyroglossal duct cysts 

• Most common midline pediatric neck mass. 
• Arise from remnant embryological tissue due to failed involution of the thyroglossal duct, which runs from the base of the tongue to the eventual site of the thyroid in the neck. Cysts may then form anywhere along this midline path.
• It most frequently presents in the first 5 years of life as a painless cystic mass near the hyoid bone. The cyst moves up and down with swallowing and tongue protrusion.
• Cysts may become infected, causing the overlying skin to appear erythematous. Clinicians should ask about signs of hypothyroidism, which is a rare accompanying feature.
• Treatment is by elective surgical excision.

Dermoid cysts

• Cystic germ cell tumors that include epithelial and endothelial elements. They contain epithelial appendages including hair and glandular elements.
• May be difficult to distinguish from thyroglossal duct cysts as they can occur in the midline neck and may or may not move with swallowing or tongue protrusion.
• Generally painless but can become infected and painful. Ultrasonography and fine needle aspiration are useful in unclear cases.
• Treatment is by elective surgical excision.

Branchial cleft cysts

• Second most common congenital neck mass.
• Arise from a variety of forms of incomplete involution of pharyngeal clefts and pouches as the pharyngeal arches mature. Sinuses or entire fistulae can result.
• First cleft anomalies involve the external auditory canal and the submandibular area. Second cleft anomalies are most common and involve the anterior lower lateral neck. Third and fourth cleft anomalies are rare, but when infected may cause airway or swallowing difficulty.
• Presentation ranges from draining fistulae in infancy to asymptomatic palpable cysts in adulthood. Cysts may become infected.
• Treatment is surgical excision.

Preauricular cysts

• Thought to arise due to abnormal development of the external ear. Compared to branchial cleft cysts, they are more often bilateral, may be inherited, and are rarely infected. They do not have close relationships with any critical structures.
• Treatment is simple excision

Hemangiomas

• Common tumors in infancy and are often found on the neck. Rarely, hemangiomas are present and fully-grown at birth. Typical, non-congenital lesions appear 2-4 weeks after birth, proliferate rapidly for 6-8 months, then involute over several years.
• Appearance depends on their location, but they tend to be soft, red to blue, and raised, with these findings becoming more subtle the deeper they are found. A bruit may be auscultated.
• Additional lesions may be found in the glottis and subglottis and threaten the airway, particularly when external hemangiomas are present in the beard area. These infants should be inspected for such life-threatening airway involvement.
• Cervical or facial hemangiomas are a feature of PHACE Syndrome (posterior fossa lesions, hemangioma, arterial cerebrovascular anomalies, cardiac and aortic anomalies, eye anomalies) syndrome.
• Alone and uncomplicated, hemangiomas may be observed since they are likely to self-resolve.  Otherwise, first line treatment is topical or systemic propranolol; other treatment options include laser therapy, steroids (intralesional or systemic), and surgery. Lesions that bleed, ulcerate, become infected, or threaten key structures may be treated similarly. 

Vascular malformations

• May have lymphatic, arterial, and/or venous features, and can sometimes be diagnosed on prenatal ultrasound. Postnatal ultrasound is useful to characterize flow patterns and cystic features. Vascular malformations are further subcategorized as follows:
   
  • Venous malformations: low-flow, thin-walled, compressible structures that may expand over time, bleed, cause pain, or develop phleboliths (calcified thrombi). These are best diagnosed by MRI. Surgery, sclerotherapy, and laser therapy are treatment options.
  • Capillary malformations: superficial low-flow ectatic capillary channels that form “port-wine stains.” These are associated with Sturge-Weber syndrome and can be treated with laser therapy if they are disfiguring.
  • Lymphatic malformations: formerly known as lymphangiomas or cystic hygromas, these low-flow masses are spongy, mobile, nontender, and often posterior and left-sided. Chest x-ray should be performed to look for extension into the mediastinum. Treatment depends on the size and features of the lesion and ranges from simple excision to multi-stage excision to intralesional sclerotherapy. Trauma including fine needle aspiration can cause major bleeding into the lesion and should be avoided. Other complications include infection, lymphatic leakage, and chylothorax, which may necessitate hospitalization.
  • Arterio-venous malformation: have arterial input with no capillary beds, and thus are high-flow with pulsatility. AVMs in the neck typically present in teenage years as an expanding mass and may cause bleeding or local tissue damage. Sometimes a bruit or thrill is detectable. MRI/MRA is useful for guiding treatment, which may include surgical removal, embolization, or sclerotherapy.
  • Combined malformation: multiple elements detected on imaging, which should include MRI/MRA. These complicated lesions require multidisciplinary approaches.

INFLAMMATORY

Lymphadenitis

• Refers to reactive enlargement and tenderness of lymph nodes due to local infection or inflammation. Palpable cervical lymphadenopathy is reported in 28-55% of otherwise normal pediatric patients, including infants.
• Viral respiratory tract and streptococcal pharyngeal infections are the most common causes of bilateral adenitis. In unilateral adenitis, staphylococcal and streptococcal (including group B in infancy) infections predominate. Anaerobes may be associated with dental caries or other oral lesions.
• A listing of common infectious causes of adenitis is provided:

Geddes G, Butterly MM, Patel SM, Marra S. Pediatric Neck Masses. Pediatr Rev. 2013 Mar 1;34(3):115–25. 

• Workup of enlarged lymph nodes is guided by a few key principles:
     
  • Clinicians should be familiar with the presentations and risk factors of the infections in the figure above, the details of which are not provided on this page.
  • A thorough search on physical exam, especially the HEENT exam, for a source of infection should be completed. Suspected infection may be managed initially with a trial of antibiotics targeting the most likely agents. Commonly, cephalexin, amoxicillin/clavulanate, or clindamycin are appropriate. Treatment should cover at least 5 symptom-free days. Enlarged nodes may then be observed for up to four weeks.
  • Abscesses, including suppurative lymphadenitis, that do not respond to oral antibiotics should be referred for IV antibiotics or surgical excision.
  • Other suspected infections that do not respond to initial antibiotic treatment should prompt re-evaluation. Labs including a complete blood count with differential and infectious titers of CMV, EBV, HIV, and toxoplasmosis may assist in diagnosis.
  • Signs concerning for lymphoma indicate urgent referral.
  • Steroids should not be administered in the absence of a firm diagnosis.

Sternocleidomastoid tumor of infancy

• Presents at or shortly after birth in most cases. Its etiology is unknown, but it is characterized by fibroblastic proliferation and multinucleated muscle cells on pathological examination.
• Infants present with torticollis, head tilt, and a firm, nontender, 2-3cm mass in the middle to lower sternocleidomastoid muscle. Fine needle aspiration can confirm the diagnosis.
• Management is conservative, as physiotherapy resolves the issue in most cases.

Sialadenitis

• An inflammation of one or more salivary glands (parotid, submandibular, submental) and may be unilateral or bilateral. It has many potential causes but is most commonly secondary to a viral infection, including mumps, HIV, or CMV. Bacterial infection including abscess formation, especially, due to Staphylococcus aureus, is possible. Mycobacterial and several other infections can have salivary gland involvement.
• Obstruction by stones, which may be palpable along the salivary course, can cause enlargement and inflammation, possibly requiring surgical removal.
• Inflammation as a feature of sarcoidosis or Sjogren’s syndrome, though rare in children, is possible.
• Juvenile recurrent parotitis presents as recurrent idiopathic acute swelling lasting days to weeks; both the acute swelling and the chronic condition self-resolve, requiring only symptomatic treatment.
• Pneumoparotitis caused by failing valves in patients with increased intraoral pressure (e.g. instrument players, SCUBA divers) is a rare cause of a pediatric neck mass.
• Given the wide array of possible causes, ultrasound and fine needle aspiration are the most useful initial diagnostic modalities. A helpful listing of the differential of parotid enlargement with images is provided at the LINK BELOW

http://radiopaedia.org/articles/parotid-enlargement

NEOPLASTIC

Teratomas

• Contain all 3 germ cell layers and can occasionally present in the neck. They are usually picked up before or at birth, may become very large, and can cause significant airway compromise. They are rarely malignant. After the airway is secured, they should be removed surgically.

Thyroid tumors

• Much less common in pediatric patients than in adults. Estimates of malignancy in thyroid nodules range from 9-50%, and patients frequently present with metastasis to cervical lymph nodes or beyond. Risk factors include female gender, family history of MEN-2 syndromes, and prior radiation exposure.
• Workup should begin with thyroid function tests, with I-123 uptake scanning for hyperfunctioning lesions. Ultrasonography is essential for visualizing thyroid nodules.
• Suspected cancerous lesions may be diagnosed by fine needle aspiration. Papillary thyroid carcinoma is most common at 80-95% of cases, followed by follicular at 5-15% and medullary at 5-10%.
• Medullary thyroid cancer diagnosis should prompt genetic evaluation for MEN-2.
• Management is surgical with possible radioactive iodine therapy. Prognosis is generally good, although cases of anaplastic thyroid carcinoma, which is fortunately rare in children, are associated with significantly worse prognosis.

Salivary gland tumors

• May be benign or malignant. The most common tumor, representing 65% of cases, is benign pleomorphic adenoma, treatment for which is surgical resection. Common malignant tumors include mucoepidermoid carcinoma, acinus cell carcinoma, and adenoid cystic carcinoma.
• Ultrasound, MRI or CT, and biopsy aid in diagnosis. Partial or complete excision of the gland is indicated in most cases, with radiotherapy occasionally added.

Lymphoma

• Accounts for half of pediatric head and neck malignancy, with non-Hodgkin’s lymphoma in 60% of cases and Hodgkin’s lymphoma in 40%.
• Hodgkin’s lymphoma usually appears after 10 years of age and presents with painless supraclavicular or cervical lymphadenopathy. Non-Hodgkin’s lymphoma has a male predominance and tends to arise in extra-nodal tissues.
• Concern for lymphoma should be increased with the following features:
   
  • unilateral
  • size > 2cm
  • hard, firm, rubbery consistency
  • fixed in place
  • supraclavicular node
  • accompanying B symptoms
  • enlargement for more than two weeks
  • no decrease in size after four to six weeks
  • absence of inflammation
  • ulceration
  • failure to respond to antibiotic therapy

Presence of these features, especially size, consistency, and B symptomatology, should prompt immediate workup. Further management depends on biopsy results and imaging findings; typically chemotherapy is necessary.

Rhabdomyosarcoma

• Accounts for 5% of pediatric cancers; when present, it originates in the head and neck region in 35% of pediatric cases. It is considered one of the small round blue cell tumors of childhood.
• Patients are often asymptomatic, although tumors can compress local structures and prompt evaluation.
• CT or MRI with full neck views to evaluate for cervical adenopathy is recommended, and incisional biopsy is typically used to secure the diagnosis.

Neuroblastoma

• More commonly presents in the abdomen or thorax, but occasionally presents as a neck primary tumor, where it may be found in the lateral neck or retropharyngeal space. It is a small round blue cell tumor of childhood and originates from neural crest tissue.
• In the neck in particular, it can present with Horner’s syndrome or respiratory symptoms. Rarely it presents with opsoclonus-myoclonus syndrome or a VIP-induced secretory diarrhea.
• Urine and plasma dopamine, vanillylmandelic acid, and homovanillic acid are often elevated.
• Biopsy and CT/MRI should be performed. Treatment by surgical resection, radiation, and/or chemotherapy lends a generally favorable prognosis in cases restricted to the neck.

Metastasis

• From a primary tumor may result in a palpable enlarged lymph node. Up to 9% of head and neck cancer is metastatic in origin. Head and neck squamous cell cancer is most common.
• Lung, esophagus, stomach, ovary, and pancreas tumors are other common primary sites. A thorough search for the primary tumor must be undertaken, with lymph node biopsy results used to guide the search strategy.

Algorithm and Summary Figures

Below is a summary of diagnostic approaches commonly used in outpatient pediatric settings in the management of a pediatric neck mass.

Meier JD, Grimmer JF. Evaluation and management of neck masses in children. Am Fam Physician. 2014 Mar 1;89(5):353–8

Meier JD, Grimmer JF. Evaluation and management of neck masses in children. Am Fam Physician. 2014 Mar 1;89(5):353–8

References

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