Fibromatosis colli presents in the neonatal period as a mass
in the SCM muscle. Histologically, part of the involved muscle is replaced
by dense fibrous tissue.19 Parents will often notice
the mass in the first weeks of life and may notice limited range
of motion of the neonate’s neck (torticollis). There may
be a history of birth trauma. Physical examination reveals a firm,
solid, immobile mass located within the SCM muscle itself, often
associated with some degree of torticollis. The mass moves with
the muscle when the head is turned on examination. The diagnosis
can often be made clinically or confirmed by US. The natural history
is spontaneous resolution over a period of 4 to 8 months, and gentle
stretching or physical therapy is the only treatment needed.12,19
Hemangiomas are congenital vascular malformations. Infantile
hemangiomas usually grow rapidly until 9 to 10 months
of age, after which spontaneous regression is the norm, although
involution may take up to 10 years. Hemangiomas of the head and
neck may compromise the airway as they enlarge, presenting as respiratory
distress often associated with biphasic stridor unrelated to upper
respiratory infection and unresponsive to racemic epinephrine. When
palpable, they are soft, mobile, and frequently have a bluish hue.20 Suspected
airway lesions may require endoscopy by a pediatric otolaryngologist.
Visible lesions are best characterized with CT or MRI. 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 steroids and laser or surgical excision.
Lipomas are benign tumors of fat that are very rare in children.
These masses are solid, homogenous, rubbery, and most commonly found
in the posterior triangle of the neck.21 They often
displace or compress rather than infiltrate surrounding structures,
which is more characteristic of the malignant character of lipoblastoma.
US and CT demonstrates the presence of fat. Surgical excision is
the treatment of choice.10
Neurofibromas and schwannomas are rare usually large tumors often
involving the orbits, the skull base, or the parotid region, and
are almost always associated with neurofibromatosis type I, which
includes multiple café au lait spots (Figure
118-8), plexiform neuromas, and schwannomas. The best modality
for diagnosis and characterization of the lesion is MRI.
Café au lait spots in neurofibromatosis. (Reproduced with
permission from Shah BR, Lucchesi M: Atlas of Pediatric
Emergency Medicine. © 2006, McGraw-Hill, New York.
Children with diffuse enlargement of the thyroid gland usually have
symptomatic thyrotoxicosis or Hashimoto thyroditis,21 although goiter
can be seen in some parts of the world (Figure
118-9). Most cystic masses in the region of the thyroid gland
are thyroglossal cysts. Thyroid masses are evaluated with thyroid
function studies, sonography, nuclear scintigraphy, and biopsy.22
Goiter resulting from Graves disease in a 16-year-old girl.
(Reproduced with permission from Shah BR, Lucchesi M: Atlas
of Pediatric Emergency Medicine. © 2006, McGraw-Hill,
New York. Figure 14-3.)
Most salivary gland inflammation is secondary to calculi that
occur most commonly in the submandibular gland in adolescents; calculi
can lead to duct obstruction (Figure 118-4C)
and swelling in the associated gland. Suppurative infection is typically
caused by S. aureus. Diagnosis is often clinical,
although US and sialogram are helpful in unclear cases. Treatment
consists of antibiotics and local measures such as mouth rinses,
heat, and lozenges. Although uncommon, neoplasms of the salivary
glands do occur, and almost 80% arise from the parotid
gland. Fortunately, 80% of parotid tumors are benign.23 CT, MRI,
and fine needle aspiration biopsy may be needed to diagnose solid masses
of the parotid gland.
A few rare malignancies in children may present to the ED because
of a neck mass. The first step in evaluation is a careful history
and physical examination to identify the location of the mass (midline,
anterior, or posterior triangle).24,25 The anterior
triangle extends from the undersurface of the mandible to the junction
of the SCM muscle and the sternoclavicular joint. The posterior
triangle extends posterior to the SCM muscle from the clavicle to
the base of the mastoid, with the trapezius muscle defining the
A solitary posterior cervical mass suggests malignancy.26 In
a study of 4768 patients with nasopharyngeal carcinoma, asymptomatic
posterior triangle neck mass was the most common presenting symptom,
occurring in 76% of patients.27 Supraclavicular
masses suggest lymphomas4 or malignantmetastasis
from areas below the clavicle (lungs, GI, or gynecologic).28 By
contrast, central midline swelling suggests the thyroid with ducts,
cysts, or dermoids as likely diagnoses.
The most common childhood malignancies presenting as neck masses are
briefly reviewed below.
Lymphoma is the most common malignancy of the head and neck in
children (see Chapter 136, Oncology and Hematology Emergencies in Children).10 Almost 50% of head
and neck lymphomas are Hodgkin disease, which usually presents in teenagers (Figure 118-9.1). Neck mass is the primary
presenting sign in 80% of children with Hodgkin disease
and 40% of children with non-Hodgkin lymphoma. Non-Hodgkin
lymphoma primarily affects children between 2 and 12 years of age,
and extranodal involvement is common, occurring more commonly in
patients with acquired or congenital immune disorders. Burkitt lymphoma is
a non-Hodgkin lymphoma and is considered the fastest growing human
tumor. Most lymphomas present as large, firm, mobile masses, commonly
located in the anterior triangle or the supraclavicular area. CT
and MRI are used for diagnosis.29
Hodgkin lymphoma in a 13-year-old boy who presented with
a painless, rubbery, firm mass that had appeared 4 months earlier.
Lymph node biopsy confirmed the diagnosis of Hodgkin lymphoma. (Reproduced
with permission from Shah BR, Lucchesi M: Atlas of Pediatric
Emergency Medicine. © 2006, McGraw-Hill, New York,
Rhabdomyosarcoma is the second most frequent malignancy of the
head and neck in children.10,29 This disease has
a peak presentation at 2 to 5 years old and again at 15 to 19 years
old, with a slight male predominance. Tumors can involve the orbit,
nasopharynx, middle ear, nasal cavity, and paranasal sinuses.30 Neck
tumors may present with brachial plexus palsy. Contrast-enhanced
CT and MRI are the best imaging modalities for diagnosis and staging.24 For
further discussion, see Chapter 136, Oncology and Hematology Emergencies in Children.
Neuroblastoma is a malignancy of the sympathetic chain and can
originate anywhere along its path, although the adrenal glands are
the most common primary site.12,31 Most present
before the age of 5 years old, and those arising in the neck (5%)
have a better prognosis than those tumors of adrenal origin.10 Neuroblastoma
can rarely present as an asymptomatic mass, alone or with local
compressive signs such as hoarseness, dysphagia, airway obstruction,
Horner syndrome, or cranial nerve palsies. Diagnostic imaging includes
US, which typically shows an echogenic mass; CT and MRI provide
better detail.32 For further discussion, see Chapter 136, Oncology and Hematology Emergencies in Children.
Malignant cervical lymphadenopathy may also represent metastatic
disease. Common tumors that metastasize to the neck include nasopharyngeal
carcinoma and some GI tumors. CT and MRI are best to diagnose and
define extension of the tumor.29 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 mediastinal mass.