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Pediatric Neck Masses

Michael R. Goins, MDa,*, Michael S. Beasley, MDb

KEYWORDS
 Pediatric  Neck masses  Cervical adenitis  Neoplasms

KEY POINTS
 Children with neck masses require a thorough clinical examination and follow-up to determine the
biological nature and course of the abnormality. Malignancy must always be considered, as it occurs
in 11% to 15% of all pediatric cervical masses.
 Inflammatory disease represents the most common cause of neck masses or lumps in children, with
cervical adenitis being the most common entity of the inflammatory processes.
 The second branchial arch represents about 90% of all branchial cleft/arch-related cysts and
sinuses.
 Cysts of the thyroglossal sinus tract should be preoperatively studied for remnant thyroid tissue
along its track of descent from the base of tongue. All thyroglossal sinus tract excisions should
include the mid portion of the hyoid using the Sistrunk technique to ensure eradication.

INTRODUCTION etiology. These aspects of the workup and diag-


nosis of the cervical mass in children are pre-
Primary care physicians often see a child with sented in this article, with proper surgical
a neck mass or an enlarging mass of several management outlined for each category of lesion.
weeks’ duration. The possible entities are many; The differential diagnosis of pediatric neck
however, it is important to be able to make an early masses is broad. Although the majority are benign,
distinction based on history and presentation and approximately 1 in 10 of biopsied masses were
to subsequently make an appropriate referral to malignant in a review from the Children’s Hospital
a pediatric specialist. Depending on the suspected of Pennsylvania. In this review, Torsiglieri and
lesion, imaging modalities are obtained to better colleagues1 examined 445 pediatric neck masses
define the nature and extent of the mass before and classified them into congenital, inflammatory,
definitive diagnosis and treatment. noninflammatory benign lesions, benign neo-
Most neck masses are congenital or inflamma- plasms, and malignant neoplasms.
tory in origin, although 5% of all pediatric neo- Not surprisingly, congenital lesions were the
plasms occur in the head and neck.1 The initial most common mass based on Torsiglieri’s review,
investigation must include a very precise history with inflammatory lesions the second most
as to time of appearance, duration and changes common. However, many inflammatory lesions
of the mass, prior occurrence or multiple lesions, resolve with conservative therapy and are never
associated pain or dysphagia, leakage of fluid removed or biopsied, and are likely the most
from the mass, associated injury, illness or sys- common pediatric neck mass seen in clinical prac-
temic condition, and exposure to animals and tice. This review1 also likely overestimates the
travel. A careful examination of the head and
oralmaxsurgery.theclinics.com

frequency of malignant masses, as the Children’s


neck will help to further narrow the possible Hospital of Pennsylvania is a tertiary care facility

a
Ear, Nose and Throat Associates of Charleston, 500 Donnally Street, Suite 200, Charleston, WV 25301, USA;
b
Private Practice of Otolaryngology, Eye and Ear Clinic Physicians, 1306 Kanawha Boulevard East, Charleston,
WV 25301, USA
* Corresponding author.
E-mail address: mgoins8@yahoo.com

Oral Maxillofacial Surg Clin N Am 24 (2012) 457–468


doi:10.1016/j.coms.2012.05.006
1042-3699/12/$ – see front matter Ó 2012 Published by Elsevier Inc.
458 Goins & Beasley

Fig. 1. Categories of neck masses presenting in children. (Data from Torsiglieri AJ Jr, Tom LW, Ross AJ 3rd, et al.
Pediatric neck masses: guidelines for evaluation. Int J Pediatr Otorhinolaryngol 1988;16(3):199–210.)

for referred complex pediatric patients. However, and portions of the carotid vasculature. It also
Torsiglieri’s survey provides the most comprehen- forms the inferior parathyroids, thymic duct, and
sive review of pediatric neck masses. thymus. The cranial nerve is the glossopharyngeal
(ninth).
The fourth arch forms the inferior constrictor and
EMBRYOLOGY AND ANATOMY cricothyroid muscles. Its nerve is the superior laryn-
geal, and vascular elements contribute to the right
A review of fetal development of the cervical subclavian and on the left, the aortic arch. The
region helps in understanding the nature and path- superior parathyroids form from the fourth arch.
ogenesis of some cervical masses.2–4 During the The remaining sixth arch gives rise to most of the
latter part of the third week of craniocervical devel- laryngeal intrinsic musculature supplied by the
opment, the buccopharyngeal membrane begins recurrent laryngeal nerve. The arterial portion forms
to break down into 6 paired pharyngeal arches. the ductus arteriosus and parts of the pulmonary
The fifth arch is small and disappears in the fourth artery. The fourth and sixth arches fuse to form
week. The external surfaces are covered by ecto- the laryngeal cartilages.
derm and internally by pharyngeal derived endo- The thyroid gland forms from descending endo-
derm. Each pharyngeal (branchial) arch contains derm at the tuberculum impar at the end of the third
musculoskeletal, vascular, and neural elements, week. It travels to and around the developing hyoid
which contribute to future corresponding entities. bone to take up position in the lower anterior neck.
The first arch contains 2 cartilaginous structures:
a maxillary process and a mandibular projection BACKGROUND
known as Meckel cartilage. Lateral ossification
around Meckel cartilage forms the mandible, sphe- History and physical examination are paramount in
nomandibular, and anterior mallear ligaments. The the evaluation of the pediatric neck mass. The
nerve component is the trigeminal (fifth cranial history and physical examination produce the dif-
nerve), and the arterial elements give rise to some ferential diagnosis of the mass and often allow
portions of the internal maxillary artery. The the differentiation of lesions into congenital, in-
muscles of mastication, tensor palatini and tensor flammatory, benign, or malignant, if a definite diag-
tympani, anterior digastric and mylohyoid muscu- nosis cannot be made.
lature, are all derived from the first arch. Certain caveats of the history and physical ex-
The second branchial arch, also called Reichert amination are crucially important. Location helps
cartilage, primarily forms musculoskeletal elements: to narrow down the differential diagnosis. A midline
muscles of facial expression, posterior digastric, neck mass may represent a thyroglossal duct cyst,
stylohyoid, stapedius, and platysma muscles, and dermoid and epidermoid cysts, cervical clefts, or
portions of the hyoid and the styloid process. The teratomas.3,4 Lateral neck masses are more likely
facial (seventh cranial nerve) nerve is derived from to be branchial cleft anomalies, lymphatic or
the second arch. Very little remains of the arterial vascular malformations, and thyroid nodules.
component except for the stapedial artery. Congenital anomalies are more likely to present
The third arch gives rise to the remainder of the early, whereas malignancies are more frequently
hyoid, the stylopharyngeus, upper constrictors, encountered in older children.5
Pediatric Neck Masses 459

Fig. 2. Categories of congenital neck masses in children. (Data from Torsiglieri AJ Jr, Tom LW, Ross AJ 3rd, et al. Pediatric
neck masses: guidelines for evaluation. Int J Pediatr Otorhinolaryngol 1988;16(3):199–210.)

Neck masses in children can be generally cate- CONGENITAL LESIONS


gorized by their location in the cervical anatomy.6
Congenital masses are the most common nonin-
Anterior Triangle flammatory neck mass in children. Each type has
Lymphadenopathy a typical location and presentation in the neck.
Infectious/Inflammatory: lymphadenitis, Children with congenital malformation of the
mycobacterial neck require comprehensive pediatric evaluation
Neoplastic: lymphoma, secondary spread to ensure it is not a manifestation of a systemic
Thyroglossal duct cysts syndrome, which could modify the treatment plan.
Branchial cleft cysts
Dermoids and lipomas Branchial Cleft Anomalies
Thyroid masses (adolescent females), goiter. During embryologic development, the tissues of
Posterior Triangle the neck are derived from branchial arches that
Lymphadenopathy are separated externally by grooves and internally
Vascular malformations (lymphatic) by pharyngeal pouches. Incomplete or aberrant
Fibromatosis colli (pseudotumor of infancy) fusion of 2 adjacent arches can result in the forma-
Glandular (parotid). tion of branchial cleft anomalies that include cysts,
internal sinuses, external sinuses, and fistulas.2–4
Once the likely cause is determined by history Anomalies can arise from each embryologic
and physical examination, further testing can be groove and have characteristic locations and
used to confirm the clinical suspicion. Laboratory anatomic boundaries based on the structures
testing, fine-needle aspiration (FNA) biopsy, and derived from the adjacent embryologic arches.
imaging of pediatric neck masses are often carried Cysts arising from the first branchial arch
out to further classify the lesion. comprise about 8% of cervical sinus tracts and

Fig. 3. A type I first branchial arch sinus and cyst presenting in the preauricular region. (A) Type I fistula (arrow)
and cystic swelling preoperatively. (B) Sinus tract and cyst being delivered from its attachment to the external
auditory canal cartilage.
460 Goins & Beasley

cysts.6 These cysts often present with recurrent


swelling and drainage with secondary infection.
Type I first-arch cysts typically open in the preaur-
icular or postauricular region (Figs. 1–3). The sinus
tract usually courses parallel to the external audi-
tory canal to the middle ear or deep portion of
the cartilaginous canal (Fig. 4).7 Surgical excision
is undertaken when no infection is present.
Type II cysts of the first arch are classically
located in the anterior neck, superior to the hyoid.
Sinuses course anteriorly to the hyoid, often
through and around the parotid and facial nerve,
respectively (Fig. 5).4–7 Great care must be exer-
cised in the surgical exposure of the area through
superficial parotidectomy to avoid damage to the
facial nerve.
Cysts of the second branchial arch are the most Fig. 5. Pathway of type II first branchial cleft anoma-
common branchial entity, accounting for about lies. (From Drake A, Hulka G. Congenital neck masses.
In: Shockley W, Pillsbury H, editors. Neck: diagnosis
90% of the cervical cysts.3,4,6 If a skin opening is
and surgery: Mosby; 2004. p. 98; with permission.)
present, it is found along the anterior border of
the sternocleidomastoid muscle (Fig. 6). As the
cyst passes into the deep neck, it travels deep
and posterior to the submandibular gland and structures and thyroid gland (Fig. 9). Imaging is
between the internal and external carotid arteries important, as is endoscopic examination of the
to terminate in the tonsillar fossa (Fig. 7).7 Imaging pyriform fossa, to identify the internal sinus
to delineate its course and endoscopy to visualize opening. The unusual presentation of suppurative
the pharynx for sinus openings are requisites thyroiditis in children may be due to secondarily in-
before surgery (Fig. 8). fected third- or fourth-arch sinuses.
Cysts of the third and fourth branchial arches
are uncommon (<2%) and represent sinus tracts,
Thyroglossal Duct Cyst
which course deep into the anterior cervical
Thyroglossal duct cysts are the second most
common congenital pediatric neck mass and
form as a result of the thyroid’s embryologic
“journey” to the anterior neck.7,8 The thyroid gland
begins to develop in the third week in utero at the
foramen cecum and then descends into the ante-
rior neck to overlie the larynx. Occasionally,
a remnant of embryologic ductal tissue persists
following this process and can later develop into
an epithelial lined tract (Fig. 10). In fact, the pres-
ence of the pyramidal lobe of the thyroid repre-
sents failure of closure of the duct at its most
inferior location.8
Thyroglossal duct cysts primarily present in the
midline, and can occur anywhere between the
base of the tongue and the thyroid gland
(Fig. 11). The cysts may lie dormant in a similar
fashion to branchial cleft anomalies until they
become swollen and painful, which can occur after
an upper respiratory infection. Clinical diagnosis,
based on a thorough history and physical ex-
Fig. 4. Pathway of type I first branchial cleft anomalies.
EAM, external auditory meatus; SCM, sternocleidomas- amination, is usually accurate. Thyroglossal duct
toid muscle. (From Drake A, Hulka G. Congenital neck cysts are most easily seen with the neck in an
masses. In: Shockley WW, Pillsbury HC, editors. Neck: extended position, and they will elevate with swal-
diagnosis and surgery: Mosby; 2004. p. 97; with lowing and tongue protrusion because of their fixa-
permission.) tion to the hyoid bone.
Pediatric Neck Masses 461

Fig. 6. (A, B) Second branchial arch cysts in 2 patients, both presenting anterior to the sternocleidomastoid
muscle (arrow).

During the preoperative evaluation, additional and investing scarred infrahyoid musculature may
imaging and thyroid uptake studies may be indi- be necessary to achieve eradication.
cated. The best modality of imaging in children is
ultrasonography, which can adequately document Dermoid Cysts and Teratomas
the presence and configuration of the thyroid
gland or any derivatives in the descent route. It is The term dermoid cyst is often used in a generic
important to confirm the presence of normal thy- sense to describe 3 separate entities that include
roid tissue outside of the thyroglossal duct cyst epidermoid cysts, true dermoid cysts, and tera-
before excision, as well as identification and tomas.10 These pathologic entities are distin-
distinction from lingual thyroid tissue. guished by their derivation. Epidermoid cysts
Complete excision of a thyroglossal duct cyst is arise from ectodermal tissue alone and are nor-
performed by the Sistrunk procedure, first des- mally found superficially in the subcutaneous
cribed in 1920.9 This procedure requires removal tissues. Histologically they have a squamous epi-
of the cyst and any associated tract as well as thelial lining and often contain keratinaceous
the middle portion of the hyoid bone. Failure to re- debris (Fig. 12). True dermoid cysts are composed
move the middle portion of the hyoid bone will of both ectodermal and mesodermal components
often lead to recurrence and infection. Patients at including hair follicles, sebaceous glands, and/or
greater risk of recurrence are those with infected, sweat glands.
draining sinuses. Excision of sinus, anterior hyoid, Both epidermoid and dermoid cysts may be
congenital or acquired. Congenital lesions are
thought to arise from the sequestration of ecto-
derm or endoderm within the deeper tissues along
embryologic “folds.”11 In this respect they are
similar to branchial cleft cysts, although they typi-
cally do not have sinus tracts nor do they arise
from deep structures in the neck. Acquired cysts
are commonly termed inclusion cysts because
they are believed to result from traumatic implan-
tation of a portion of the skin into the underlying
layers,10,11 which can result in the ectopic forma-
tion of a dermal cyst lined with squamous epithe-
lium in any associated location. Depending on
the size and depth of these lesions, preoperative
imaging with ultrasonography, computed tomog-
raphy (CT) or magnetic resonance imaging (MRI)
may be indicated for diagnosis and surgical plan-
ning (Fig. 13).12 The majority are amenable to
Fig. 7. Pathway of second branchial cleft anomalies. direct surgical excision, and every effort should
(From Drake A, Hulka G. Congenital neck masses. In: be made to remove the cysts without rupture of
Shockley W, Pillsbury H, editors. Neck: diagnosis and the cyst and spillage of contents. Epidermoid exci-
surgery: Mosby; 2004; with permission.) sion should include the overlying skin, whereas
462 Goins & Beasley

Fig. 8. Sagittal (A) and axial (B) computed tomography (CT) images of a branchial cleft cyst of the left neck.

dermoids are often attached to the underlying more rapidly than other dermoid cysts.13,14 In
periosteum and require removal of the involved addition, their location is less predictable, and
segment of periosteal attachment.10 proximity to vital structures can make surgical
Teratomas of the head and neck are rare tumors excision difficult. Preoperative imaging with CT
that are composed of all 3 embryologic layers: or MRI is normally indicated for diagnosis and
ectoderm, mesoderm, and endoderm.13 These surgical planning. Teratomas can present as an
tumors may include any type or combination of epignathus that may impinge on the airway. In
tissues normally found within the human body. the developed world, these are normally diag-
Although they are benign, they tend to grow nosed prenatally with ultrasonography. If peripar-
tum respiratory compromise is feared, the
obstetric plan should include options for airway
control including intubation or tracheotomy. An
Ex Utero Intrapartum Therapy (EXIT) procedure
may be indicated depending on the size and loca-
tion of the mass, and should be considered and
coordinated in advance of delivery.15

Lymphatic Malformations
Lymphatic malformations, previously termed lym-
phangiomas or cystic hygromas, are presently
classified as vascular malformations, and occur
in 1 out of every 2000 to 4000 births.16 These mal-
formations are more commonly found as isolated
lymphatic lesions that tend to be of low flow in
character, but can be seen in combination with
arterial or venous vasculature as mixed lympho-
vascular lesions.17 Lymphatic malformations are
thought to arise as a benign growth of the
lymphatic system; however, they can have signifi-
cant local impact because of their growth potential
and infiltrative nature.
Embryologically the lymphatic system develops
from vascular endothelial cells.18 The system is
composed of thin-walled channels with limited
Fig. 9. Pathway of third branchial cleft anomalies. basement membrane structure, allowing for in-
(From Drake A, Hulka G. Congenital neck masses. In: creased permeability. The lymphatic system acts
Shockley W, Pillsbury H, editors. Neck: diagnosis and to drain extravascular fluid, direct antigens to
surgery: Mosby; 2004; with permission.) regional lymph nodes, and absorb ingested fat.
Pediatric Neck Masses 463

disturbances, including airway compromise and


long-term distortion of facial growth. Although
smaller lesions have been reported to regress
without intervention, all of such lesions have the
potential for rapid expansion secondary to hemor-
rhage, trauma, or infection.
Clinical diagnosis is confirmed with CT and/or
MRI for complete characterization (Fig. 15). This
imaging includes identification of microcystic and
macrocystic components as well as delineation
of anatomic location and extent of disease in rela-
tion to the surrounding vital structures.19 The
mainstay of treatment is surgical resection or de-
bulking. Timing is debatable, and depends on the
extent and impact of the lesion. Complete excision
may be difficult without sacrifice of vital structures,
and often multiple debulking procedures are per-
formed. Multiple protocols regarding timing of
surgical intervention and potential reconstruction
are reported in the literature. Nevertheless,
patients must be monitored for recurrence and
long-term growth disturbances.
Sclerotherapy has been increasingly studied
and used as a viable option to surgical resection,
particularly in macrocystic lesions. Multiple proto-
cols and sclerosant agents are currently being
evaluated, including OK-432 (Picinabil), bleomy-
Fig. 10. Pathway of the thyroglossal duct with depiction cin, alcohol, and hypertonic saline.20 Consider-
of the typical cyst anterior to the hyoid. (From Drake A, ation must be given to the technique and control
Hulka G. Congenital neck masses. In: Shockley W, of infiltration as well as potential postoperative
Pillsbury H, editors. Neck: diagnosis and surgery: Mosby;
edema and pain.
2004; with permission.)
For more in-depth discussion of lymphatic mal-
formations, the reader is referred to the article on
Lymphatic malformations are most commonly vascular malformations by Abramowicz and Padwa
seen in the neck, but can be found anywhere in elsewhere in this issue.
the soft tissues of the face and oral cavity; they
are classically noted as a soft and compressible INFLAMMATORY LESIONS
cystic mass that can be transilluminated. Lym-
phatic malformations can range in size and se- Inflammation and enlargement of the cervical
verity from small isolated lesions to extensive lymph nodes represent the most common cause
cervicofacial lesions (Fig. 14). The malformations of neck masses in children. The review by Torsiglieri
may cause both cosmetic and functional and colleagues1 demonstrated a high percentage

Fig. 11. Thyroglossal duct sinus and cyst. (A) Anterior neck of an infant with draining sinus tract (arrow). (B) Axial
CT with contrast showing cystic cavity in fistula (arrow). (C) Fistulogram of thyroglossal sinus tract extending from
tongue base to thyroid.
464 Goins & Beasley

and (2) an FNA cannot appreciate histologic


architecture.

NONINFLAMMATORY BENIGN LESIONS


The review by Torsiglieri and colleagues1 grouped
inclusion cysts, fibromatosis colli, and keloids into
the category of noninflammatory benign lesions,
which comprises about 5% of the cervical masses
encountered in children. Inclusion cysts, which
may develop from traumatically or iatrogenically
induced means, and keloids are not be discussed
Fig. 12. Cut specimen of a dermoid cyst. here, as they make up only a very small compo-
nent of the noninflammatory benign masses seen
in children.
(27%) of neck masses as inflammatory in etiology Fibromatosis colli, or pseudotumor of infancy,
(Fig. 16). Roughly half of all young children have presents as torticollis with contracture of the ster-
palpable lymph nodes. The most common site nocleidomastoid muscle (SCM).21,22 The neck is
for cervical adenitis is the submandibular and flexed and foreshortened on the affected side
superior cervical nodes, although postauricular with the child’s head tilted ipsilaterally, with the
and occipital adenitis may result from chronic otitis chin deviated away. This firm mass typically
and scalp infections (dermatitis, tinea, impetigo, follows a breech delivery with forceps, or a last
and so forth). In general, a node larger than 2 trimester with little movement and the head
cm2 of longer than 2 months’ duration should be engaged downward and tilted. Muscular damage
sampled unless an infectious source can be docu- to the SCM results in hematoma and fibrosis.
mented. Vigilance through recall, examination, and Pseudotumor usually becomes evident soon after
systemic review is recommended. birth as the traumatized muscle is replaced with
History and physical examination are paramount exuberant fibrosis tissue, which can be confirmed
in deciding whether to pursue further workup of on ultrasonography (Fig. 17) or FNA or open
a pediatric neck mass. One indication for FNA or biopsy.21 On physical examination a firm, well-
open biopsy can be a mass that does not resolve circumscribed, immobile soft-tissue mass may
after seemingly appropriate treatment. Others be felt in the belly of the sternomastoid muscle.
can be an enlarging mass, a suspicious clinical Treatment of torticollis is primarily with aggressive
course, unusual imaging findings, or systemic physical therapy. In rare cases, the SCM may be
symptoms. FNA can allow for Gram stain, culture, surgically lengthened if torticollis persists beyond
acid fast stain, immunocytochemistry, and cyto- the first year of life. Physical therapy must resume
genetics. All FNAs have 2 inherent weaknesses: immediately after the operation. Failure to treat
(1) the needle may miss the lesion of interest, can result in disfigurement of the craniofacial

Fig. 13. Axial (A) and coronal (B) images of a large dermoid cyst.
Pediatric Neck Masses 465

Fig. 14. An infant with a large cervical-facial lymphatic malformation of the left anterior neck. (A) Lateral view
showing extent of lesion from a cephalad-caudal perspective. (B) Inferior view of large lymphatic malformation
with mild external airway impingement.

skeleton through skewing of the cranial base, re-  Two or more neurofibromas or one plexi-
sulting in deformational plagiocephaly (Fig. 18). form (mass grouping) tumor
 Freckling of the groin or axilla
 Café-au-lait spots
BENIGN NEOPLASMS  Skeletal deformity or scoliosis
 Lisch nodules (iritic hamartomas)
Benign neoplasms are relatively uncommon pedi-  Optic gliomas
atric neck lesions, accounting for only 3% of neck  Macrocephaly  hydrocephalus
masses. Neurofibromas may present as either  History of seizure
a solitary lesion or part of a generalized syndrome  Juvenile lenticular opacity.
of neurofibromatosis (NFM). Neurofibromas, prin-
cipally Schwann cells and melanocytes, are When presumptive diagnosis of NFM is made,
believed to arise from the neural crest. imaging (CT or MRI) may elucidate craniocervical
Type I NFM or Recklinghausen disease is the most involvement, particularly any tumor involvement
common form (90%) with an incidence of 1:4000 of the airway (Fig. 19).24 A central low T2 signal
births.23 It is an autosomal dominant disorder with on MRI is a feature of NFM tumors.25 Type II
variable expressivity. Type I NFM is diagnosed NFM is less common, and generally presents as
when any 2 of 9 inclusion criteria are met: a central nervous system disorder involving the

Fig. 15. (A) Axial CT image of a large lymphatic malformation. (B) Coronal magnetic resonance image of a large
lymphatic malformation.
466 Goins & Beasley

Fig. 16. Inflammatory neck masses of the neck in children. (Data from Torsiglieri AJ Jr, Tom LW, Ross AJ 3rd, et al.
Pediatric neck masses: guidelines for evaluation. Int J Pediatr Otorhinolaryngol 1988;16(3):199–210.)

vestibular nerve (eighth cranial nerve) with subse- postoperatively, most likely with tracheostomy.
quent hearing loss. Imbalance, headaches, facial Genetic counseling is encouraged.
palsies, and other craniospinal tumors accompany Lipomas, though more common in the adult po-
type II NFM. pulation, also occur in children. Lipomas are the
Surveillance and selective tumor excision or de- most common benign mesenchymal tumors.26,27
bulking is the mainstay of management, as cure is In children, lipomas typically arise before 8 years
not possible. A child with known NFM of the cer- of age and have a rapid growth history. Lipomas
vical region who begins to develop signs of airway are soft, compressible lesions, which may distort
obstruction (progressive snoring, stridor, and the neck and even compress the airway, although
hoarseness) should undergo airway evaluation this is rare. Lipomas are one of the few lesions
with endoscopy. Airway impingement from mul- with anterior and posterior location in the neck, par-
tiple or large tumors may necessitate prophylactic ticularly with infiltrating masses. Multiple lipomas
removal, if possible, with insurance of the airway should alert the clinician to possible syndromic
involvement with imaging, and FNA or open biopsy
will confirm the diagnosis so that plans can be
made for excision of the tumor.

MALIGNANT NEOPLASMS
In the child who first presents with a neck lump and
who is sick, that is, febrile and lethargic, it is rea-
sonable to initiate a course of antibiotics to treat
a presumed infectious source, which is most
common. However, if after 10 to 14 days of no im-
provement or worsening systemic signs and
symptoms, a more serious condition should be en-
tertained. Any child with a progressively enlarging
neck mass and signs of systemic disease (fatigue,
weight loss, fever/chills, night sweats, and so
forth) should be highly suspect for malignancy.
Malignancy is a diagnosis that should be con-
sidered in the workup of all pediatric neck masses,
as they represent 11% to 15% of all neck masses
in children.1,28,29 Lymphoma is the third most com-
mon pediatric cancer and is the most common
Fig. 17. Ultrasonogram of neck and sternocleidomas- malignancy presenting as a mass in the neck of
toid muscle showing pseudotumor (arrowheads) in a child.29,30 In Torsiglieri’s review,1 35% of patients
the muscle belly. (Courtesy of http://pediatricimag- with head and neck lymphoma presented with
ing.wikispaces.com/; with permission.) a supraclavicular mass. This finding confirms the
Pediatric Neck Masses 467

Fig. 18. Fibromatosis colli (pseudotumor) in an infant resulting in “twisted” face. (A) Infant with right facial full-
ness (and consequent flatness on the left side) and mandible deviated to the right, secondary to foreshortening
of the sternocleidomastoid muscle. (B) Three-dimensional CT image demonstrating persistent deformational pla-
giocephaly caused by fibromatosis colli and torticollis.

need to have a high index of suspicion in the child infection, surgical management does not play
who presents with a long-standing neck mass (>6 a role in the treatment of lymphoma in children.
to 8 weeks) and has accompanying systemic signs
of illness. SUMMARY
Because of accompanying involvement of the
chest and abdomen, children may complain of The differential diagnosis of the pediatric neck
shortness of breath, chest and abdominal discom- mass includes a wide array of congenital, inflam-
fort, and gastrointestinal disorders. The workup con- matory, benign, and malignant lesions in many lo-
sists of CT imaging, FNA or open biopsy for cellular cations of the neck. The initial history and physical
type, blood panel, marrow biopsy, and surgical examination is of utmost importance, and should
staging. Treatment is based on cellular type and cor- be used to place the mass into one of these cate-
responding clinical staging paradigms. Except for gories if a definitive diagnosis is not possible. The
bony involvement and pathologic fracture or most common cervical masses are inflammatory
in nature and represent a benign course, with little
treatment other than watchful waiting. A mass that
does not respond to seemingly appropriate treat-
ment, and is accompanied by systemic symptoms
or unusual imaging findings, should prompt the
consideration of fine-needle aspiration and/or
surgical biopsy.

REFERENCES
1. Torsiglieri AJ Jr, Tom LW, Ross AJ 3rd, et al. Pediatric
neck masses: guidelines for evaluation. Int J Pediatr
Otorhinolaryngol 1988;16(3):199–210.
2. Carstens MH. Neural tube programming and cranio-
facial cleft formation. Eur J Paediatr Neurol 2004;
8(4):181–210.
3. Nicollas R, Guelfucci B, Roman S, et al. Congenital
cysts and fistulas of the neck. Int J Pediatr Otorhino-
laryngol 2000;55(2):117–24.
4. Schroeder JW Jr, Mohyuddin N, Maddalozzo J.
Fig. 19. Coronal CT image of a neurofibroma in the Branchial anomalies in the pediatric population. Oto-
left neck. laryngol Head Neck Surg 2007;137(2):289–95.
468 Goins & Beasley

5. Roh JL. Lymphomas of the head and neck in the 20. Smith ML, Zimmerman B, Burke DK, et al. Effi-
pediatric population. Int J Pediatr Otorhinolaryngol cacy and safety of OK-432 immunotherapy of
2007;71(9):1471–7. lymphatic malformations. Laryngoscope 2009;119:
6. Gross E, Sichel JY. Congenital neck lesions. Surg 107–15.
Clin North Am 2006;86(2):383–92. 21. Lowry KC, Estroff JA, Rahbar R. The presentation
7. Moir CR. Neck cysts, sinuses, thyroglossal duct and management of fibromatosis colli. Ear Nose
cysts, and branchial cleft anomalies. Oper Tech Throat J 2010;89(9):E4–8.
Gen Surg 2004;6(4):281–95. 22. Turkington JR, Paterson A, Sweeney LE, et al. Neck
8. Rovet JF. Congenital hypothyroidism: an analysis of masses in children. Br J Radiol 2005;78:75–85.
persisting deficits and associated factors. Child 23. Torpy JM, Burke AE, Glass RM. Neurofibromatosis
Neuropsychol 2002;8(3):150–62. Type I peripheral nerve tumors. JAMA 2008;300(3):
9. Sistrunk WE. The surgical treatment of cysts of the 352–7.
thyroglossal tract. Ann Surg 1920;71(2):121–2. 24. Rahbar R, Litrovnik BG, Vargas SO, et al. The
10. Pryor SG, Lewis JE, Weaver AL, et al. Pediatric der- biology and management of laryngeal neurofibro-
moid cysts of the head and neck. Otolaryngol Head mas. Arch Otolaryngol Head Neck Surg 2004;130:
Neck Surg 2005;132(6):938–42. 1400–6.
11. Thomson HG. Common benign pediatric cutaneous 25. Steen RG, Taylor RS, Langston JW, et al. Prospec-
tumors: timing and treatment. Clin Plast Surg 1990; tive evaluation of the brain in asymptomatic children
17(1):49–64. with NFM type I. Am J Neuroradiol 2001;22(5):
12. Ahuja R, Azar NF. Orbital dermoids in children. 810–7.
Semin Ophthalmol 2006;21(3):207–11. 26. de Jong AL, Park A, Taylor G, et al. Lipomas of the
13. Wakhlu A, Wakhlu AK. Head and neck teratomas in head and neck in children. Int J Ped Otolaryngol
children. Pediatr Surg Int 2000;16(5–6):333–7. 1998;43(1):53–60.
14. Bailey BJ. Congenital neck masses and cysts. In: 27. Lerosey Y, Choussy O, Gruyer X, et al. Infiltrating
Bailey BJ, Johnson JT, Newlands SD, editors. lipoma of the head and neck. Int J Ped Otolaryngol
Head and neck surgery—otolaryngology. 3rd 1999;47(1):91–5.
edition. Baltimore (MD): Lippincott Williams and 28. Neck masses. Publication of the American Academy
Wilkins; 2001. p. 933–9. of Otolaryngology—Head and Neck Surgery, Inc.
15. Rahbar R, Vogel A, Myers LB, et al. Fetal surgery in One Prince Street. Alexandria (VA). 22314–23357.
otolaryngology. Arch Otolaryngol Head Neck Surg Available at: www.entnet.org/EducationAndResearch/
2005;131:393–8. Journal.cfm. Accessed April 27, 2012.
16. Perkins JA, Manning SC, Tempero RM, et al. 29. Smith MA, Seibel NL, Altekruse SF, et al. Outcomes
Lymphatic malformations: review of current treatment. for children and adolescents with cancer: chal-
Otolaryngol Head Neck Surg 2010;142(6):795–803. lenges for the twenty-first century. J Clin Oncol
17. Kennedy TL, Whitaker M, Pellitteri P, et al. Cystic hy- 2010;28(15):2625–34.
groma/lymphangioma: a rational approach to 30. Percy CL, Smith MA, Linet M, et al. Lymphomas and
management. Laryngoscope 2001;111:1929–37. reticuloendothelial neoplasms. In: Ries LA, Smith MA,
18. Perkins JA, Manning SC, Tempero RM, et al. Gurney JG, editors. Cancer incidence and survival
Lymphatic malformations: current cellular and clin- among children and adolescents: United States
ical investigations. Otolaryngol Head Neck Surg SEER program 1975-1995. Bethesda (MD): National
2010;142(6):789–94. Cancer Institute, SEER Program; 1999. p. 35–50. NIH
19. MacArthur CJ. Head and neck hemangiomas of Pub.No. 99-4649. Available at: http://seer.cancer.gov/
infancy. Curr Opin Otolaryngol Head Neck Surg publications/childhood/lymphomas.pdf. Accessed
2006;14(6):397–405. April 29, 2012.

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