congenital neck masses

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Yonatan Avraham Demma
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Patient history
Physical examination
Differential diagnosis
Imaging studies
Blood test
The most important element in the
evaluation of a neck mass is….
Most pediatric neck masses are inflammatory
or congenital and resolve spontaneously or
after appropriate medical therapy.
In contrast, a neck mass in an adult over the
age of 40 should be considered neoplastic
in origin unless proven otherwise,
particularly in the setting of tobacco or
alcohol use.
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duration,
growth pattern,
and absence or presence of pain.
change in voice,
hoarseness,
difficulty with swallowing,
ear pain
generalized complaints: fever, night sweats, and
weight loss.
patient's social history: alcohol and drug use,
smoking, and recent travel.
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systematic investigation of all mucosal and
submucosal areas of the head and neck.
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mobility, consistency, and tenderness of the
mass.
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location of the neck mass is particularly
important
› Children: different branchial cyst
› adult patients: virchov metastasis
Differential diagnosis
bordered by anterior border of
SCM, midline of neck, and
mandible
› muscular triangle
formed by midline, superior
belly of omohyoid, and SCM
› carotid triangle
formed by superior belly of
omohyoid, SCM, and
posterior belly of digastric
› submental triangle
formed by anterior belly of
digastric, hyoid, and midline
› submandibular triangle
formed by mandible,
posterior belly of digastric,
and anterior belly of digastric
bordered by posterior
border of SCM,
trapezius, and
clavicle
› supraclavicular
triangle
formed by inferior belly
of omohyoid, clavicle,
and SCM
› occipital triangle formed by inferior belly
of omohyoid, trapezius,
and SCM
The structures that make up the anterior
neck include
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the larynx,
trachea,
esophagus,
thyroid and parathyroid glands,
carotid sheath,
and suprahyoid and infrahyoid strap
muscles.
Contains:
lymph node,
 the spinal accessory nerve,
 the cervical plexus.
 the brachial plexus
 subclavian vessels.
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Congenital neck mass
Inflammatory Neck mass:
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Infectious
Non infectious
Neoplastic Disorder
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BRANCHIAL CLEFT CYSTS
THYROGLOSSAL DUCT CYSTS
LARYNGOCELES
PLUNGING RANULAS
LYMPHANGIOMAS
HEMANGIOMAS
TERATOMAS - DERMOID CYSTS
THYMIC CYSTS
STERNOCLEIDOMASTOID TUMORS OF INFANCY
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failure of the
pharyngobranchial ducts
to obliterate during fetal
development.
most frequently present in
late childhood or early
adulthood when the cysts
become infected usually
after an upper respiratory
tract infection.
tender, inflammatory mass
located at the anterior
border of the
sternocleidomastoid
muscle.
three categories:
 first, less than 1%
 second, the most common
 and third branchial cleft anomalies.
initial control of the infection
2. surgical excision of the cyst and tract.
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incision and drainage procedures should be
avoided
one-third of all congenital neck masses.
midline masses of the anterior neck .
may be asymptomatic and appear only
when they become infected.
 Thyroglossal duct cysts that occur off the
midline may be difficult to differentiate from
branchial cleft cysts.
 pathognomonic sign on physical
examination is vertical motion of the mass
with swallowing and tongue protrusion,
demonstrating the intimate relation to the
hyoid bone.
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excised with a cuff
of tissue, including
the center portion of
the hyoid bone.
care is taken not to
injure the
hypoglossal nerves;
thyroid carcinomas
can be present in a
small percentage of
thyroglossal duct
cysts
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abnormal dilation or herniation of the saccule of
the larynx.
Laryngopyocele is a Secondary infection of a
laryngocele.
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present with hoarseness, cough, dyspnea,
dysphagia, a foreign body sensation.
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Dx: Laryngoscopy, CT.
If symptomatic, Tx:
› laryngoscopic decompression for small lesions,
› surgical excision through an external approach for larger lesions,
mucoceles of the floor of mouth
 usually present as slow-growing,
painless, submental masses.
 arise from the sublingual gland and are
defined as plunging when they extend
through the mylohyoid muscle into the
neck.
 Tx: excision.
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congenital malformations of the lymphatic
channels.
They arise owing to failure of the lymph spaces to
connect to the remaining lymphatic system.
The mass is usually soft, doughy, smooth,
nontender, and compressible.
can be transilluminated.
CT scanning and MRI are important studies both to
delineate the extent of the disease
Tx: Surgical Debulking because of the infiltrative
nature
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malformations of vascular tissue.
usually present in the first few months of life, grow rapidly
during the first year, and then begin to slowly involute at
18–24 months of age.
In 90% involution occurs without the need for any
therapy.
present as a red or bluish soft mass that is compressible
and increases in size with straining or crying.
Bruits may sometimes be auscultated over the lesion.
Dx: CT scans, MRI.
In the following symptoms: airway compromise, skin
ulceration, dysphagia, thrombocytopenia, cardiac failure
Systemic corticosteroids or surgical laser excision may be
warranted in such cases.
approximately 3.5% of all teratomas.
Their origin is from pluripotential cells, contain elements from all
three germ layers.
Usually present as midline, nontender, mobile neck masses and are
most commonly noted at birth or within the first year of life.
There is a 20% associated incidence of maternal polyhydramnios.
can cause respiratory compromise or dysphagia secondary to
compression.
Dx: CT, MRI.
Tx: Surgical excision.
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The third branchial pouch gives rise to the thymus during
the 6th week of fetal life, elongates in the pharynx, and
then descends into the mediastinum.
Thymic cysts arise when there is implantation of this
thymic tissue along this descent.
present as slow-growing, asymptomatic masses that
may be painful if infected.
On rare occasions, they grow rapidly and cause
dyspnea or dysphagia.
CT scanning and MRI are useful in the differential
diagnosis.
definitive diagnosis is made histologically by the
presence of Hassall corpuscles.
Tx: surgical excision.
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characterized histologically by dense fibrous tissue
and the absence of normal striated muscle.
intimately related to congenital torticollis.
typically present as firm, painless, discrete masses
within the sternocleidomastoid muscle;
slowly increase in size for 2–3 months and then
regress for 4–8 months.
80% resolve spontaneously and do not need any
intervention other than physical therapy to prevent
restrictive torticollis.
Surgical resection is reserved for persistent cases.
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Infectious inflammatory disorder:
 Viral (reactive, HIV)
 Bacterial (suppurative, toxoplasmosis,
tularemia, brucellosis)
 Granulomatous (cat-scratch disease,
actinomycosis, atypical mycobacteria,
tuberculosis, atypical tuberculosis, sarcoidosis)
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Non infectious inflamatory disorder:
 Sinus histiocytosis – Roni-Dorfman disease
 Kawasaki disease
 Castleman disease
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REACTIVE VIRAL LYMPHADENOPATHY
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HIV-ASSOCIATED INFLAMMATORY DISORDERS
the most common cause of cervical
adenopathy in children.
 usually associated with symptoms of an
underlying upper respiratory tract
infection.
 The most common viral agents include
adenovirus, rhinovirus, and enterovirus.
 tend to regress in 1–2 weeks.
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Usually observation
a neck mass larger than 1 cm should be
considered abnormal and warrant
further investigation if it remains for more
than 4–6 weeks or increases in size.
 If persists, biopsies can be taken to
search for other causes.
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EBV can also present with
lymphadenopathy
 usually accompanied by the
enlargement of other lymphoid tissues
such as the adenoids or tonsils.
 symptoms of fever and pharyngitis.
 4–6 weeks.
 Tx: limited to supportive management.
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Cervical Adenopathy or Persistent
Generalized Lymphadenopathy
 present in 12–45% of patients with human
immunodeficiency virus (HIV).
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other infectious or neoplastic etiologies
must be ruled out
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Tx: HIV Tx
1. Suppurative
Lymphadenopathy
2. Toxoplasmosis
3. Tularemia
4. Brucellosis
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most frequently caused by Staphylococcus
aureus and group A B-Streptococcus .
usually develop in the submandibular
region
often accompanied by sore throat, skin
lesions, and upper respiratory tract
infection.
Empirical antibiotic therapy against
anaerobic and gram-positive organisms is
recommended as the first line of
management.
If this fails, either FNA or incision and
drainage may be indicated.
caused by Toxoplasma gondii
 contracted through the consumption of
poorly cooked meat or the ingestion of
oocytes excreted in cat feces.
 present with fever, malaise, sore throat,
and myalgias.
 Dx: serologic testing.
 Tx: ABx ex: sulfonamides (resprim).
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caused by Francisella tularensis
 transmitted by rabbits, ticks, or
contaminated water.
 present with tonsillitis and painful
adenopathy with systemic symptoms of
fever, chills, headache, and fatigue.
 Dx: Serology and Bc.
 Tx: Streptomycin is the antibiotic of
choice.
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Cause by Brucella .
 most commonly transmitted to children
by the ingestion of unpasteurized milk.
 present with total body
lymphadenopathy, fever, fatigue, and
malaise.
 Dx: Serology and BC.
 Tx: Abx : trimethoprim–sulfamethoxazole
or tetracycline
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 cat-scratch disease,
 actinomycosis,
 atypical
mycobacteria,
 tuberculosis,
 atypical tuberculosis,
 sarcoidosis.
Bartonella henselae.
history of contact with cats can be
elicited in 90% of cases.
 more commonly seen in patients
younger than 20 years.
 tender lymphadenopathy, fever, and
malaise.
 lymphadenopathy typically preauricular
and submandibular.
 Dx: serologic testing.
 generally benign and self-limited.
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gram-positive bacillus.
50% to 96% of cases of actinomycosis
affect the head and neck regions.
 painless, fluctuant, neck mass in the
submandibular regions.
 DX: histologically by the presence of
granulomas with sulfur granules.
 TX: Penicillin
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typically presents in the pediatric
population
 unilateral neck mass located in the anterior
triangle of the neck or parotid region.
 brawny skin, induration, and pain.
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Dx: culture and skin testing.
Tx: Surgical excision offers definitive
treatment, although incision and curettage
along with antibiotic therapy constitute an
alternative management strategy.
more commonly in adults than in
children.
 Mycobacterium Tuberculosis
 The presenting lymphadenopathy tends
to be more diffuse and bilateral in
contrast to atypical mycobacteria.
 Tuberculin skin tests are strongly positive.
 Cervical tuberculosis is also known as
scrofula
 Tx: antituberculous medications.
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presents most commonly in the second
decade of life
 lymph node enlargement, fatigue, and
weight loss.
 Chest radiography shows hilar
adenopathy.
 An elevated angiotensin-converting
enzyme (ACE) level is seen in 60–90% of
patients with sarcoidosis.
 Dx: histologically by the presence of
noncaseating granulomas.
 TX: Corticosteroids.
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Immunocompromised patients.
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Most common organisms Candida,
Histoplasma ,and Aspergillus .
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Dx: Serology and fungal cultures.
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Tx: Aggressive, systemic antifungal
therapy with agents such as
amphotericinB
 Sinus
histiocytosis – RosaiDorfman disease
 Kawasaki disease
 Castleman disease
typically presents in children with
massive nontender cervical
lymphadenopathy, fever, and skin
nodules.
 characterized by benign, self-limited
lymphadenopathy.
 Biopsy shows classically dilated sinuses,
plasma cells, and the proliferation of
histiocytes.
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acute multisystem vasculitis in children.
present with:
› acute, nonpurulent cervical lymphadenopathy;
› erythema, edema, and desquamation of the
hands and feet;
› polymorphous exanthem;
› conjunctival injection; and erythema of the lips
and oral cavity.
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Dx: made by clinical judgment.
Early identification and treatment with
aspirin and globulin are imperative in
avoiding serious cardiac complications.
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a rare, benign lymphoepithelial disease
potential development of Kaposi sarcoma
and lymphoma.
This disease affects both sexes equally and
can occur at any age.
This disease occurs most commonly in
thoracic lymph nodes (70%).
Dx: tissue biopsy.
Tx:
Isolated - surgical resection with
excellent prognosis.
Multicentric - chemotherapy
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METASTATIC LYMPH NODE
THYROID MASSES
LYMPHOMAS
SALIVARY NEOPLASMS
PARAGANGLIOMAS
LIPOMAS
SOLITARY FIBROUS TUMOR
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tend to present as asymptomatic lesions that
progress slowly
firm to palpation.
The associated symptoms are related to the
primary site: odynophagia, dysphagia, dysphonia,
otalgia, and weight loss.
The most common metastatic lesion to the neck is
squamous cell carcinoma.
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Dx: FNA biopsy. More reccurence with excisionnal
Bx
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CT and MRI, may be helpful in the search for the
primary tumor.
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If the initial examination and imaging fail,
a direct endoscopic examination.
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Knowing the location of the node assists
in guiding the surgeon to suspicious
areas.
› posterior triangle: nasopharyngeal lesion,
› jugulodigastric nodes: tonsils, base of tongue, or
supraglottic larynx.
› supraclavicular area: digestive tract,
tracheobronchial tree, breast, genitourinary
tract, and thyroid gland.
A primary thyroid tumor manifests in the
anterior compartment of the neck.
A thyroid mass in a patient with hoarseness
and a history of neck irradiation should be
considered malignant.
Dx: Ultrasound, thyroid scans, and thyroid
function tests.
Dx: FNA.
The treatment is based on histologic findings.
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can occur in all age groups but more
common in children and young adults.
In Hodgkin disease, up to 80% will have at
least one neck mass.
presents with fever, chills, and diffuse
lymphadenopathy.
Dx: FNA suggestive for lymphoma. Open
biopsy may be necessary to obtain
sufficient tissue for histopathologic
classification.
Staging workup that includes CT scanning
of the head, neck, chest, and abdomen.
Most parotid lesions are found to be benign.
Submandibular gland tumors have an increased
incidence of malignant pathology.
 Benign salivary lesions typically present as
asymptomatic masses.
 Symptoms such as pain, cranial nerve involvement,
rapid growth, or overlying skin involvement are
highly suggestive of malignant growths.
 Dx: CT scanning, MRI, nuclear scans, and
sialography.
 FNA is the diagnostic test of choice.
 Tx: surgery
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arise from extra-adrenal paraganglia.
Carotid body tumors and glomus tumors are
paragangliomas that present as neck masses in the
upper jugulodigastric region in close proximity to the
carotid bifurcation.
 They are pulsatile, and bruits can usually be heard on
auscultation.
 are mobile from side to side but not up and down.
 10% have a family history, 10% present with multiple
paragangliomas, 10% of all paragangliomas are
malignant.
Dx: The gold standard was angiography in the past, but
today MRA.
Tx: Surgical excision, Radiation for non resectable case,
preoperative embolization may aid in the surgical
resection.
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most frequently in patients over 35 years of
age.
can occur in various neck locations.
Asymptomatic
Dx: CT, MRI.
Tx: surgical excision if symptomatic.
DD: Liposarcoma: similar imaging
appearance but more progressive and
locally infiltrative course.
 Biopsy can be considered in such cases.
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spindle cell neoplasms of mesenchymal origin.
Most solitary fibrous tumors are located in the
thorax.
An estimated 5–20% of thoracic solitary fibrous
tumors have been reported as malignant, but
malignant extrathoracic tumors are rare.
In the head and neck, the oral cavity is the
most common site, but there have been case
reports involving all head and neck sites.
present as asymptomatic slow-growing
masses.
Tx: local resection.
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CT
MRI.
› differentiate solid, cystic, and vascular masses;
› localize a mass in relation to the vital structures of the neck;
› identify a potential head and neck source for the neck mass
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Ultrasonography
› distinguishing solid from cystic masses
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Chest X-rays
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if there is a high index of suspicion for granulomatous diseases
such as sarcoidosis or tuberculosis.A chest film is also able to
detect a metastasis from a head and neck cancer or a primary
malignant neoplasm within the lungs.
Positron emission tomography
looking for systemic diseases:
 antinuclear antibody in Sjogren syndrome,
which can present with parotid
enlargement and lymphadenopathy.
 tuberculosis,
 atypical mycobacteria,
 mononucleosis,
 toxoplasmosis,
 cat-scratch disease.
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use of a 23- or 25gauge needle.
can differentiate a
cystic mass from an
inflammatory mass,
malignant tissue from
benign tissue,
lymphoma from
carcinoma.
PCR for EBV, HPV in
suspicious Scc.
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