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common in infants and children less than 2 years of age, is a neuroendocrine tumor
arising in the adrenal glands, but can originate in the high thoracic and cervical
sympathetic ganglia or metastasize to cervical and supraclavicular lymph nodes. Again,
prompt imaging with chest radiograph, as well as abdominal imaging with computed
tomography (CT) or magnetic resonance imaging (MRI) should be initiated in cases of
suspected neuroblastoma.
Rare noninfectious causes of chronic cervical lymphadenopathy deserve consideration
in the appropriate clinical context. Sarcoidosis is a multisystem granulomatous disorder
that affects young adults, particularly African Americans, causing systemic symptoms
such as weight loss, cough, fatigue, and joint pain. Patients often have chronic bilateral
cervical adenopathy, and chest radiograph in such patients may reveal hilar adenopathy as
well.
Autoimmune lymphoproliferative syndrome (ALPS), also known as Canale–Smith
syndrome, is a genetic disorder of lymphocyte apoptosis leading to lymphadenopathy,
splenomegaly, and pancytopenia. Children with ALPS present in the first year of life with
massive cervical adenopathy and splenomegaly.
Histiocytic disorders, another uncommon group of diseases that cause prominent
cervical adenopathy, occur when there is an overproduction of dendritic cells or
macrophages which cause organ damage and tumor formation. Langerhans cell
histiocytosis (LCH) affects children less than 3 years of age, causing lytic bone lesions
and unilateral or bilateral soft and matted cervical lymphadenopathy. Sinus histiocytosis
with massive lymphadenopathy (Rosai–Dorfman disease) presents in children as
massively enlarged, nontender cervical lymphadenopathy, along with involvement of the
nasal cavity, lytic bone lesions, pulmonary nodules, or rash.
Finally, the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical
adenitis (PFAPA) is an autoinflammatory disease presenting in young children that cycles
every 2 to 9 weeks. Children with PFAPA experience tender cervical lymphadenopathy
with flares. There is no specific diagnostic test, and though corticosteroids and
nonsteroidal anti-inflammatory drugs alleviate symptoms, the condition resolves
spontaneously by 10 years of age.

Other Lymphadenopathy of the Head and Neck
Beyond cervical lymphadenopathy, acute localized adenopathy of the head and facial
regions has a more narrow differential diagnosis, and the location of lymphadenopathy
often suggests a cause, based on the location of focal infections and drainage patterns of
nodes. Submandibular and submental nodes, draining the lips, buccal mucosa, and floor
of the oropharynx, enlarge with infection within the oral cavity. This includes dental
caries and abscesses, as well as gingivostomatitis. Herpetic gingivostomatitis generally
affects the anterior tongue, buccal mucosa, or lips, while Coxsackievirus
gingivostomatitis affects the posterior oropharynx, sparing the gingiva and lips, and
causes characteristic lesions on palmar and plantar surfaces. Lymphadenopathy in the