++
While neoplasms of the head and neck are rare, several types
of benign and malignant neoplasms are unique to the skull base and
neck and require a variety of treatments (eTable
373.1). Many of these neoplasms are discussed more fully in
other areas of this textbook.
++
The primary tumor site of nasopharyngeal carcinoma is in the
nasopharynx, but cervical metastasis at the time of presentation
is common.39 The undifferentiated form of this
tumor that is most common in children is associated with Epstein-Barr
virus exposure. Nasal obstruction and middle ear effusion from nasopharyngeal
mass effect are frequent with nasopharyngeal carcinoma, as is the
presence of a solitary solid mass in the posterior neck. Treatment
involves primary radiation therapy and adjuvant chemotherapy, with
surgical therapy only rarely considered.
++
Rhabdomyosarcoma can occur in any head and neck location, frequently
involving parameningeal areas (see Chapter 455).40 Rhabdomyosarcoma
frequently causes significant skull base destruction from asymptomatic
or rapid tumor growth before eventual diagnosis (eFig.
373.4).
Treatment generally consists of multimodality therapy with radiation
and chemotherapy. Surgical intervention is considered for initial diagnosis.
Several series demonstrate that after initial treatment, resection
of any residual tumor is beneficial if complete resection is achieved. Localized
rhabdomyosarcoma, such as those seen in the orbit, has a favorable
prognosis, while parameningeal tumor sites are difficult to completely
eradicate. Ewing sarcoma can occur in or around any bone of the
skull base and facial skeleton, and it is commonly seen in the mandible
(see Chapter 454). Esthesioneuroblastoma is
an uncommon malignant tumor that has been thought to arise from
olfactory epithelium but can occur anywhere in the superior nasal
cavity.41 These tumors cause nasal obstruction
and facial deformation and are usually treated with aggressive craniofacial
resection followed by radiation therapy.
++
++
Neuroblastoma is the most common malignant neoplasm in infants,
and up to 9% present with a primary head and neck lesion
(see Chapter 457).54 Children
with head and neck neuroblastoma can present with a neck mass, compressive
airway symptoms, or even a Horner syndrome from cervical sympathetic
trunk involvement. While surgical resection is favored for an isolated
lesion, combined modality therapy is used for advanced disease.
++
Lymphomas, both Hodgkin’s and non-Hodgkin’s
types, can present at head and neck sites in children (see Chapters 451 and 452).
Persistent enlargement of lymph nodes, particularly when systemic
signs or symptoms suggestive of malignancy are present, should warrant
open biopsy to exclude lymphoma.
++
Ten percent of thyroid cancer occurs in patients under 21 years
old (see Chapter 530). While well-differentiated
thyroid carcinomas are most common in young patients, cervical metastasis
at the time of presentation is unfortunately also common in children.43 Overall
survival is excellent despite aggressive disease, with surgical
treatment followed by possible radioactive iodine therapy and careful
long-term surveillance. Malignant salivary tumors can occur in children,
most commonly mucoepidermoid carcinoma. These tumors are treated
with surgical excision and facial nerve preservation, with postoperative
radiotherapy decisions based on tumor type, histologic grade, and
disease stage.44
++
Juvenile nasopharyngeal angiofibroma is an unusual, histologically
benign but locally aggressive tumor that arises in the nasopharynx
in older male children (see Chapter 370 and eFig. 370.4). This tumor causes nasal obstruction
and epistaxis. They can be localized to the nasopharynx, or they
can spread throughout the skull base and into the middle cranial
fossa.
++
A variety of benign tumors present as head and neck masses in
children. Children with neurofibromatosis can have large plexiform
neurofibromas that cause airway symptoms and other difficulties
from mass effect (eFig. 373.5). Benign salivary tumors, most commonly
pleomorphic adenoma, can arise from any of the major or even minor
salivary glands in older children. These tumors are treated with
complete surgical resection.
++