Pedi Teratomas and Dermoids Flashcards
What pediatric tumor arises from all three germ
cell layers and occurs most commonly in the soft
tissues of the neck, face, infratemporal fossa, orbit,
and upper aerodigestive tract when it occurs in the
head and neck?
Teratoma
True or False. All congenital teratomas manifest
with impressive respiratory distress and are benign.
False. Although most do present with respiratory distress
requiring interventions such as tracheostomies or extra-
corporeal membrane oxygenation and EXIT (ex utero
intrapartum treatment) procedures, a small number do
metastasize and are therefore considered malignant.
Malignancy does not indicate maturity of tissue on
histopathology, which tends to reflect the age of the host.
Do all metastatic teratomas result in progressive
disease and death?
No. Many actually differentiate in the metastatic location
and do not result in significant harm. However, fatal disease
has been reported in children, and aggressive malignant
disease is more common in adults.
What is the treatment of choice for malignant
congenital teratomas?
Surgical resection of the primary tumor is the treatment of
choice, as in “benign” teratomas, with consideration of
chemotherapy for metastatic disease. There are limited
data to guide decision-making for malignant disease.
What congenital, subcutaneous lesions contain
trapped epithelium and its adnexa (i.e., hair
follicles, hair, sebaceous glands) and form along
embryologic fusion lines?
Dermoid cysts
Where are the most common locations for
dermoid cysts in the head and face?
Anterior fontanelle, bregma, upper lateral forehead, upper
lateral eyelid, and submental region are the most common
locations.
Note: These cysts can occur in other body sites, such as the
ovaries, anywhere in the face, along the skull base, or spinal
axis.
How do dermoid cysts manifest?
These cysts are solitary, rubbery, firm, painless (unless
infected) mases, nonpulsatile, noncompressible, present
since birth, but often noted after minor trauma. If
associated with a sinus tract, they may have a dermal pit
with or without an associated tuft of hair. If the tract
extends to the CNS (common in the midline or nasal
dermoid cysts), the patient may have meningitis. Tumors
with infratemporal extension can result in midface widen-
ing, hypertelorism, and other issues associated with mass
effect and altered growth.
What is the most important imaging modality
when evaluating a midline dermoid cyst?
MRI
When should you remove a dermoid cyst?
Surgical resection is recommended for all dermoids to
decrease possible associated complications (e.g., infection,
meningitis, bony erosion).
