Teratomas of the Head and Neck

A teratoma is a tumor that grows from stem cells and can have virtually any sort of body tissue within it. Some teratomas are solid, with a well-developed blood supply. Some are cystic, which means they are filled with fluid. And some have a mix of solid and cystic areas. They can grow outside the body, inside the body or partially inside and partially outside the body.

Teratomas are rare. When they develop, they most often occur on the lower back (sacrococcygeal teratoma), testes or ovaries. Less often, teratomas occur on the neck (cervical teratoma) or, more rarely, in the mouth (oral teratoma), eye (orbital teratoma) or face (facial teratoma). Teratomas can also occur in the middle of the chest (mediastinal teratoma).

Teratomas most commonly develop before birth, on a growing fetus, and can be quite large by the time a baby is born — sometimes as large as or even larger than the child. Teratomas can also develop later in childhood or in adulthood. A teratoma that is entirely inside the body may not be noticed until it grows large enough to cause external swelling or a functional impairment, such as difficulty breathing or swallowing.

Large teratomas of the neck and mouth can interfere with a child’s breathing. When these are diagnosed in a fetus during pregnancy, they require specialized treatment immediately at birth.

The causes of teratomas are not known. These tumors appear to occur randomly and to have no association with genetic or environmental factors. That means that teratomas do not run in families or aren’t caused by some outside influence (what the mother ate or where she went). Having a child with a teratoma does not make it more or less likely that a family’s next child will have this rare problem.

Symptoms of teratomas vary with the size of the tumor, where on the head and neck it appears and whether it is external or internal. Small teratomas that are entirely inside the body may cause no symptoms. Large teratomas, on the other hand, can cause disfigurement and life-threatening functional impairments.

Their size and growth can compress nearby structures. Large neck teratomas can close the windpipe (trachea), and large teratomas in the mouth can make it difficult for a child to breathe or swallow.

Large teratomas that include growth outside the body are typically noticed in routine prenatal imaging. When this does not happen, as might be the case with a smaller teratoma or one that is entirely internal, symptoms can include:

• swelling (of the neck or around the eye, for example)
• difficulty breathing
• a wheezing or whistling sound when breathing
• difficulty swallowing
• a noticeable external tumor

When a teratoma of the head or neck is suspected before birth, based on prenatal ultrasound imaging, additional information is gathered and imaging and testing are done to confirm the diagnosis and identify the nature of the problem with more precision.

At Children’s Hospital of Philadelphia (CHOP), mothers carrying a fetus with a known teratoma are referred to the Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, where a specialized multidisciplinary team meets with the mother or both parents for this evaluation. A complete obstetric history is taken and physical and genetic evaluation is performed to rule out other problems with the pregnancy.

Prenatal imaging studies may include:

• a level II ultrasound, which provides more detailed information on fetal growth and development and can be focused on the area with the tumor
• an ultrafast fetal MRI, which can reveal detail not visible with ultrasound imaging

These specialized imaging techniques help the medical team determine the size and location of the tumor and can provide information that may help in planning future surgical procedures.

One potential pregnancy problem related to neck and mouth teratomas is an unhealthy buildup of amniotic fluid, a condition known as polyhydramnios. This can occur when the tumor interferes with the baby’s ability to swallow amniotic fluid in the womb. Left untreated, polyhydramnios can bring on preterm labor. Understanding the size and location of the teratoma through imaging studies helps the medical team know whether to watch for this risk.

After the baby is born, or to confirm a diagnosis of a teratoma in an older child, additional tests may be done. At CHOP, older children with a suspected teratoma of the head and neck are referred to the Head and Neck Disorders Program.

Tests to refine the diagnosis include fine needle aspiration (FNA), which draws a small sample of tissue from the tumor for study under a microscope. FNA or a biopsy can help to determine whether the tumor is benign or cancerous. The great majority of childhood teratomas are benign (not cancerous and not a risk to spread to other parts of the body).

Treatment of teratomas of the head and neck typically requires the coordinated effort of a team of specialists, including specialists in:

• maternal-fetal health (perinatology)
• pediatric ear, nose and throat (ENT) surgery
• cancer diagnosis and treatment (oncology)
• radiology
• pediatric, maternal and oncology nursing

For those diagnosed during pregnancy, the specialists in the Center for Fetal Diagnosis and Treatment work closely with the surgical team in the Head and Neck Disorders Program to provide comprehensive care to the mother and child at each stage of the treatment process. Close coordination among specialists and deep experience in treating rare conditions such as head and neck teratomas is a hallmark of CHOP’s practice and an important factor in our record of successful outcomes.

Specific treatment will vary based on the location of the teratoma, its size and whether the tumor is cancerous. Treatment may include:

Prenatal monitoring when the teratoma is diagnosed before birth, to watch for any health problems with the baby or the mother during pregnancy.

• Periodic ultrasound imaging may be done to observe the baby’s development and the growth of the tumor.
• Fetal echocardiograms may be used to check the health of the baby’s heart.

Prenatal treatment when the baby’s or the mother’s life is at risk. This may include

• draining fluid from a cystic tumor to reduce its size
• draining amniotic fluid from the womb if an excess of fluid accumulates
• treating the mother with a medication that reduces blood flow to the baby’s kidneys if an excess of amniotic fluid accumulates

Delivery using an EXIT procedure and adjacent operating rooms for mother and baby.

• In an EXIT procedure, the baby is delivered in stages, remaining attached to the umbilical cord until breathing can be established through a breathing tube if the airway is blocked. The medical team at CHOP has performed more EXIT procedures than any other hospital in the world, and has adapted the procedure for delivering babies with cervical and oral teratomas.
• The practice of delivering at-risk babies in adjacent operating rooms for mother and baby is an established practice in the Garbose Family Special Delivery Unit, the world’s first birth facility in a pediatric hospital specifically designed for healthy mothers carrying babies with known birth defects.

Surgery to remove the tumor. Because head and neck teratomas press on and often damage adjacent tissue, this surgery must be performed with special care to preserve function, especially airway function. And because these tumors are often in locations on the face or neck, skilled surgeons will preserve or restore a natural appearance.

• Surgery to remove a cervical (neck) teratoma focuses on restoring a clear airway. After long compression, the cartilage of the airway is often weakened and floppy. It often takes time — as long as a year or two after the tumor is removed — for it to recover the stiffness required for healthy breathing.
• Reconstructive surgery may be needed if the removed teratoma leaves distortion in the facial structure, as might happen with an orbital (eye) tumor or gaps or weaknesses in the airway after removal of a cervical or oral teratoma. Children needing airway construction will be referred to CHOP’s Center for Pediatric Airway Disorders.

Babies who survive the fetal period with a teratoma and have the tumor removed after birth have very good outcomes. The great majority of these childhood teratomas are benign, which means they are not cancerous and do not spread to other parts of the body. Most children go on to lead normal lives after treatment.

Babies who are treated for teratoma are monitored for about three years to be sure the tumor does not grow back, which can happen if tumorous tissue is missed in the initial surgery. This monitoring is done with imaging studies, sometimes supplemented by blood tests to check for indicators of tumor growth.

When recurrence of a tumor is detected early, follow-up treatment is generally effective.

If the thyroid must be removed as part of the procedure to remove a cervical teratoma, its function can be replaced through thyroid replacement therapy — a daily dose of medication.
Periodic follow-up through later childhood and into adolescence and adulthood is highly recommended to detect any later tumor recurrence and minimize the risk of any regrowth becoming cancerous.