Understanding Sacrococcygeal Teratoma (SCT)

Child with SCT treated through the Grant Scott Bonham Fetal Center at Primary Children’s Hospital

What is a sacrococcygeal teratoma?

A sacrococcygeal teratoma is a tumor composed of all tissues types of the fetus arising from the coccyx, or tail bone. It is much more common in females than in males (4:1) and can be solid, cystic, or mixed

1 in 23,000-40,000 live births
Malignant change more frequently observed in males
Solid SCTs can be very vascular which may lead to heart failure
Mortality for prenatally diagnosed SCT approaches 50%
Classified by the amount of external tumor present

(Pic: Newborn child with a large SCT)

Types of sacrococcygeal teratoma?

A sacrococcygeal teratoma is classified by the amount of tissue that is outside the fetus.

Type I - primarily external
Type II - External with an internal component
Type III - Both external and internal
Type IV - Internal only

(Pic: Fetus with different types of SCT)

Multidisciplinary care team at the Grant Scott Bonham Fetal Center at Primary Childrens Hospital

Fetal Center: the first step in SCT care

Multidisciplinary Evaluation: meet with MFM specialist, pediatric surgeon, neonatologist, and other experts who will care for your baby

Comprehensive Imaging: detailed ultrasounds and other tests when indicated to assess your baby's condition and help plan treatment

Coordinated Delivery Planning: our team works to ensure your baby receives immediate specialized care at birth

MRI of fetus with SCT treated in the Grant Scott Bonham Fetal Center at Primary Children’s Hospital

How is an SCT evaluated?

Regular monitoring with a detailed ultrasound throughout pregnancy is recommended to track changes in tumor size and rate of growth

A fetal echocardiogram is important to obtain due to the probability of the fetus developing heart failure (or hydrops), more commonly seen in large, solid SCTs

A fetal MRI is routinely obtained to help further delineate the anatomy, especially the amount inside the fetus, and to evaluate for other anomalies

Genetic testing may be recommended based on additional findings seen on imaging (ultrasound/MRI)

(Pic: fetal MRI with arrow demonstrating a large, solid SCT)

Echocardiogram of a fetus with an SCT treated at the Grant Scott Bonham Fetal Center in Utah

How is a large SCT followed closely during pregnancy?

A large SCT, especially when primarily solid, can lead to heart failure, seen by extra fluid and swelling in the fetus, called hydrops. If this develops, the fetus has a very high likelihood of dying.

Therefore, mothers whose fetus has a large SCT are followed very carefully, undergoing frequent ultrasound to evaluate tumor volume, placental thickness, amniotic volume, and for evidence of hydrops amongst other things.

A fetal echocardiogram is also obtained often to obtain a measurement as to how hard the heart is working, called combined cardiac output (CCO), as well as other signs that the heart is under strain.

These findings will help the doctors know how the child is doing and when best to move forward with delivery or surgery

(Pic: Echocardiogram of a fetus with large SCT)

How are large SCTs treated?

A fetus with a large SCT will require surgery, although timing will depend on how sick the child is from the tumor

Fetal resection - Very rarely needed. Surgery is performed while the fetus is still early in the womb (< 27 weeks), to remove as much tumor as possible, to help the fetus survive and get closer to term.
EXIT-to-resection – rarely needed. Allows resection of the SCT of a severely compromised fetus > 27 weeks, while receiving support from the placenta, followed by delivery of the baby
C-Section-to-resection – Controlled Cesarean delivery of the child, followed by neonatal resuscitation and immediate resection of the lesion. Typically performed after 27 weeks gestational age in a child with compromise from the lesion
Planned postnatal surgical resection - performed when the child is stable afterbirth within the first week of life

(pic: shows the fetal surgery team preparing for a C-section-to-resection of a large saccrococcygeal teratoma)

Multidisciplinary care treat treating a child with an SCT at Primary Childrens Hospital in Utah

Neonatal Intensive Care

Babies with a large SCT should be delivered at our center where a neonatal intensive care unit (NICU) is available that specializes in the care of these infants and where a pediatric surgeon can immediately evaluate your child. The neonatologist is a specially trained pediatrician that will manage your baby’s medications, feeding, and daily needs while in the NICU.

Once the child is born, a neonatologist will evaluate the baby and make sure the heart and lungs are working appropriately. Some infants will require a breathing tube and machine to assist with breathing. Some may also require medications to help maintain a normal blood pressure.

A tube placed through the mouth (oral gastric or OG) all the way to the stomach. This tube will suck out any fluid with in the stomach to prevent your baby from choking or breathing stomach contents into the lungs and to prevent the intestines from becoming too dilated with air.

Your baby will receive fluids and antibiotics through a special IV called a PICC line, initially through the umbilical cord, then placed in one of the limbs. Because the child will not be initially allowed to eat, they will also receive nutrition through the PICC line called TPN, or total parenteral nutrition. TPN contains protein, fat, sugar, vitamins, and minerals and will meet all your baby’s nutritional needs

(Pic: Neonatal care team treating a newborn with a large sacrococcygeal teratoma)

Definitive Surgical Resection

Surgical resection of the SCT will be performed, either as a primary procedure in stable newborn infants, or as a definitive procedure in a child that required emergent resection (fetal surgery, EXIT, or c-section to resection) due to severe compromise of the heart or hydrops.

The pathological findings of the mass will determine if further treatments such as chemotherapy will be required.

All children after resection will undergo close surveillance over the course of the next few years, including imaging and laboratory evaluation to watch for possible recurrence of the tumor.

(pic: Shows a child who underwent resection of a large sacrococcygeal teratoma)

Long-term care and support

What to expect after discharge from the hospital

Children with an SCT that undergoes treatment, especially at an earlier age, often require continued support after discharge from the hospital.

This may include:

NG Tube Feedings
Physical, Occupational, and feeding therapies
Nutrition (HEFT) clinic
Wound/scar care (pediatric surgery/plastic surgery)
Colorectal Clinic (for constipation and bladder emptying management)
Cardiology (to manage heart failure)
Oncology (for tumor surveillance depending on pathology)

(pic: Dr. Fenton with a patient who underwent care for a large SCT)