Parents,Have you ever heard of sacrococcygeal teratoma? This is a tumor that can appear since the baby is not born and grows from the baby’s tailbone. However, treatment for this disease can only be done after the baby is born.
Although rare, this tumor can develop into malignant cancer at birth in some cases. Therefore, surgery must be performed immediately so that this case does not happen.
Definition of Sacrococcygeal Teratoma
Sacrococcygeal teratoma (SCT) is a tumor that develops before a baby is born and grows from the baby’s tailbone. This tumor is one of the most common in newborns.
The appearance of this tumor is usually covered with skin. The skin in question is a thin, transparent tissue called a membrane. Most tumors have many blood vessels passing through them.
The shape of this tumor can vary from person to person. It can come in various sizes, and sometimes it can grow out from behind or towards your child’s stomach.
When categorized according to location and severity, SCT can be divided as follows:
- Type I tumor , namely an external tumor (outside the body) and attached to the tailbone.
- Type II tumors have internal (inside the body) and external parts.
- Type III tumors can be seen from the outside, but most of the tumors are inside the child’s abdomen.
- Type IV tumor , the most serious tumor, cannot be seen from the outside. This tumor is located inside the body at the level of the tailbone.
Symptoms of Sacrococcygeal Teratoma
Symptoms that occur with sacrococcygeal teratomas vary greatly depending on the size and specific location of the tumor. Small tumors often do not cause any symptoms (asymptomatic) and can usually be surgically removed after birth without difficulty.
While SCT tumors with larger sizes can cause various complications before and after birth. Some symptoms that may appear both before and after delivery include:
Sacrococcygeal teratomas can grow rapidly in the fetus and require a very high blood flow. This condition can lead to fetal heart failure or a condition known as hydrops. This manifests as an enlargement of the heart, and fluid collection in body tissues, including the skin and other body cavities such as around the lungs (pleural effusion), around the heart (pericardial effusion), and/or in the abdominal cavity (ascites). If left untreated, hydrops can also be dangerous for the mother, resulting in similar symptoms such as swelling, hypertension , and fluid in the lungs with shortness of breath.
- Polyhydramnios , too much amniotic fluid
- Hydronephrosis , fetal urinary obstruction
- Bleeding into the tumor or tumor rupture with bleeding into the amniotic space
- Dystocia , a condition in which the fetus cannot be born due to the large size of the tumor.
Approximately 15% of cases of large fetal SCT show the above symptoms in addition to hydrops. Therefore, it is important to have very close monitoring during pregnancy to recognize these symptoms as early as possible.
In adults, sacrococcygeal teratoma may be asymptomatic. However, in some cases, it can cause progressive lower back pain, weakness, and abnormalities due to obstruction of the genitourinary and gastrointestinal tracts.
These symptoms include constipation and increased stool frequency or urinary tract infections. In rare cases, SCT tumors can cause partial paralysis (paresis) of the legs and tingling or numbness (paresthesia).
Causes of Sacrococcygeal Teratoma
The specific cause of SCT is still unknown. Sacrococcygeal teratoma is a germ cell tumor. Germ cells are cells that develop into embryos and then into cells that make up the male and female reproductive systems.
Most germ cell tumors occur in the testicles, ovaries (gonads) or lower back. When these tumors occur outside the gonads, they are known as extragonadal tumors. Researchers do not know how extragonadal germ cell tumors form.
One theory is that germ cells accidentally migrate to unusual locations early in embryonic development (embryogenesis).
Normally, these misplaced germ cells degenerate and die, but in the case of extragonadal teratomas, researchers speculate that these cells continue to undergo mitosis, the process by which cells divide and multiply, eventually forming a teratoma.
Sacrococcygeal teratomas are thought to arise from an area below the tailbone called “Henson’s Node.” This is an area where primitive cells persist (germ cells) that can give rise to cells from the three main tissue layers of the embryo: ectoderm, endoderm, and mesoderm.
This embryonic layer then gives rise to various cells and structures of the body. These SCT tumors can contain mature tissue that looks like other tissues in the body, or immature tissue that resembles embryonic tissue.
Frequency of Occurrence
Sacrococcygeal teratoma tumors are among the most common in infants, affecting at least 1 in every 35,000 to 40,000 births, according to the Children’s Hospital of Philadelphia .
Quotes from rare disease estimate the prevalence of this tumor occurs between 1 in 30,000-70,000 live births.
In addition, SCT tumors are more common in women than men with a ratio of 4:1. However, malignant tumors are more common in men.
Risk Factors
There are no specific factors that increase the risk of a baby being born with a s acrococcygeal teratoma. However, recurrence of the tumor is possible and can affect the outcome of children with SCT.
The risk factors for this can be tumor spillage, immature and malignant histology, or incomplete resection. Therefore, regular follow-up after surgery is mandatory to find tumor recurrence early and to improve the outcome.
Diagnosis
The signs and symptoms of SCT tumors depend largely on their size and location. Here are some ways to confirm the diagnosis of this disease:
USG
Some tumors can be diagnosed by ultrasound before the baby is born. An abnormally sized uterus is usually the first sign that your baby may have a tumor. The difference in size can be caused by a massive tumor or polyhydramnios (excess amniotic fluid). Less common presentations include maternal preeclampsia .
Fetal SCT may be cystic, solid or mixed in appearance on ultrasound. The heterogeneous appearance of the mass may be due to mixed areas of tumor necrosis, cystic degeneration, hemorrhage or calcification.
Amniotic Fluid Sample Test
In some cases, a sample of amniotic fluid or maternal serum may be taken and studied to determine the level of alpha-fetoprotein (AFP). AFP is a normal fetal plasma protein that, when elevated, may indicate the presence of certain conditions such as s acrococcygeal teratoma .
MRI of the fetus
If a s acrococcygeal teratoma is diagnosed prenatally, a careful examination is usually performed to rule out other anomalies. In some institutions, fetal MRI scans are also performed to better depict the anatomy of the tumor and displaced structures.
Echocardiogram
For large SCT tumors, very frequent ultrasounds and echocardiograms (to measure the size of the heart chambers and blood flow) are needed to monitor for signs of developing hydrops. During an echocardiogram, reflected sound waves are used to create images of the heart.
It is important to choose a medical team experienced in treating these tumors during pregnancy. All fetuses with large SCTl require delivery by “classic” cesarean section (large incision in the uterus) to avoid tumor rupture and hemorrhage during delivery. Most fetuses with large tumors are born prematurely and require expert perinatal care from a multidisciplinary team.
Monitoring Postpartum Symptoms
Other tumors may not be visible until after the baby is born. After delivery, your child may have symptoms that indicate a possible SCT, such as being unable to urinate or have a bowel movement because the tumor is pressing on their bladder or rectum. Some children have no symptoms at all.
Handling
Treatment for SCT requires surgery to remove the tumor. This depends on the diagnosis and the severity of your child’s tumor. The medical team may perform fetal surgery to remove the SCT before birth, or surgical resection of the tumor after the baby is born. Fetal surgery is only indicated if fetal hydrops is present, which is life-threatening for your child.
Possible Complications
When your baby is diagnosed with s acrococcygeal teratoma before birth and the condition is associated with fetal hydrops, the tumor can be life-threatening for both mother and baby. In severe cases, the tumor can take a lot of blood from the fetal circulation and cause the heart to work extra hard, possibly leading to heart failure.
Heart failure presents as fetal hydrops, a large accumulation of fluid in the fetus. Fetal hydrops associated with SCT progresses rapidly and is almost always fatal.
In addition, mothers who are pregnant with babies with SCT are at risk of experiencing “maternal mirror syndrome” where the mother’s condition is parallel to the sick fetus. When fetal hydrops is present, the mother may feel similar to the fetus. The pain appears with signs of preeclampsia.
Preeclampsia, also called toxemia, is a condition characterized by pregnancy-induced high blood pressure, protein in the urine, and swelling due to fluid retention.
Prevention
There is no specific way to prevent SCT from occurring in the fetus. However, conducting regular pregnancy check-ups can detect the presence of tumors early. Therefore, treatment steps can be taken immediately to prevent worse conditions.
Those are some things that Parents need to know about s acrococcygeal teratoma. Hopefully this article is useful.
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Republished with permission from theAsianParent Indonesia
