Sacrococcygeal teratomas (SCTs) are large tumors of germ cell origin arising from the coccyx. These tumors occur in approximately 1 per 35,000 live births and are often diagnosed prenatally (fetal diagnosis). The surgeons at Pediatric Surgeons of Phoenix manage these often massive tumors in conjunction with neonatologists, pediatric anesthesiologists, and pediatric oncologists. This multidisciplinary approach allows us to offer the families the best care available. A small number of fetuses develop “hydrops fetalis” which may result from the tumor stealing too much of the circulating blood for the baby to do well. There patients may be candidates for fetal intervention since the loss rate in this subset of fetuses is so high. All other infants are treated after birth.
The tumors may be benign, malignant, or “immature.” All patients with sacrococcygeal teratomas diagnosed prenatally are seen in our office for a detailed discussion of the condition and treatment options. Please see Prenatal Consultations sections for a detailed presentation on Sacrococcygeal Teratomas.
Data source for chance of survival charts: Usui N, Kitano Y, Sago H, KanamoriY, YonedaA, Nakamura T, NosakaS, Saito M, Taguchi T. Outcomes of prenatally diagnosed sacrococcygealteratomas: the results of a Japanese nationwide survey. J PediatrSurg. 2012 Mar;47(3):441-7.
Basics
A teratoma is a true tumor or neoplasm composed of multiple tissues of kinds foreign to the parts in which it arises.
Where do they come from? Several theories abound. One suggests wandering Germ Cells are left behind during the migration of embryonic germ cells from yolk sac to gonad during fetal development.
The tumor must (by definition) contain elements derived from more than one embryonic germ layer, must be foreign to the anatomic site and cannot have resulted from metaplasia. It must also show a highdegree of disorganization
The tumor may be either: (1) Benign (2) Malignant (3) Benign with Immature Elements (kind of benign, but worrisome).
Benign Teratomas contain only recognizable adult mature tissues such as epithelium, bone, cartilage, epithelium
Malignant Teratomas contain a malignant tumor among the benign parts. Examples include Embryonal carcinoma, Yolk Sac Tumors, endodermal sinus tumor, Choriocarcinoma, Germinomas, seminomas, dysgerminomas, Mixtures of Malignant Cells
Immature Teratoma contain cells that are not malignant, but are fetal and should not be present in a newborn. These immature elements may or may not represent cancer.
Because benign and malignant, mature and immature elements may exist side by side in the same teratoma, malignant areas may escape being sectioned.
Treatment
Sacrococcygeal Teratomas
After the Surgery
Chemotherapy may deter recurrence
Recurrent malignant tumors are at risk for death from tumor progression
For immature, but benign tissue, the decision to treat is more difficult
Recommendation:
Many oncologists recommend chemotherapy for patients with Immature Elements and elevated alpha-Fetoprotein or other markers. Consult with an experienced pediatric oncologist who is a member of the Children’s Oncology Group and works at a COG-affiliated hospital.
See www.curesearch.org and search “teratoma” for even more info.
