Sacrococcygeal Teratoma: A Case Report and Literature Review
Amal Bayar1, Imen Ghadhab1, Yosra Jemaa2*, Rahma Issa3, Haifa Bergaoui1, Amina Mnejja2, Ines Miledi1, Rahma Issa2, Wiem Ben Slamia2, Ines Zouari1, Dhekra Toumi1, Olfa Zoukar1 and Raja Faleh1
1Department of Gynecology and Obstetrics, University Hospital Fattouma Bourguiba, Monastir, Tunisia
2Department of Gynecology and Obstetrics, Hospital HAJ ALI SOUA Ksar Hellel, Monastir, Tunisia
3Department of Gynecology and Obstetrics, University Hospital Taher SFAR Mahdia, Tunisia
Submission: May 10, 2022; Published: June 20, 2022
*Corresponding author:Yosra Jemaa, Department of Gynecology and Obstetrics, Hospital HAJ ALI SOUA Ksar Hellel, Monastir, Tunisia
How to cite this article:Amal B, Imen G, Yosra J, Rahma I, Haifa B, et al. Sacrococcygeal Teratoma: A Case Report and Literature Review. Acad J Ped Neonatol 2022; 11(5): 555879. 10.19080/AJPN.2022.11.555879
Sacrococcygeal teratomas are the most common fetal germ cell tumors but remain rare with an incidence of 1 in 35,000 to 40,000 live births. Prenatal ultrasound not only allows for early diagnosis but also for detection of possible complications of these tumors. Management is multidisciplinary. Fetal treatment in utero is now a conceivable alternative thanks to technical progress. We report the case of a sacrococcygeal teratoma which was diagnosed in utero and which was complicated by neonatal death due to the very significant increase in tumor size and the resulting shunt effect.
Sacrococcygeal teratomas represent the most common congenital tumor in newborns and infants, with an incidence of 1 in 35,000 to 40,000 live births . These teratomas account for 40% of all germ cell tumors and 70% of extra gonadal germ cell tumors in the pediatric population . It is a benign disease in 90% of cases, affecting girls 4 times more than boys . Diagnosis is usually made during the second or third trimester of pregnancy by ultrasound, where they appear as a solid, cystic or mixed mass in the sacral region. The prognosis of sacrococcygeal teratomas depends on several factors such as size, tumor growth rate, nature of the predominantly solid mass and tumor vascularity . Perinatal mortality varies between 13% and 50%, and most of these deaths are attributed to heart failure, when the tumor becomes so large during pregnancy, to exsanguinating tumor hemorrhage or both .
We report the case of a sacrococcygeal teratoma complicated by heart failure that was diagnosed in utero with ultrasound, with a review of the literature.
We report the case of Mrs. L.M, 40 years old, who presented to our emergency room with a threat of severe premature delivery at 29 weeks of amenorrhea (SA). Her history included a spontaneous abortion at 9 weeks of amenorrhea and a full-term delivery by cesarean section for fetomaternal disproportion with a healthy newborn.
The ultrasound examination revealed a sacro-coccygeal mass with exopelvic development, suggestive of a teratoma classified as type I, with poly-lobed contours, heterogeneous with a predominantly solid component measuring 18 cm in diameter. In addition, the presence of associated hydramnios and the absence of hyperemia or shunt effect on Doppler examination were noted.
The diagnosis of sacrococcygeal teratoma was made at 22 weeks’ gestation during the morphological ultrasound, where it measured 47x58mm (Figure 1). Her gynecologist asked her for a complementary exploration through magnetic resonance imaging (MRI) which better studied the lumbar spine to confirm its integrity with the absence of associated anomalies (Figure 2).
The acute episode of threatened preterm delivery was controlled by tocolysis with the administration of a corticosteroid pulmonary maturation cure. Close fetal monitoring (especially echocardiographic) was organized during the multidisciplinary decision. The evolution was marked by the development of maternal fever, which was due to COVID-19 infection, further complicating the management. During fetal heart rate monitoring,repetitive decelerations were noted attesting to a state of fetal
suffering at 31 SA. An emergency cesarean section was indicated.
The extraction was dystocic because of the sacro-coccygeal teratoma, which was 30 cm long, and the baby, male, was in an
apparent dead state (Figure 3). Immediate resuscitation of the
neonate was done but he did not recover.
Fetal teratomas are rare. The reported incidence is between
0.07 and 2.8 per 1000 pregnancies . Approximately 90% of
human teratomas develop in the gonads, mostly in the testes,
but a few cases develop in extra-gonadal sites such as the brain,
neck, mediastinum, retroperitoneum, and sacro-coccygeal area,
which remains the primary site of development of extra-gonadal
teratomas in newborns and young infants . CST develops from
the sacrum and coccyx, protruding outward and increasingly into
the pelvic cavity. It develops from the remnants of the primitive
streak that normally degenerates and disappears around the 4th
week of development. It is derived from pluripotent cells of the
three germ cell layers (entoderm, ectoderm and mesoderm) from
the primitive streak, resulting in a formation that is often made up
of different tissues (bone, hair, teeth, nerves...) . Sacrococcygeal
teratomas occur sporadically and no genetic or chromosomal
link has been established . Therefore, the indication for
amniocentesis for fetal karyotype is not recommended in simple
forms of SCT. However, associated structural abnormalities may be
found in 11% to 38% and may include sacral and spinal anomalies
and concomitant anorectal malformations .
In 1974, ALTMAN et al  proposed a topographic
classification of sacrococcygeal teratomas in their report to the
American Academy of Pediatric Surgery:
Type I: type I teratomas are almost exclusively external with a
minimal pelvic component.
Type II: Type II teratomas have a significant pelvic component.
Type III: the intra-abdominal and intra-pelvic component is
much greater than the external component.
Type IV: They are exclusively pre-sacral with no external
component; this is the most difficult form to diagnose both
clinically and on ante- or postnatal ultrasound.
Large TSS can exert a mass effect on the intra-pelvic organs
and present serious consequences such as constipation, fecal
incontinence, neurogenic bladder, and urinary incontinence.
The generalization of prenatal ultrasound has made it possible
to diagnose sacrococcygeal teratomas as early as intrauterine
life, and this can be very early in the first trimester of pregnancy
. Indeed, in the two series of Tongson et al and Ho et al
, the antenatal diagnosis was made from the 13th week of
amenorrhea. The use of ultrasound makes it possible to limit the
formation in question, to specify its solid or cystic nature, to detect
complications such as intra-tumoral haemorrhage or necrosis
and to look for associated malformations. It also allows close
monitoring of pregnancy, tumor size, and assessment of tumor
vascularity using Doppler flow. This is due to the fact that tumors,
richly vascularized and presenting a rapid growth or having a solid predominance, tend to undergo the phenomenon of vascular flight
which exposes the fetus to a high risk of developing hydrops or
feto-placental hydrops with the consequence of maternal pseudo
pre-eclampsia or the mirror syndrome . For high-risk tumors,
the frequency of surveillance is set at twice a week; for low-risk
tumors, respectively small, cystic, or with decreased vascularity,
the frequency can be reduced to once every two weeks.
Ultrasound is of great help in sacrococcygeal teratomas, but
it is sometimes limited and not very sensitive, especially when
compared with magnetic resonance imaging (MRI). Indeed, MRI
is an excellent tool to evaluate the exact size of the tumor, its
content and its extension to the pelvic organs to optimize the
pre and post-natal management. It also allows, thanks to its good
sensitivity when imaging the lower spine, to search for associated
malformations such as hip dislocation and spinal dysraphism .
Because of the fear of possible complications of sacrococcygeal
teratomas, the possibility of fetal death and maternal consequences
in hypervascularized forms, therapists are increasingly moving
toward a more interventional approach than simple prenatal
monitoring. This in utero therapeutic approach aims either at
the removal of the tumor or at reducing its vascularization and
thus decreasing its growth potential; which allows to diminish the
burden of the SCT on the fetal cardiovascular system . Open
uterus” fetal surgery allows access to the fetus through a maternal
laparotomy with hysterotomy . The major problem, and the
real Achilles’ heel of this therapeutic approach, is significant
maternal and fetal morbidity. Hence the role of fetal endoscopy,
which has proven its effectiveness with fewer complications.
Minimally invasive procedures used for the management of
complicated sacrococcygeal teratomas include radiofrequency
ablation, YAG laser ablation, ultrasound-guided percutaneous
puncture-drainage, and thermoablation. Researchers in Toronto
 tried laser ablation, radiofrequency ablation and intravascular
embolization in five fetuses with CST; only two patients survived
after these procedures. Other experiences from Brazil and France
have shown similar results of percutaneous interventions .
Therefore, fetal treatments of SCT, surgical or interventional,
are still experimental. However, given the poor prognosis of
fetuses developing heart failure and hydrops in utero, these early
intervention techniques deserve to be investigated and further
CSTs are rare congenital tumors with high risk of perinatal
morbidity and mortality. Ultrasound remains the technique of
choice to make an early diagnosis and establish a therapeutic
regimen. As soon as the diagnosis of CST is made, parents must be
informed and given psychological support. The management of this
tumor is multidisciplinary. Endoscopic interventions have made
considerable progress, allowing nowadays to address serious fetal
pathologies, formerly lethal, but remain to be improved.