PRETERM PREGNANCY WITH SACROCOCCYGEAL TERATOMA

Murwani Emasrissa Latifah1*, Peby Maulina Lestari2, Jackson Mandala Putra1, Kemala Andini Prizara1,
Robertus Erik Kantona1
1
Department of Obstetrics and Gynecology dr. Mohammad Hoesin General Hospital, Faculty of Medicine
Universitas Sriwijaya, Palembang, South Sumatera, Indonesia
2
Department of Pharmacology, Sriwijaya University, Palembang, Indonesia

ABSTRACT
Introduction: Teratomas belong to the germ cell tumor group and are one of the tumors
commonly found in neonates, with a prevalence of approximately 1 in 20,000-40,000
pregnancies. This study aimed to illustrate diagnosis and management of preterm pregnancy with
sacrococygeal teratoma.
Case Illustration: A pregnant patient, referred for congenital anomalies, diagnosed with a
sacrococcygeal teratoma at 23 weeks. Cesarean section at 38 weeks delivered a baby with a
6x7x3 cm teratoma, weighing 3100g. CT scan confirmed a 1.7 x 0.8 cm lesion in the coccygeal
region. Chromosome analysis revealed a 46XX del(11XP15X?) karyotype, suggesting a small
deletion on chromosome 11p. Anatomical pathology confirmed a mature teratoma in the sacral
region.
Discussion: Sacrococcygeal teratomas (SCT), rare congenital tumors originating from totipotent
cells, present challenges in prenatal diagnosis and management. This case involves a female baby
with SCT and Beckwith-Wiedemann Syndrome (46,XX,del(11)(p15)), impacting developmental
outcomes. Perinatal mortality is high, with termination considered in severe cases, following
Indonesian guidelines. Surgical resection, typically postnatal, is the primary treatment, with
cesarean delivery recommended for low-risk cases. Vigilant monitoring is crucial due to variable
recurrence rates based on tumor type.
Conclusions: Early diagnosis of sacrococcygeal teratoma relies on Ultrasonography, essential
for informed counseling with the family. A multidisciplinary team is crucial for holistic
management, addressing medical, ethical, and religious considerations in neonatal care.
Keyword: Preterm Pregnancy, Sacrococcygeal Teratoma

Introduction
Teratomas belong to the germ cell tumor group and are one of the tumors commonly found
in neonates, with a prevalence of approximately 1 in 20,000-40,000 pregnancies. 1
Sacrococcygeal teratomas are believed to originate from totipotent cells along the Hensen's node,
anterior to the coccyx. Usually, these cells decrease in size over time and eventually disappear
due to the degeneration process. As mesoderm proliferates, the primitive streak becomes more
caudal, while the Hensen's node is located anterior to the coccyx. 2 This study aimed to illustrate
diagnosis and management of preterm pregnancy with sacrococygeal teratoma.
Case Illustration
The patient routinely visited the RSMH clinic, referred by an obstetrician, diagnosed as
G1P0A0, 23 weeks pregnant with congenital anomalies. Upon examination at 32 weeks of
pregnancy, the findings were: uterine fundal height at the level of the umbilicus 1⁄2 between the
xyphoid process and umbilicus (28 cm), longitudinal fetal position, cephalic presentation, 5/5
engagement, right occiput, fetal heart rate 143 beats per minute, no contractions. The ultrasound
examination revealed a cystic mass with solid components in the caudal sacrum, size 5.22 x 4.45
cm, suggestive of sacrococcygeal teratoma. A cesarean section was performed at 38 weeks of
pregnancy with result : a female baby with a sacrococcygeal teratoma measuring 6x7x3 cm was
delivered with a total weight (baby and teratoma) of 3100 grams and Apgar scores of 8-9-10.
Subsequently, a multislice CT scan was performed to confirm the diagnosis, showing a lesion
with insodense impressions, approximately 1.7 x 0.8 cm in the posterior coccygeal region
(clinical correlation is requested), and no evidence of meningocele. Chromosome analysis
revealed a karyotype of 46XX del(11XP15X?) with interpretation of metaphase analysis
showing no abnormal chromosomal count. Chromosome 11 short arm (11p) structure suspected
a small deletion. An anatomical pathology examination resulted in a mature teratoma in the
sacral region.

Discussion
Teratomas are classified as germ cell tumors and are among the tumors frequently
encountered in neonates, with a prevalence of approximately 1 in 20,000-40,000 pregnancies. 1
Sacrococcygeal teratomas (SCT) are believed to originate from totipotent cells along the
Hensen's node, anterior to the coccyx. Typically, these cells reduce in size over time and
eventually disappear due to the degeneration process. As mesoderm proliferates, the primitive
streak becomes increasingly caudal, while the Hensen's node is located anterior to the coccyx. 2
SCT generally presents in utero as a mass at the caudal end of the fetus or as a tumor in the
baby, which can be asymptomatic or exhibit symptoms of rectal or bladder obstruction. On
ultrasound examination, SCT usually appears as an irregular mass with a thick wall and both
cystic and solid components. A small percentage of children may experience weakness, pain, or
paralysis.3,4
Prenatal diagnosis is typically possible in the second trimester during routine sonography,
although first-trimester diagnoses have also been reported. Sonography usually reveals a mass
near the distal spine. The majority of prenatally diagnosed SCTs are solid or mixed cystic and
solid; calcifications often occur. Associated structural abnormalities may include bladder outlet
obstruction and hydronephrosis, rectal stenosis or atresia, and secondary cardiomegaly due to
vascular steal phenomena and high-output heart failure. Most SCTs diagnosed in utero are of
Altman types I or II.3,4
Evaluation of fetal ultrasound, placenta, and tumor during pregnancy is a crucial
component of the overall treatment plan. The primary goal is to identify fetuses at an increased
risk of fetal death due to hydrops fetalis (i.e., abnormal fetal fluid accumulation) and intervene
appropriately.
In this patient, there is also a genetic anomaly, a deletion in the short arm of chromosome 11,
short arm 15 (46,XX,del(11)(p15)), consistent with Beckwith-Wiedemann Syndrome (BWS).
Individuals with BWS mostly have normal intelligence and developmental outcomes. Poor
developmental outcomes are generally attributed to complications of prematurity or extreme
hypoglycemia rather than the syndrome itself. An exception is BWS caused by paternal
duplication of chromosome 11p15, which can be associated with moderate to severe intellectual
disabilities.5 The recurrence risk of BWS varies depending on its etiology. Most families have a
recurrence risk of less than 1%, but specific etiologies carry recurrence risks as high as 50%. 6
Prenatal diagnosis and strict monitoring have improved outcomes for fetal SCT, but
perinatal mortality remains high. Perinatal mortality estimates for prenatally diagnosed SCT
range from 25-50%, including termination of pregnancy, intrauterine death, and neonatal death.
Potential perinatal complications include preterm birth, spontaneous bleeding or tumor rupture,
and maternal mirror syndrome. The high perinatal/neonatal mortality and morbidity associated
with sacrococcygeal teratomas are linked to preterm birth and complications such as malignant
invasion, bleeding into the tumor, umbilical flow obstruction, high-output heart failure, hydrops
fetalis, and bladder outlet obstruction. In cases like these, termination of pregnancy may be
considered. In Indonesia, pregnancy termination must involve medical indications, i.e., for the
purpose of saving the mother's life.
 Regarding the issue of pregnancy termination in young pregnancies in Indonesia, the
Indonesian Ulema Council (MUI) issued a fatwa regarding the permissibility of terminating
young pregnancies under certain conditions: the fetus threatens the life of the pregnant woman,
the fetus is the result of rape, termination of a young pregnancy is done before the fetus is 40
days old (before the embryo develops at the 'alaqah stage’), and the woman is at risk of mental
health, so termination of a young pregnancy is considered an essential and urgent solution.7
The primary treatment for SCT is surgical resection of the tumor and coccyx to prevent
recurrence. This surgery is often performed after birth and is only done in utero as a temporary
measure in specialized healthcare centers and specific cases with tumors at high risk for hydrops
and pregnancies earlier than 32 weeks. Delivery by cesarean section at 36 weeks is
recommended for fetuses with low-risk SCT. Typically, for SCT without malignant elements,
complete surgical resection is sufficient, followed by 3 years of measuring AFP and beta-human
chorionic gonadotropin hormone levels. Complete excision surgery, including coccygectomy, is
recommended to avoid recurrence. In malignant cases, adjuvant chemotherapy with platinum-
based regimens is advised.4,8 SCT has a recurrence rate of 10% with complete resection and
mature teratoma types, while immature teratomas and malignant teratomas have consecutive
recurrence rates of 33% and 18%, respectively.9

Conclusion
The capability of Ultrasonography is crucial for confirming the diagnosis of
sacrococcygeal teratoma in early pregnancy. Counseling regarding fetal anomalies in such cases
should be conducted in detail with the family and mother to determine appropriate actions for the
neonate. An optimal multidisciplinary team is required for the proper management of both the
mother and the fetus, taking into account medical, ethical, and religious perspectives.

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