This document discusses sacrococcygeal teratoma (SCT), a rare congenital tumor. SCT arises from embryonic cells and can be benign or malignant. It presents prenatally via ultrasound in most cases. Risk of malignancy increases with age of diagnosis. Treatment involves complete surgical excision, with early surgery associated with better outcomes. Large or rapidly growing tumors may require intervention like chemotherapy or early delivery to prevent complications of high output cardiac failure. Complete resection remains the main treatment for benign SCT to prevent malignant transformation.

1. Sacrococcygeal teratoma
Dr. Muteb alruwaili
Pediatric surgery Fellow
Security Forces Hospital -Riyadh
4/6/2017

2. • most common congenital germ cell tumor.
• Incidence: 1 in 35,000-40,000 live births.
• F: M 3:1-4:1 ratio
• arises from the Hensen node, which is located
in the coccyx.
• The tumour is composed of the all three germ
cells (i.e. ectoderm, mesoderm and
endoderm)

3. classification

5. - When the tumor is found before 1 month of age, the risk of
malignancy is about 5%.
- whereas when the tumor is discovered between 1 and 12
months of age, the risk of malignancy is 60%
- in children older than 1 year, 75% of tumors are malignant.

6. Clinical presentation
• SCTs present in 3 clinical patterns:
-Fetal tumors present during prenatal ultrasound exams,
with or without maternal symptoms.
-?Neonates with large external, predominantly benign
lesions
-?Children between infancy and 4 years of age with
predominantly malignant lesions arising primarily within
the pelvis

7. Clinical presentation
• many neonates with SCTs do not have
symptoms BUT, some require intensive care
because of:
 prematurity
 high-output cardiac failure.
 disseminated intravascular coagulation
 tumor rupture or bleeding within the tumor.
 Lethal hyperkalemia

8. Diagnosis
• Prenatally:
• UltraSound:
 Gold standard
 Excellent detection rate for types 1,2,3.
 Average detection time 23 weeks of gestation.
 size , site , content ( cystic vs solid ) , growth pattern
and intrapelvic extension
 Method of delivery.

11. • MRI :
 As adjunct to US.
 Tumor composition.
 Better assessment for intrapelvic-intraabdominal
extension .
 additional fetal malformations

12. Fetal echocardiography :
 sensitive test to determine how hard the heart is
working .
 cardiothoracic ratio.
 If hydrops does develop , usually in rapidly growing
solid tumors, the fetus usually will not survive
without immediate intervention before birth.

13. Postnatally :
• Clinically ( Types 1,2,3 )
• MRI
• CT scan :
 Same indication for MRI
 As alternative to MRI

14. MANAGEMENT
• ANTENATALLY:
• intrauterine endoscopic laser ablation.
• Alcohol injection
• Radiofrequency ablation
• Open fetal surgery
o type I SCTs
o gestational age 20 to 30 weeks
o absence of placentomegaly
o an early hydrops,
o combined cardiac output 1600 to 1900 mL/kg/min adjusted for
gestational age.
• Early delivery , as early as 26 wks in selected cases.

15. Journal of Pediatric Surgery (2006) 41, 388– 393

16. • adverse outcomes:
 >61 cm3/week
 TFR (tumor volume to fetal weight ratio) >0.12
Journal of Pediatric Surgery 49 (2014) 985–989

17. CONCLUSION :
In the absence of fulminant hydrops , between 27- 32
weeks of gestation , preemptive early delivery can be
associated with surprisingly good outcomes in
appropriately selected fetuses with high-risk SCT.

19. POSTNATAL:
• Surgery
• The mainstay of the treatment of benign sacrococcygeal
teratomas is early and en-bloc excision of the lesion, given that:
●● the risk of malignant change in benign lesions
increases with
(a) age
(b) incompletely excised residual lesions
●● the tumor’s rich vascularity makes it vulnerable
to spontaneous ulceration and hemorrhage if left
unexcised

20. preoperative
• Imaging
• Labs ( including tumor markers)
• Adequate intravenous access and blood products
• General anesthesia is mandatory.
• Broad-spectrum antibiotics
• The stomach is emptied with a nasogastric tube
and an
• indwelling bladder catheter is inserted.

21. Operation

23. Removal of tumor and coccyx en bloc and
ligation of sacral vessels

25. Pelvic floor reconstruction

26. closure

27. • Retrospective study.
• 235 children ( 49 were males ) with SCT treated from 1970 to
2010 in the Netherlands in six pediatric surgical centers.
• risk factors for hemorrhagic mortality:
• -sex
- prematurity
- Altman type
- tumor volume and histology
- necessity of emergency operation and
- time of diagnosis.

28. • type I (n = 81; 35.5%)
• type II (n = 57; 23.7%)
• type III (n=36; 15.8%).
• type IV (n = 54; 23.7%)
• mature teratoma (66.2% )
• an immature teratoma ( 18.4%)
• malignant teratoma ( 15.4%)

29. Approach:
- Sacral : 77.5%
- Abdominal : 3.2 %
- Combined : 19.3%
• In two patients the median sacral artery was laparoscopically
clipped prior to sacral tumor resection with coccygectomy.
( first patient was Type I of unknown size , the other one was
Type 4 tumor of 1200 cm3 )

30. • The overall mortality was 18/235 ( 7.7%)
• two main causes:
- long term :(9/235), malignant transformation/ recurrence
- short term : ( 9/235) , 2/9 due to sepsis/menngitis
7/9 due to circulatory failure and/or
tumor hemorrhage

32. Case report
Journal of Pediatric Surgery (2011) 46, 566–569

33. • a 31-year-old primigravida , 16 week gestation of twins with
one diagnosed with SCT Type 2.
• at 28 weeks, the mass was measured at 9.6 × 9.3 cm.
• at 29 weeks showed the mass measuring 20 × 17 × 14 cm.
• at 32 weeks, combined cardiac output of approximately 850
mL /kg/min with reversal of end-diastolic flow, indicating a
rapidly deteriorating condition.
• An urgent cesarean delivery was performed.
• Despite transfusion of blood, fresh frozen plasma and
platelets, there was ongoing bleeding into the tumor, and the
neonate was taken emergently to the operating room

35. STEPS
• Compression near the base of the tumor achieved hemostasis.
• A safe distance from the anus was maintained to avoid rectal
injury.
• Teflon strips were applied circumferentially with horizontal
mattress sutures to maintain this external compression

36. • An 18-gauge spinal needle was used to pass the prolene sutures
across the base of the tumor into the Teflon strip on either side.
• This controlled hemorrhage into the tumor and ligated most large
feeding blood vessels that were responsible for the high-output
cardiovascular instability.
• Subtotal resection of the tumor was then performed, with
removal of approximately 80% of the tumor mass.
• The cut surface of the tumor was covered with a vacuum
dressing.
• On postoperative day 7, complete resection of the mass and
wound closure from a posterior sagittal approach was performed

38. Outcome
• Antenatal diagnosed SCT – overall survival of ~75%.
• If live born – >90% survival.
• Recurrence occurs in 10–20% of benign SCTs and in
~30% of malignant stage 1 SCTs
(often late presenters).

39. REFERENCES
• Marijke E.B. Kremer, Hemorrhage is themost common cause of neonatal mortality
in patients
• with sacrococcygeal teratoma, Journal of Pediatric Surgery xxx (2016) xxx–xxx
• T. VAN MIEGHEM, Minimally invasive therapy for fetal sacrococcygeal teratoma:
case series and systematic review of the literature, Ultrasound Obstet Gynecol
2014; 43: 611–619
• Marijke E.B. Kremer, Hemorrhage is themost common cause of neonatal mortality
in patients
with sacrococcygeal teratoma, Journal of Pediatric Surgery xxx (2016) xxx–xxx
• Jessica L., Early delivery as an alternative management strategy for selected high-
risk fetal sacrococcygeal teratomas, Journal of Pediatric Surgery (2011) 46,
1325–1332.
• Erica C., Outcome of antenatally diagnosed sacrococcygeal teratomas: single-
center experience (1993-2004). Journal of Pediatric Surgery (2006) 41, 388– 393
• JAN DEPREST, Invasive Fetal Therapy .
• Bryan J. Dicken and Frederick J. Rescorla, Sacrococcygeal Teratoma
• ASHCRAFT’S PEDIATRIC SURGERY

40. THANK YOU