Sacrococcygeal teratoma
Key points
1 in 35,000 - 40,000 live births
3:1 female to male
Familial if Currarino triad (autosomal dominant) - exclusively pre-sacral
MYCN gene amplification, distal 10q trisomy syndrome, mosaic trisomy
Antenatal features
Risk of high output cardiac failure and non-immune hydrops in utero
Risk of maternal mirror syndrome - oedema, hypertension, pre-eclampsia
MRI if diagnosis in doubt
Tumour volume to foetal weight ratio (TFR)
Tumour measured in 3 dimensions, divided by foetal weight measured on USS
If >0.12 = 80% hydrops and 60% mortality
If <0.12 = 100% survival
Altman Classification
Type I
Completely external
47% of cases
Type II
Predominantly external with significant intra-pelvic component
34% of cases
Type III
Predominantly intra-pelvic with an external component extending into the abdomen
9% of cases
Type IV
Entirely internal
10% of cases
Complications
>10 cm - 50% mortality
Yolk sac tumour most common malignant transformation, but can be other GCT
11% recurrence due to incomplete macro- or microscopic resection, unresected coccyx, tumour rupture, age, and immature/malignant histology
Approx 30% neurogenic bladder + constipation
7% faecal incontinence
Sexual function mostly normal
Cosmetic dissatisfaction in most
Limb function normal - but compensatory gait in some
Standard scenario
Concerns:
1. Achieving an oncological resection due to possible yolk sac elements
2. Mass effect and cardiac failure
If antenatal presentation
Calculate TFR
Look for non-immune hydrops
Can open hysterotomy and resect if hydrops up to 26 weeks
Early delivery weeks 27-32 if hydrops
If large cyst - can be aspirated to ease delivery
Consider if size of tumour puts risk of dystocia
EXIT + resect if highly vascular tumour
If postnatal presentation
Ensure stable and managed on neonatal unit
Nurse on side
History
Look for above on antenatal features
Examination
Size of mass, where to resect
Press on mass and check for bulging fontanelle - meningocoele
Look for oedema
Dysmorphic features
Investigations
aFP to look for yolk sac tumour elements
USS/MRI for Altman stage
Echo for heart failure
Management
Altman I + II - Sacral approach
III - abdo + sacral
IV - abdo
Place Heger in rectum
Chevron incision - pointing up
Follow tumour capsule
Resect coccyx
Control middle sacral artery - if bleeding and retracted - options: Pack, close and come back in 48 hours after seeking other opinions, or flip patient, open abdomen and control aorta
Avoid pelvic splanchnic nerves from sacral plexus
Pressure if presacral veins bleeding
Chemotherapy is not utilised for mature and immature SCT or completely resected malignant SCT
Chemotherapy only for metastases/malignant recurrence
Follow up
aFP surveillance - 10% recurrence rate
Bowel and bladder assessments and management - neuropathic bladder + constipation in approx 30%
Check: Cosmetic appearance, limb function
References
Sun, Raphael C, et al. "Sacrococcygeal Teratoma." Pediatric Surgery NaT, American Pediatric Surgical Association, 2023. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829143/all/Sacrococcygeal_Teratoma.
Rodriguez MA, Cass DL, Lazar DA, Cassady CI, Moise KJ, Johnson A, Mushin OP, Hassan SF, Belleza-Bascon B, Olutoye OO. Tumor volume to fetal weight ratio as an early prognostic classification for fetal sacrococcygeal teratoma. J Pediatr Surg. 2011 Jun;46(6):1182-5. doi: 10.1016/j.jpedsurg.2011.03.051. PMID: 21683219.
Altman RP, Randolph JG, Lilly JR. Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey-1973. J Pediatr Surg. 1974 Jun;9(3):389-98. doi: 10.1016/s0022-3468(74)80297-6. PMID: 4843993.