Sacroccocygeal Teratoma: Short Notes

Dr Vijay called me up to show a ultrasound picture of a patient he was seeing. The patient was referred for twins. What was revealed was instead a sacrococcygeal mass. A SCT, sacrococcygeal teratoma.

Born 18 May 2013, LSCS at 34 weeks.

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SCT is a spectrum of germ cell tumors most commonly seen in the pediatric age groups.

Most germ cell tumors manifest extragonadally (sacrococcygeal, mediastinal, retroperitoneum) compared to adults where germ cell tumors present at gonadal areas (ovary, testes).

Extragonadal germ cell tumors are believed to arrive from arrested or abnormal migration of primordial germ cells to the genital ridge.

SCT are mostly benign.

Female predominance 4:1

Classification (Altman's Study)

Type I - Predominantly external
Type II - external with intrapelvic extension
Type III - Visible externally but predominantly internal
Type IV- Entirely presacral

- Type I and II constitutes about 80% of cases.
- Type III and IV (hidden types) have increased rates of malignancy.


Diagnosis and Manifestations

Prenatally

- Detected via ultrasound scans.
- May present as hydrops fetalis with polyhydromnios due to fetal blood shunting to tumor due to arteriovenous malformations in the tumor.

Perinatally

- Sacrococcygeal tumor

Older Children

- Sacroccocygeal tumor
- Urinary or bowel compression symptoms


-Alpha fetoproteins are elevated but should return to normal by 9 months of age.

Currarino Triad:
- Presacral teratoma
- Anal stenosis
- Sacral defect

Prognosis

- Reported 77% survival in antenatally diagnosed SCT.
- Only 14% survival for those with hydrops.
- A study reported 100% survival in lesions less than 10 cm, 48% in more than 10 cm.


Management:

Pre-op assessment:
MRI, CT scan or ultrasonography.

Resection
- Coccyx to be removed as recurrence rate is at 37% if not removed.

If deemed unresectable:
- Biopsy
- Neoadjuvant chemotherapy (cisplatin, bleomycin)

Adzick et al. was able to resect the tumore in-utero in a patient manifesting with polyhydromnios and hydrops.

Future Topics:

- In-utero surgery
- Surgical and post-op management
- Other germ cell tumors


References:

1. Coran, Arnold G., et al., Pediatric Surgery, 7th Ed., Elsevier. 

2. Nyberg, David A., et al., Diagnostic Imaging of Fetal Anomalies, Lippincott Williams & Wilkins, 2003.