extra gonadal tumors germcell, germ cell migration

1. SACCROCOCCYGEAL TERATOMA
DR PRANAYA
PAEDIATRIC SURGERY

2. Teratomas are germ cell tumors commonly
composed of multiple cell types derived from
one or more of the 3 germ layers. Teratomas
range from benign, well-differentiated
(mature) cystic lesions to those that are solid
and malignant (immature)

3. Primordial germ cells arise near
the allantois of the embryonic
yolk sac endoderm and are evident
at the fourth fetal week.

4. Migrate Along The Midline Dorsal Mesentery To The Genital
Ridge, Arriving By The End Of The Sixth Fetal Week.

5. Mediated by the c-KIT receptor and stem cell
factor
• Arrested migration - extragonadal locations in
the normal path of the germ cells –
retroperitoneum
• Aberrant migration results in cells at other
extragonadal sites - pineal, sacrococcygeal

6. In adults - 90% of germ cell tumors are
at gonadal locations.
• Abnormal or arrested migration of primordial
germ cells results in deposition in
sacrococcygeal region, retroperitoneum,
mediastinum, and pineal gland of the brain –
EXTRAGONADAL GERM CELL TUMORS.

7. • Majority tumor are benign and extragonadal.
• Yolk sac tumor is the predominant malignant histology.
• Serum marker (alpha fetoprotein, AFP) exists to follow
response to therapy and monitor for recurrent disease.
• Cisplatin and Bleomycin responsive t/t

8. SACCROCOCCYGEAL TERATOMA

9. Sacrococcygeal teratomas [SCT]
are the M/C extragonadal tumor in neonates,
[70% of all teratomas in childhood]
MORE IN FEMALE BABIES.

10. PRESENTATION-
Neonates with large
predominantly external
lesions, which are detected in
utero or at birth
older infants and children who
present with primarily hidden
pelvic tumors with a much
higher rate of malignancy
• In-utero shunting can lead to fetal hydrops,
which is associated with high mortality.

11. Altman Types- Type I (46.7%)- External Predominantly.
Type II (34%)- External With Intrapelvic Extension.
Type III (8.8%) Visible Externally But Predominantly Pelvic And Abdominal
Type IV (9.8%) Entirely Presacral.
Malignancy Rate Increased With The More Hidden (Type
III And IV) Lesions

12. D/D
• Meningomyelocele
• Infantile Hamartoma
• Dermoid
• Osteomyelitis Of
Sacrum
• Neuroenteric Cyst
• Currarino Triad-
Presacral teratoma
Anal Stenosis
Sacral Defects

13. • Ultrasonography
• CT
• Magnetic resonance imaging (MRI)

14. Surgical Mx
• Removal of the coccyx is an essential step,
because 37% recurrence if left.

17. Surgical steps Highlights
• chevron incision
• gluteus maximus dissection & sacrum and coccyx
identification
• En bloc
removal of
tumor
with coccygectomy

18. • Pelvic floor reconstruction
• Skin closure

19. Followup
• malignant germ-cell tumors can recur either
incomplete resection or from malignant
conversion of benign residual tissue.
• Chemotherapy if malignant histology
• AFP monitoring

20. • AFP levels- produced by embryonic yolk sac
and the fetal liver
• normally elevated in 1mnth of age, return to
normal by 9 months age.[less than 10 ng/mL]
• Goal of any treatment is to return AFP to
normal levels. Tumor recurrence - sudden
elevation of the AFP level.
• Half Life- 5-7days

21. Thank you