Teratomas are typically found in the midline or gonads. The most common locations are the sacrococcygeal region (40%), ovary (25%), and testicle (12%). Teratomas are due to abnormal differentiation of fetal germ cells and can contain tissues from all three germ layers such as hair, teeth, and bone. While most teratomas are benign (95%), some can be immature or malignant (5%) depending on the amount of immature tissue present. Sacrococcygeal teratoma is the most common congenital germ cell tumor, occurring in 1 in 35,000-40,000 births predominantly in females. Prenatal diagnosis and treatment may
2. Sacrococcygeal - 40%
Ovary - 25%
Testicle - 12%
Brain - 5%
Other (including the neck and mediastinum) - 18%
Introduction
● due to abnormal differentiation of fetal germ cells that
arise from the fetal yolk sac
● Teratomas are typically found in the midline or gonads.
3. Introduction
Teratomas have been reported to contain hair, teeth, bone
(rarely: eyes,torso,and hands, feet, or other limbs)
usually benign
95%
5%
more commonly
men
immature/malignant
women
mature/benign
4. Introduction
● Congenital (unknown: germ cells)
● nonseminomatous germ cell tumor (N.S.G.C.T.)
● abnormal development of pluripotent cells
● germ cells and embryonal cells
● ectoderm, endoderm, and mesoderm
germ cells testes or ovaries
embryonal cells Congenital
unknown
5. Mature teratoma
● grade 0 teratoma
● solid, cystic, or a combination
● skin, muscle, and bone
● generally benign
Dermoid cyst
Skin may surround a cyst and grow abundant hair
6. Dermoid cyst
● a cystic teratoma
● grows slowly and contains mature tissue
● always benign
rare malignant
in adult: squamous cell carcinoma
in infants and children: endodermal sinus tumor
7. Dermoid cyst
Ovarian dermoid cysts
● totipotential germ cells differentiated abnormally
● Complications: torsion, rupture and infection
Periorbital dermoid cysts
● in young children
● near the lateral aspect of the eyebrow
● can recur if not completely excised
9. Gonzalez-Crussi grading system
Grade 0 mature
mature (benign)
Grade 1 <10%
immature, probably benign
Grade 2 10-50%
immature, possibly malignant (cancerous)
Grade 3 >50%
frankly malignant
depends on amount of immature elements
10. Diagnosis
● Prenatal ultrasound
● Prenatal MRI
● “steal syndrome”
fetus' blood flow is redirected toward the teratoma
● AFP
Some teratomas contain yolk sac elements, which secrete AFP.
11. Introduction
The most commonly diagnosed fetal teratomas are
sacrococcygeal teratoma (Altman types I, II, and III) and
cervical (neck) teratoma.
12. Fetal Sacrococcygeal Teratoma
● the most common congenital germ cell tumor
● 1 in 35,000-40,000
● female predominance (3:1-4:1 ratio)
● prenatally diagnosed: 30-50%
● 36-41% require fetal intervention
● survival rate of prenatally diagnosed SCT is 47-83%
● 50% have long-term morbidity
obstructive uropathy, bowel and bladder incontinence
http://emedicine.medscape.com/article/2109544-overview#showall
13. Cervical teratoma
● 1:20,000-40,000
● 50% presence of calcification
● 20-40% polyhydramnios
● hyper-extended to flexed towards a side
● neonatal airway obstruction
http://radiopaedia.org/articles/congenital-cervical-teratoma
14. Fetiform teratoma
● Fetus in fetu
● highly developed mature teratomas
● retroperitoneal teratomas
Teratoma theory
Parasitic twin theory
http://en.wikipedia.org/wiki/Fetus_in_fetu
anencephalic