educational purpose

2. INTRODUCTION
 By definition, teratomas include tumors
derived from all three embryonic layers:
ectoderm,endoderm, and mesoderm.
 These tumors are derived from totipotential
stem cells and are typically paraxial or mid-
line in location

4.  They may occur in both gonadal and extragonadal
locations.
 Locations and specific tumor types depend on the
age of the child.
 The common location of teratoma
 sacrococcygeal teratoma (57%) is themost common
gonadal teratoma (29%)
Other documented sites of occurrence includes the
mediastinum (7%), the retroperitoneum (4%), the
cranial cavity (3%) and the neck region (3%)

6. EPIDEMIOLOGY
 Sacrococcygeal teratoma occurs in 1 in 2-5
in 100000 live births.
 The female-to-male ratio is 4:1.
 The overall incidence of malignant germ cell
tumors is approximately 3% of all childhood
malignancies, or approximately 3 cases per
million population per year.

7. ETIOLOGICAL FACTIORS
 With sacrococcygeal teratomas, no causative
agents are known.
 With respect to ovarian germ cell tumors, a
familial predilection may be present.
 Diets high in polyunsaturated fat were associated
with the development of teratomas
 Disorders of sex development (DSDs) have been
associated with development of germ cell tumors.
 Gonadoblastoma is observed in roughly one third
of patients with DSDs.

8. ETIOLOGICAL FACTOR CONT…
Turner syndrome is similarly a risk
factor for gonadoblastoma.

9. CLASSFICATION
 Teratomas may be classified as mature or immature on
the basis of the presence of immature
neuroectodermal elements within the tumor.
Mature tumors (grade 0) : have no immature
elements.
Grade 1: tumors, immature elements are limited to one
low-power field per slide
Grade 2 : tumors, fewer than four fields are present per
slide
Grade 3: tumors, more than four fields are present per
slide

10. PATHOPHYSIOLOGY
 Several theories about the origin of these
tumors are recognized.
 The best evidence suggests that most are
due to abnormal differentiation of fetal
germ cells that arise from the fetal yolk sac.
 Normal migration of these germ cells may
cause gonadal tumors, whereas abnormal
migration produces extragonadal tumors.

11.  Teratomas are thought to arise from
totipotent primordial germ cells.
 These cells develop among the endodermal
cells of the yolk sac near the origin of the
allantois and migrate to the gonadal ridges
during weeks 4 and 5 of gestation
 Some cells may miss their target destination
and give rise to a teratoma anywhere from
the brain to the coccygeal area, usually in
the midline.

12. Commonly cited theory on the origin of teratomas. (A) Drawing of embryo during
week 4
(longitudinal section), showing primordial germ cells at the base of the yolk sac.
(B, C) During week 5, these cells migrate toward the gonadal ridges. According to
this theory, some cells could miss their intended destination

13. CLINICAL PRESENTATION
The clinical presentation of these tumors
depends on the location of the tumor.
 Sacrococcygeal teratomas may be
diagnosed prenatally as an incidental
ultrasonographic finding
 they may occur in an infant who is large for
age, premature, or has fetal hydrops

14.  A teratoma larger than 5 cm is likelyto cause
dystocia and possible rupture; elective
cesarean delivery should be performed.
 Sacrococcygeal teratomas that are not
diagnosed prenatally may be noted at
delivery, within the first few weeks after
birth, or discovered late.
 Ovarian masses typically cause abdominal
pain, mass, distention, or emesis.
 Two thirds of affected girls present with
pain as their primary symptom.

15.  In situations of acute pain, the diagnosis is often
related to torsion of the ovary with consequent
compromise of the blood supply.
 Palpable masses are less frequent and appear
later in the clinical course.
 Testicular tumors typically occur as a scrotal
mass with or without pain.
 The differential diagnosis may include hydrocele
because some cystic teratomas may
transilluminate.
 In some situations, the tumor may cause
symptomatic metastasis; this is more common in
older patients.

16.  chest pain, cough,dyspnea, or symptoms related to
recurrent pneumonitis in mediasternal teratoma
 Many patients present with respiratory findings, and
the pathognomonic finding of trichoptysis

17. INVESTIGATION
 Biopsy for histology, it specifies the classification
(mature or immature teratoma) and grade of the
tumor
 Serum tumor maker especially Beta–human chorionic
gonadotropin (β-HCG)
 CBC
 Ultrasound scan of pelvic and abdomen, testis
 Chest x ray
 Ct scan of the abdomen and pelvis for staging of tumor
at presentation
 PET scan
 Bony scan to detect metastatic disease

18. TREATMENT
 Management of teratoma depends on the stage and
location of the tumor
 General treatment of teratoma
Surgical excision of the tumor
Chemotherapy
radiotherapy

19. COMPLICATION
The complications include
 torsion (16% of ovarian teratomas),
 rupture (1%–4%),
 malignant transformation (1%–2%),
 infection (1%),
 Autoimmune hemolytic anemia (<1%)
 Hydrops fetalis
 Testicular teratoma

20. PROGNOSIS
 Mortality for congenital teratomas depends on
gestational age and the size and location of the
tumors.
 Survival of preterm infants younger
 than 30 weeks' gestation with sacrococcygeal teratoma
is only 7%, whereas the survival for infants older than
30 weeks' gestation is 75%

21. THANK YOU FOR YOUR ATTENTION…….
PREPARED AND PRESENTED BY
IDFONCE JUSTINY
TAMIKA JAPHET
MED. STUDENT AT PARADIGM COLLEGE OF
HEALTH SCIENCE