Pediatric Central Nervous System Teratoma

Etiology:
— Mature type – composed of fully differentiated adult-type tissues with absent or low mitoses
— Immature type – fetal-type incompletely differentiated tissues
— Malignant type – contains cancerous tissues such as sarcomas and carcinomas and other embryonal malignancies, malignant yolk sac endoderm can be aggressive component of teratomas
Imaging:
— Characteristically involve midline, nearly always heterogenous, often contain fat
— Heterogenous appearance due to presence of fat, cysts (mucous-laden), calcium (bone and chondorid nodules), soft tissues
— Enhancing soft tissues present in all types of tumors with enhancement of capsule and heterogenous enhancement of soft tissue components
DDX:
Complications:
Treatment:
Clinical: patients with malignant teratomas may have elevated levels of AFP or beta-HCG in serum and or CSF, 90% of teratomas are found in < 20 years with most 10-12 years old,
80% occur around third ventricle and thus most symptoms are due to hydrocephalus and increased intracranial pressure

Congenital teratoma

Intracranial teratomas are rare, accounting for 0.5-2.0% of intracranial tumors
Comprise 50% of congenital brain tumors (those presenting in first 60 days of life)
Typically benign tumors containing elements of all 3 germinal layers: ectoderm, mesoderm, endoderm
— Develop from embryonic cells which become misinvolved during formation of primitive streak in 3rd week of life
— Some of these cells become misenfolded as intracranial rests of tissue

Pineal teratoma

Tissue rests typically found in midline, specifically pineal, suprasellar, 3rd ventricle regions

Suprasellar teratoma

Differential diagnosis for T1 bright and T2 dark includes aneurysm, dermoid, lipoma, craniopharyngioma

Spinal teratoma

Type I mainly external with small presacral component
Type II external presentation with large intrapelvic component
Type III external component with majority of tumor in pelvis and abdomen
Type IV entirely presacral without externally visible component
Sacrococcygeal region is most common site for teratomas in CNS
Often associated with other abnormalities including anorectal and genital malformations, ventricular septal defect, hip dislocation, vertebral anomalies (spina bifida, sacral

Radiology Cases of Central Nervous System Teratoma

Radiology Cases of Nasal Teratoma

CT of nasal teratoma — Lateral radiograph of the skull (above) shows a large nasal mass. Axial CT without contrast of the skull (below) shows a large heterogenous mass extending via a thin stalk from the unopposed nasal bones that contained soft tissue, fat and calcified components. There was no intracranial extension.

Clinical Cases of Central Nervous System Teratoma

Clinical Cases of Nasal Teratoma

Clinical image of nasal teratoma — Clinical image shows a purple mass arising from the nasal septum.

Clinical image of nasal teratoma — Clinical image shows a purple nasal mass, cystic in consistency, attached by a 2 cm stalk to the entire nasal tip.

Gross Pathology Cases of Central Nervous System Teratoma

Gross Pathology Cases of Nasal Teratoma

Pathology image of nasal teratoma — Gross pathological image shows a large, round, primarily solid mass which also had some cystic components.

Gross pathology image of nasal teratoma — Gross pathological cut image shows the irregular nature of the interior of the mass including cysts and cartilage.

Histopathology Cases of Central Nervous System Teratoma

Histopathology Cases of Nasal Teratoma

Histopathology image of nasal teratoma — Histopathological image H&E stained lower power photomicrograph shows the diagnostic presence of haphazardly arranged / ectopic endodermal (cystic spaces lined by enteric mucosa), mesodermal (island of cartilage) and ectodermal (large areas of neuroglial) elements.

Histopathology image of nasal teratoma — Histopathological image H&E stained higher power photomicrograph shows some additional types of tissue which may be seen including ciliated, respiratory type epithelium and exocrine pancreas.