Teratomas of the Brain and Head and Neck

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Teratomas of the Brain and Head and Neck

• Grimme JD, Camacho DLA, Spampinato MV, Castillo M
• Section of Neuroradiology
• Department of Radiology
• University of North Carolina

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• We present examples of teratomas found in the
  brain and extracranial head and neck taken from
  our teaching files, collected over a 10-year
  period. Along with the imaging findings we
  discuss important clinical and pathological
  information regarding these lesions. Because of
  the presence of cysts and calcifications, both CT
  and MRI provide critical information that helps
  in formulating a differential diagnosis.

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Histologic Classification

• Mature type composed of fully differentiated
  adult-type tissues, absent or low mitoses
• Immature type fetal-type incompletely
  differentiated tissues
• Malignant type contains cancerous tissues such
  as sarcomas, carcinomas and other embryonal
  malignancies

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Tumor Markers

• Malignant yolk sac endoderm can be an aggressive
  component of teratomas and these patients may
  have elevated levels of AFP or beta-HCG in serum
  and/or CSF
• Transcription factors GATA-4 and GATA-6 may also
  be elevated in mature and immature teratomas

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Clinical Features

• 90 of teratomas are found below 20 years of age
  (most 10-12 years)
• Male-to-female ratio 2.51
• 80 occur around region of 3rd ventricle thus
  most symptoms are due to hydrocephalus and
  increased intracranial pressure

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General Imaging Features of Teratomas

• Heterogeneous appearance
• Presence of fat, cysts (mucous-laden), calcium
  (bone and chondroid nodules), soft tissues
• Enhancing soft tissues
• Present in all types of tumors
• Enhancement of capsule
• Heterogeneous enhancement of soft tissue
  components

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Congenital Teratoma

• Intracranial teratomas are rare, accounting for
  0.5-2.0 of intracranial tumors. They comprise
  50 of congenital brain tumors (those presenting
  in the first 60 days of life).

Above Contrast enhanced CT of congenital
teratoma shows a centrally located heterogeneous
mass containing cystic/solid components and
severe hydrocephalus.
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Congenital Teratoma
Teratomas are typically benign tumors containing
elements of all 3 germinal layers ectoderm,
mesoderm and endoderm. They develop from
embryonic cells which become misinvolved during
formation of the primitive streak in the 3rd week
of life. Some of these cells become misenfolded
as intracranial rests of tissue.

• Above Axial T1 (left) and T2 (right) images of
  a congenital teratoma (arrows). The mass is
  centrally located and has a heterogeneous
  appearance. There is hydrocephalus.

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Pineal Teratoma
Tissue rests are typically found in the midline,
specifically, the pineal, suprasellar and 3rd
ventricle regions. Above Contrast enhanced CT
(left) and pre- (middle) and post-Gd (right) T1
images. There is peripheral enhancement on CT
(arrows) and mild heterogeneous enhancement
(arrows) on MRI. Ventricular air was introduced
by a ventriculostomy.
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Pineal Teratoma
Another example of pineal teratoma seen on
sagittal T1 (left), axial post-Gd T1 (middle) and
axial T2 (right) images. There is heterogeneous
signal from cystic and solid components, capsular
(arrow) and tumoral enhancement.
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Suprasellar Teratoma

• Differential diagnosis for a T1 bright and T2
  dark includes aneurysm, dermoid, lipoma and
  craniopharyngioma.
• Above Coronal T1 (left) and T2 (right) images
  of a suprasellar teratoma with considerable fatty
  contents.

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Suprasellar Teratoma
Large suprasellar mostly cystic mass (left T1
coronal, right T2 axial) initially believed to
be a craniopharyngioma but proven to be a
teratoma.
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Suprasellar Teratoma
Intracranial teratomas usually manifest in
younger children adult presentation is
unusual. Left Suprasellar teratoma in a child.
Axial FLAIR (top left) and T2 (top right) images
show bright lesion. T1 sagittal images without
(bottom left) and with (bottom right) Gd show
heterogeneous enhancement of mass.
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Suprasellar Teratoma

• Example of childhood suprasellar teratomas.
  Axial non-contrast CT (left) and sagittal T1
  image (right) demonstrate fat (arrows) in both
  lesions.

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Intraventricular Teratoma
Axial non-contrast CT (left), axial T2 (middle)
and coronal post-Gd T1 (right) images in
intraventricular teratoma. Fat, cysts and
calcifications (arrows) are present. The tumor
shows central heterogeneous enhancement. Note
associated hydrocephalus.
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Cerebellar Teratoma
Teratomas are classified by cell/tissue types as
mature or immature, and graded histologically
from 0-3, with grade increasing with amount of
immature tissues. Grade 0 only mature tissues.
Grade 3 large amounts of immature tissues.
Above Cerebellum is an unusual location for
teratoma. Axial contrast enhanced CT (left),
axial T2 (middle) and sagittal T1 (right) images
show a heterogeneous mass containing fat (arrows).
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Facial Teratoma
Differential diagnosis for a facial teratoma
includes lymphatic malformation, arteriovenous
malformation, hemangioma, neuroblastoma, and
dermoid cyst.
Above Axial T1 pre- (left) and post-Gd (right)
images show a heterogeneous cystic mass in the
region of the left parotid tail with a
heterogeneously enhancing solid component
(arrows).
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Facial Teratoma
Axial T2 (left), axial T1 (middle) and sagittal
T1 (left) images of a heterogeneous mass in the
left facial region with cystic and solid
components, which proved to be a teratoma.
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Facial Teratoma

• Head and neck teratomas commonly occur in the
  anterior midline, usually in the oropharynx or
  nasopharynx, but may also involve the orbit,
  temporal fossa, and face. Some teratomas,
  especially those arising in the nasopharynx, may
  traverse the skull base and have extensive
  intracranial extension.

Above Axial CECT of a child with a large
exophytic heterogeneous mass, which was
originating from the oropharynx. Note the
presence of fat adjacent to the coarse
calcifications (arrow). C/O Dr. Bernadette Koch
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Upper Neck Teratoma
Cervical teratomas typically present at birth as
firm ovoid masses with palpable cystic
areas. Calcifications are seen on plain
radiographs in up to 45 of teratomas.
Above Lateral radiograph (left) shows coarse
calcifications (arrow) in an anterior upper neck
teratoma in a child. Axial CT images (middle and
right) of the same patient show macroscopic fat
(arrows) in addition to the calcifications.
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Upper Neck Teratoma
Presenting symptoms of cervical teratomas include
respiratory distress, feeding difficulties and
torticollis.

• Axial CECT image of the upper neck shows a large
  complex mass on the right side with cystic
  components and heterogeneous enhancement. Note
  presence of endotracheal tube. C/O Dr.
  Bernadette Koch

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Cervical Teratoma
Above Post-contrast axial CT image (left) and
ultrasound image (right) of a cervical teratoma.
Note tracheal narrowing and deviation, and
presence of calcification seen in both studies
(arrows).
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Lower Neck Teratoma
Teratomas comprise 9 of head and neck tumors in
children.
Above Radiograph (left) and axial CT image
(right) of a teratoma arising from the region of
the thyroid gland, extending inferiorly into the
superior mediastinum. Note leftward tracheal
deviation and coarse calcifications within the
mass on the CT image (arrow).
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Conclusion

• Teratomas involving the head and neck are rare
  tumors characteristically involving the midline,
  nearly always having a heterogeneous appearance
  and often containing fat and/or calcifications.

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