Teratoma
Figure 1: This complex pineal-region teratoma demonstrates areas of solid and cystic change and calcification on a CT image (top left) and areas of hyperintense fat signal intensity on a T1-weighted MR image (top right). Heterogeneous enhancement is a hallmark finding that illustrates the complexity of teratomas on T1WI after contrast administration (bottom).
BASIC DESCRIPTION
- Midline intracranial tumor arising from multipotential germ cells
PATHOLOGY
- Contains tissue from all three germ cell types, ectoderm, endoderm, and mesoderm
- Fat, calcification, teeth, soft tissue, sebaceum, and cysts
- Three types
- Mature: well-differentiated, WHO grade 1, often with cystic tumor component
- Immature: intermediate differentiation
- Malignant: malignant degeneration of immature teratoma, may contain somatic tumors
CLINICAL FEATURES
- Arises during fetal development as a result of aberrant formation of the primitive streak
- Mean patient age at diagnosis: 15 years; may be detected on fetal ultrasound
- Male gender predilection (4:1)
- Laboratory findings: increased serum carcinoembryonic antigen (CEA) ± α-fetoprotein
- Common presenting signs/symptoms: macrocephaly/hydrocephalus, Parinaud syndrome
- Treatment: surgical resection
- Prognosis: majority are lethal in utero or during first week of life; patients with malignant teratomas have poor 5-year survival rate (<20%)
IMAGING FEATURES
- General
- Midline intracranial mass
- Pineal region, sellar/suprasellar, basal ganglia, and spine
- Mass effect on tectum, optic chiasm, and hypothalamus common
- Contains calcifications, solid and fluid/cystic components, and fat
- Size is variable, can be large in neonates (holocranial mass)
- Midline intracranial mass
- CT
- Heterogeneous and contain very low-density fat, hyperdense calcification (teeth), intermediate-density soft tissue, and low-density cysts
- Soft tissue may enhance on contrast-enhanced CT imaging
- MRI
- T1WI: heterogeneous hyperintensity due to fatty components and calcification
- T2WI: isointense to hyperintense soft tissue, cysts/fluid; variable hyperintense peritumoral edema
- T2*GRE: hypointense signal blooming in areas of calcification
- DWI: diffusion restriction due to hypercellular solid components
- T1WI+C: soft tissue components enhance, nonenhancing fatty or calcified portions
IMAGING RECOMMENDATIONS
- MRI without and with intravenous contrast including fat-suppressed sequences; CT imaging to detect calcification
For more information, please see the corresponding chapter in Radiopaedia.
Contributor: Rachel Seltman, MD
References
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Liang L, Korogi Y, Sugahara T, et al. MRI of intracranial germ-cell tumours. Neuroradiology 2002;44:382–388. doi.org/10.1007/s00234-001-0752-0.
Liu Z, Lv X, Wang W, et al. Imaging characteristics of primary intracranial teratoma. Acta Radiol 2014;55:874–881. doi.org/10.1177/0284185113507824.
Noudel R, Vinchon M, Dhellemmes P, et al. Intracranial teratomas in children: the role and timing of surgical removal. J Neurosurg Pediatr 2008;2:331–338. doi.org/10.3171/PED.2008.2.11.331.
Osborn AG, Salzman KL, Jhaveri MD. Diagnostic Imaging (3rd ed). Elsevier, Philadelphia, PA; 2016.
Sawamura Y. WHO histological classification of tumors of the central nervous system: germ cell tumors (WHO, 1993). In Sawamura Y, Shirato H, de Tribolet N. (eds), Intracranial Germ Cell Tumors. Springer, Vienna, Austria; 1998;3–4. doi.org/10.1007/978-3-7091-6821-9_2.
Zygourakis CC, Davis JL, Kaur G, et al. Management of central nervous system teratoma. J Clin Neurosci 2015;22:98–104. doi.org/10.1016/j.jocn.2014.03.039.
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