Pineal teratoma
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Synonyms and keywords: Pineal teratomas; Pineal teratoblastoma; Pineal teratoid tumor; Pineal germ cell tumors; Pineal gland tumors; Brain tumor
Overview
- Pineal teratoma is an uncommon extra-axial intracranial cancer, which can have varied components and thus a wide range of appearances.[1]
- Pineal teratoma may be classified into three subtypes: mature, immature, and mature with malignant transformation.
- Mature teratomas are benign, mature, well-differentiated cystic lesions; whereas immature teratomas are poorly differentiated lesions with solid components and malignant transformation.[2]
- On other occasions, mature teratomas contain elements that undergo malignant transformation (most commonly squamous components).
- Fat
- Cystic spaces due to mucous production or other exocrine products
- Soft-tissue from any part of the body
- Calcification, including teeth
- On microscopic histopathological analysis, pineal teratoma is characterized by cells originating from at least two and usually all three embryonic layers (ectoderm, mesoderm, and endoderm). The histological subtype may not necessarily determine the biological behavior.[3]
- Pineal teratomas may be associated with elevated levels of serum alpha fetoprotein (AFP) or serum carcinoembryonic antigen (CEA).[3]
- Pineal teratoma must be differentiated from pineal lipoma, pineal dermoid, and other pineal gland tumors.[4]
- Pineal teratoma is a rare disease that tends to affect the children and young adult population.[5]
- Common complications of pineal teratoma include:[5]
- The clinical presentation of pineal teratoma is mainly from the obstructive hydrocephalus secondary to compression of the tectum of the midbrain and obstruction of the aqueduct. Symptoms of pineal teratoma include headache, vomiting, somnolence, and weakness.[5]
- Compression of the superior colliculi by pineal teratoma can lead to a characteristic gaze palsy, known as Parinaud syndrome.
- Head CT scan and brain MRI may be helpful in the diagnosis of pineal teratoma.[6] Given their extremely variable histological components, CT/MRI imaging also tends to be heterogeneous, with tumors typically demonstrating a mixture of tissue densities and signal intensity. Fat, if present, is helpful in narrowing the differential.
- On head CT scan, pineal teratoma is characterized bya mass with fat and calcification, which is usually solid / "clump-like". It usually has cystic and solid components, contributing to an irregular outline. Solid components demonstrate variable enhancement on contrast administration.[6]
- On brain MRI, pineal teratoma is characterized by:[6]
| MRI component | Findings |
|---|---|
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T1 |
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T1 with contrast |
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T2 |
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- The mainstay of therapy for immature pineal teratoma is combined radiotherapy and chemotherapy. The residual or mature component is removed surgically.[7]
References
- ↑ Intracranial teratomas. Dr Alexandra Stanislavsky and Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-teratoma. Accessed on December 10, 2015
- ↑ Teratoma. Dr Jeremy Jones and Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/teratoma. Accessed on December 10, 2015
- ↑ 3.0 3.1 Pathology of extra-axial intracranial teratoma. Dr Alexandra Stanislavsky and Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-teratoma. Accessed on December 10, 2015
- ↑ Differential diagnosis of extra-axial intracranial teratomas. Dr Alexandra Stanislavsky and Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-teratoma. Accessed on December 10, 2015
- ↑ 5.0 5.1 5.2 Clinical presentation of extra-axial intracranial teratoma. Dr Alexandra Stanislavsky and Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-teratoma. Accessed on December 10, 2015
- ↑ 6.0 6.1 6.2 Radiographic features of intracranial teratoma. Dr Alexandra Stanislavsky and Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-teratoma. Accessed on December 10, 2015
- ↑ Friedman JA, Lynch JJ, Buckner JC, Scheithauer BW, Raffel C (2001). "Management of malignant pineal germ cell tumors with residual mature teratoma". Neurosurgery. 48 (3): 518–22, discussion 522-3. PMID 11270541.