These rare embryonic remnant tumors may present anywhere in the CNS but most commonly occur in the posterior fossa and the cauda equina. They are congenital tumors and may be seen at any age; however, they are more likely to occur in children or young adults of either sex. They are slow growing and present with symptomatology specific for compressive syndromes at their location within the central nervous system. These symptoms can be insidious, and blockage of cerebrospinal flow occurs rarely. Seizures or headaches are not common. Skin lesions are commonly associated with these tumors, and any infant with a dermal sinus tract should undergo neuroradiological evaluation to exclude these growths. Communication with a dermal tract may lead to recurrent meningitis, and any patient with repeated meningitis, whether associated with a bacterial cause or not, should undergo evaluation for an unnoticed dermal sinus. Prenatal diagnosis with ultrasound and resection shortly after birth are now possible. y
Epidermoid tumors, or cholesteatomas, are composed of ectodermal remnants and present as cystic structures in lateral positions within the central nervous system, especially around the brain stem or thoracic cord. Neuroradiological imaging usually demonstrates a hyperdense structure with poor enhancement characteristics. MRI scans demonstrate low but variable signals on T1-weighted images and high but variable signals on T2 images. CT scans may demonstrate bone destruction and calcium within the lesion. These tumors have a thick ring that may be calcified and can appear "pearl-like." Epidermoids can interdigitate around vital neuronal structures, complicating surgical removal. Care should be taken during removal to prevent spilling the contents to avoid a chemical meningitis. Complete surgical removal is curative.
Dermoid tumors, composed of ectoderm and mesoderm, are more likely to be found in the midline, especially in the lumbar area and in the brain stem. Neuroradiological imaging usually shows an appearance similar to that of epidermoids with poor enhancement characteristics. These tumors are round multilobulated lesions containing fluid that also leads to a risk of chemical meningitis if the cysts are ruptured. Dermoids should be considered whenever lumbar puncture yields fat in the CSF.y Surgical removal is usually curative.
Teratomas are composed of ectoderm, mesoderm, and endoderm and are most often seen in infants and children, especially in the pineal region or the spinal canal. Because these tumors are composed of cells of all embryonic layers, they can generate hair, teeth, or other elements. Surgical removal, often not complete, is curative. Observation and an attempt at more complete re-resection may be feasible.
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