Intracranial presentation of teratocarcinosarcoma

Teratocarcinosarcoma (TCS), an aggressive and extremely rare neoplasm, usually presents as a nasal or paranasal mass. TCS can have intracranial or dural extension from a nasal mass. We found only two instances in the literature that described this lesion as primarily manifesting as an intracranial mass while arising from a primary nasal focus. We describe a patient who had a predominantly frontal-lobe TCS without any symptoms of a primary nasal mass. The gross surgical appearance was a glistening, slimy, firm white mucoid lesion, in contrast to the friable, necrotic and tan-colored lesion usually described. We report a patient with a rare intracranial TCS, and focus on the differential diagnoses of intracranial tumors. Neuropathologists and neurosurgeons should be aware of this mode of presentation for an accurate diagnosis and a well-targeted therapeutic approach.