Atypical teratoid rhabdoid tumor in an adult patient.
Tumor teratoide rabdoide atípico en un adulto joven.
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The case of a 28-year-old man is presented; he was suffering from a progressive loss of visual acuity and headache associated with polydipsia, polyuria and hypersomnia. Brain magnetic resonance spectroscopy revealed a heterogeneous mass located in the sellar/suprasellar areas having intense enhancement after contrast. A partial resection was made, pathology revealing a rhabdoid tumoral lesion accompanied by loss of INI-1 expression. Such findings were compatible with an atypical teratoid/ rhabdoid tumour which was then treated with teletherapy and ICE chemotherapy which led to a suitable response. The result following five months’ treatment pending the administration of dose-intensity chemotherapy plus peripheral-blood progenitor cells (PBPC) as hematopoietic rescue is discussed. A review of the pertinent data regarding managing this neoplasia is also presented, along with the molecular mechanisms implicated in its physiopathology.
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