Radiological Image Features of the Atypical Teratoid/Rhabdoid Tumor in Adults: A Systematic Review

Atypical teratoid/rhabdoid tumor (AT/RT) occurs in children less than 3 years old, and has a very poor prognosis. AT/RT seldom occurs in adult. We have experienced four cases of AT/RT at our institute. The purpose of this study is to evaluate the radiological image findings of adult-onset AT/RT and to conduct a systematic review. Image findings of four AT/RTs in our institute were retrospectively evaluated by an experienced neuroradiologist. If the images were unavailable, image findings were evaluated from the former image interpretation report. We assembled papers of adult-onset AT/RT (n = 38) and evaluated the image findings. AT/RT occurs in a variety of sites (spinal region, pineal region, suprasellar region, jugular foramen, and so on). High density on computed tomography (CT) was seen in 10 of 11 cases; mixed intensity in T2-weighted image was seen in 13 of 18 cases; and high intensity on diffusion-weighted image (DWI) was seen in 3 of 3 cases. Contrast enhancement was observed in all cases in which images were available. We have experienced four adult-onset AT/RT cases at our institute and have evaluated image findings through systematic review. The image findings of high density on CT, high intensity on DWI, with low apparent diffusion coefficient, and a heterogenous component should lead to an inclusion of AT/RT in the differential diagnosis of a tumor; these findings may be able to suggest AT/RT; however, they cannot make the diagnosis.