Imaging findings of atypical terratoid/rhabdoid tumors in infant,teenager and adult

ZHANG Yuyan; HAN Wenwen; NIU Chunyan; NI Litong; QI Qi; CAO Doudou; LI Shijun

doi:10.20258/j.cnki.1006-9011.2025.03.003

Abstract：Objective To explore the imaging manifestations,pathological features and differential diagnosis of patients with primary intracranial atypical teratoid/rhabdomyosarcoma(AT/RT)at different ages,including infants,adolescents and adults,so as to improve the understanding of the disease and the clinical diagnostic compliance rate.Methods The clinical,imaging and case data confirmed by surgical pathology of five patients with AT/RT were retrospectively analyzed.Among them,one case underwent CT,three cases underwent MRI,and one case was subjected to CT and MRI.Results 3 cases were infants and chil-dren(1 female,2 males,with a mean age of 32 months),and 1 case was adolescent(male,age 12)and 1 case was adult(fe-male,age 28).The lesion was large and may be in supratentorial/subtentorial or both supratentorial and subtentorial areas,and the subtentorial area was also seen in adults;the image showed mixed density/signal,with multiple calcifications and few cystic changes and hemorrhages located at the margins;SWI bleeding in infants and children was prominent with little calcification,while the opposite was true in adolescents;MRS showed elevated Cho and Lac peaks and diminished Naa peaks;ASL showed partial hyperperfusion in infants and young children and low and moderate perfusion in adolescents.Conclusion AT/RT is most common in infants and young children,and rare in adolescents and adults.In infants and young children,the tumor is mostly located in the infratentorial area,or both supratentorial and infratentorial areas,while in adults,the tumor is usually in supratentorial or infratentorial area.The large size of the lesion,mixed density/signal,cystic degeneration and hemorrhage at the edge of the tumor are suggestive features,but diagnosis still needs to rely on histopathology.