Atypical Teratoid Rhabdoid Tumor: Two Case Reports and an Analysis of Adult Cases with Implications for Pathophysiology and Treatment.

摘要: We present the first quantitative analysis of atypical teratoid rhabdoid tumors (ATRT) in adults, including two patients from our own institutions. These are interest as one occurred during pregnancy and is a long-term survivor. Our review pathological findings 50 reported cases adult ATRT leads us to propose solely ectodermal origin for tumor that epithelial-mesenchymal transition (EMT) defining feature. Thus, term may be misleading. clinical shows tends originate mid-line structures adjacent CSF, leading high rate leptomeningeal dissemination. we hypothesize residual undifferentiated ectoderm circumventricular organs, particularly pituitary pineal glands, most common these tumors. 13 note if growth not arrested soon after diagnosis, or relapse/ progression, death almost universal. While typically rapidly fatal (as case), remission possible second). Significant predictors prognosis were extent resection use chemotherapy. Glial differentiation (GFAP staining) was strongly associated with metastases (chi-squared p=0.02) markedly worse outcomes. Clinical trials adults rare. primarily disease infancy radiotherapy generally avoided those aged less than three years old. Treatment options differ infants cranio-spinal irradiation viable adjunct systemic chemotherapy population. Given grave prognosis, this combined approach appears reasonable. As effective likely cause myelosuppression, recommend stem-cell rescue available locally.