Rare-60. atypical rhabdoid teratoid tumor: a case in pregnancy and analysis of adult cases to date

We present the 48th case to date, the third such during pregnancy. The disease tends to follow a biphasic course i.e. if growth is not arrested following the first relapse/ progression, death is almost universal. While typically rapidly fatal, as in our case, long-term remission appears possible. Uni-variable Cox proportional-hazards models of adult cases showed significantly worse survival with: glial differentiation (HR 7.3, p<0.01), leptomeningeal dissemination (HR 4.3, p<0.01) - these were highly correlated (chi-squared <0.01) and pregnancy (HR 3.9, p=0.09). The finding that glial differentiation worsens outcomes is novel. Improved survival was seen with extent of resection (ordinal scale; p<0.01 for model) and use of craniospinal irradiation (CSI; HR 0.2, p=0.07). Systemic chemotherapy was of marginal significance (HR=0.5, p=0.13). Use of intrathecal chemotherapy was too rare to draw any conclusions. 22/46 cases originated in midline structures next to CSF, thus leading to a high rate of leptomeningeal dissemination. All 10 cases in the pituitary were in females. 3/6 cases in those aged >50 began in/beside the spinal cord. We hypothesise that the cicumventricular organs, particularly the pituitaty and pineal glands are the most common origin of these tumors. There appears to be a pool of cells dividing in the above organs, rapidly in infancy and slowly throughout much of adulthood. Review of immunohistochemistry shows ectodermal elements, with epidermal-to...
Source: Neuro-Oncology - Category: Cancer & Oncology Authors: Tags: RARE TUMORS Source Type: research