9/21/2004 Case 2: Atypical teratoid/Rhabdoid tumor


Clinical History:

EMM was 4 years 11 months old when he developed a terior fossa mass that was initially resected in December, 2001 in Puerto Rico. He received craniospinal radiotherapy. One year later, he developed proptosis and a nasopharyngeal tumor with bone erosion and loss of vision in his left eye. He was seen at Children's Hospital of Philadelphia who treated him with intravenous and intrathecal chemotherapy. He developed ototoxicity but he recovered vision in his left eye. In April, 2003, he had severe Ara-C toxicity to the CNS, but he recovered. No additional intrathecal therapy was given. He continued maintenance chemotherapy. In March, 2004, right proptosis developed. The patient came to Montefiore for a second opinion. A large right orbital mass was present, with mark~ proptosis. The mass was subtotally (90%) resected.

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