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Washington University Experience | NEOPLASMS (EMBRYONAL) | ETMR - Embryonal Tumor Multilayered Rosettes | 6A0 Case 6 History
Case 6 History ---- The patient is a two year old male with a history of a fourth ventricular/posterior fossa mass. He initially presented in February of 2014 with balance problems and two to three days of vomiting. Up until the time of presentation, the patient was meeting developmental milestones. MR imaging performed 2/26 showed a mass centered in the fourth ventricle, resulting in complete obliteration of the fourth ventricle and severe mass effect on the brainstem. There is diffusion restriction and patchy areas of mild-to-moderate enhancement. No evidence of drop metastases in the spine was noted. The patient underwent subtotal resection on 2/28. He then began a clinical trial of chemotherapy on 3/20. The patient was continued on chemotherapy (methotrexate) until 5/27 when cytoxan/etoposide was added. In addition, carboplatin-thiotepa was completed on 5/27. MR imaging performed 9/22 showed a small amount of residual nodular enhancement along the left tentorial leaf, likely representing scar tissue. However, MR imaging performed 1/8 showed interval development of a 4.2 x 3.4 x 3.0 cm mass which was solid and cystic in the right cerebellum. The solid component is T1 isointense to slightly hypointense, and slightly T2 hyperintense. There was a 5 mm focus of enhancement in the left supralateral aspect of the mass. Additionally, there is a new non-enhancing 1.5 x 1.6 x 1.7 cm solid lesion in the superior aspect of the left cerebellar pontine angle, extending to the left ambient cistern. No evidence of drop metastases were seen in the spine. The patient then underwent a reresection on 1/9.
