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Medulloblastoma Case Study

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4.3 Medulloblastoma- Group 3/Treatment
Group 3 accounts for 28% of all patients diagnosed with medulloblastoma. Unfortunately, these patients face the worst outlook in comparison to their SHH, WNT, and Group 4 counterparts, which are in part due to the high rate of metastasis in this subtype [41]. Group 3 tumors are most frequently found in infants and children with very few adults and older individuals. Molecular characterization of these tumors demonstrates many of these patients display MYC amplification with an associated poor overall prognosis. Furthermore, many of these patients have an overexpression of OTX2. There has been a correlation found between MYC over-expression and OTX2. More precisely, OTX2 overexpression has been thought to contribute to the over-expression of MYC [41, 44] This known interaction serves as a potential therapeutic target in these patients, but at present, very few treatment specific …show more content…

The overall prognosis of this tumor is not optimistic with mean survival sitting between 6 and 11 months [52]. This diagnosis is most often associated with infants and young children. Given the significant neurologic complications of radiation to the developing brain, physicians are often hesitant to radiate. Furthermore, the efficacy of chemotherapy has been historically difficult to quantify given a wide variety of regimens used with few patients enrolled in each study [53].
Historically, rhabdoid tumors have been difficult to diagnose given their heterogeneous nature. When using histological features alone, these tumors would often times be diagnosed as medulloblastoma or PNET [54]. Now, these tumors must be positive for mutations in INI1 or BRG1 in order to be diagnosed as an AT/RT [8]. This creates a much more objective standard for diagnosing these tumors, which in turn will lead to more homogenous groups of patients to better inform treatment and

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