The day started out with a morning meeting in the internal medicine department. The case presented in the meeting was of a 19 year old male with Desmoplastic small round cell tumors (DSRCT). DSRCT is a soft tissue sarcoma that presents primarily in the abdominal area. It is a childhood cancer that affects mostly boys and young adults. DSRCT is associated with a chromosomal translocation (t11;22)(p13;q12) which results in an EWS/WT1 transcript. This results in an transcription activation that causes rapid tumor growth. This rare sarcoma is typically treated with a high dose of chemotherapy, but even with successful treatment, the prognosis is poor (average survival rate of 5 years).
The patient first presented in the ER with abdominal discomfort and bloating. It was discovered that in addition to ascites, he had several tumors growing in his peritoneal cavity. The patient initially refused treatment after hearing his cancer diagnosis, but eventually relented and accepted chemotherapy as he grew tired of having his ascites drained repeatedly. He was also in a lot of pain and discomfort due to the number of tumors in his abdomen. By this time, the cancer had already metastasized to the patient's liver and lungs which made it even more difficult to treat. Dr. Chen first treated him with IV cisplatin but achieved poor results with that chemotherapy. He then switched pazopanib, which is a tyrosine kinase inhibitor to help decrease tumor cell growth and tumor angiogenesis. Treatment with pazopanib proved to be successful as after one week of treatment, the tumors and ascites both decreased in size and number. Though the chemotherapy was successful, the patient still faces a grim prognosis.
Additional treatment options for DSRCT are radiation therapy, debulking surgery, hyperthermic intraperitoneal chemotherapy, and stem cell transplant. Dr. Chem explained that stem cell transplant treatments are rarely performed and are mostly targeted towards patients who are sensitive to chemotherapy or if debulking surgery is unsuccessful or cannot be done.
Dr. Chen further explained the differences in treatment systems in different countries. He said that in America, cancer patients are often treated with a single high dose chemotherapy monthly, where as in European countries and in Taiwan, patients receive lower doses of chemo on a weekly bases. Dr. Chen believes that the benefits of receiving smaller doses more frequently helps lower the risks of side effects and since the body is exposed to the drugs more frequently, it has less of a severe response to the drug toxicities. On the other hand, receiving a high dose of chemo monthly poses certain risks such as more severe side effects and possible leukopenia. I believe it would be interesting to see a study done on analyzing the prognosis of the patients on these different chemotherapy regimens as it had never been studied before.
I look forward to learning more tomorrow, but I just realized Dr. Chen never told us when/where to meet tomorrow. It shall be an interesting morning hunting him down by bothering busy nurses in our awkward Chinese.
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