Soft Tissue Sarcoma Update, Issue 1, 2017 (Video Program)Case discussion: A 55-year-old woman with Li-Fraumeni syndrome and a history of sarcoma is diagnosed with a high-grade, unresectable liposarcoma in the mediastinum
3:50 minutes.
TRANSCRIPTION:
DR POLLACK: We had a patient, a 55-year-old who had Li-Fraumeni syndrome. So she had a history of sarcoma. She had 2 sarcoma diagnoses as a child, or 1 as a child and 1 as an adolescent. And she had a history of receiving anthracycline-based chemotherapy as well as radiation and multiple surgeries. And at 53, she developed a 12-cm high-grade, unresectable dedifferentiated liposarcoma in her mediastinum. And basically she had had some chest discomfort, but she was still an active person. I mean, she was still able to do the things that she wanted to do. She didn’t really have shortness of breath or chest pain that was debilitating to her, but it was enough to have her get checked out. And because of her history with Li-Fraumeni syndrome, there was a low index to do imaging, and they found this tumor. DR LOVE: So when you evaluated her, what were you thinking in terms of options for her treatment? DR POLLACK: Right. So she had a biopsy. And it was actually an unusual liposarcoma in that histopathologically it had features of myxoid liposarcoma and pleomorphic liposarcoma, but there’s a test that you can do for MDM2 amplification. And that was very strong with her. And because she had that, the pathologist thought this was most consistent with a dedifferentiated liposarcoma. At the time, trabectedin hadn’t been FDA approved here in the States, but there was a program called the Expanded Access Trabectedin Trial. And trabectedin had been approved in Europe, in the UK, for a really long time. And we had this Expanded Access Trabectedin Trial. So we treated her on that. And she had 16 cycles of that, and she had stable disease on trabectedin, but she had a lot of toxicity on trabectedin. She had fatigue. She had nausea. She had some very profound cytopenias. And she was really, really tired of treatment by the time she progressed. By the time she progressed, she developed some hepatic metastases. To be honest, she was not sure she wanted to keep going with treatment, because she was just worn out from this trial of trabectedin. But we decided to treat her with eribulin. And we gave her a cycle at the standard doses of eribulin, 1.4 mg/m2. And she did have some neutropenia, but it was really — she actually didn’t have that much toxicity, but because she had had so much toxicity on the trabectedin, we just had a really low threshold for reducing her. And so she came down to the 1.1 mg/m2. I mean, she had neutropenia, so it made sense to reduce her. But I was actually happy that we did that, because she had had so much problems before. And she was on the eribulin for 12 months, and she had stable disease. And she had some regression of her liver metastasis. Not a partial response, but just a little bit of shrinkage of her liver metastasis. She did have some slight nausea the days after the eribulin infusion. She had mild leukopenia and fatigue. She had a little bit of neuropathy, and that can be a big problem with eribulin in some patients. She didn’t have severe neuropathy, but in some patients, especially patients that have had taxanes before, that’s something that you want to watch out for. But eventually she had progressive disease. And at that point, she didn’t want to keep going. She just wanted to focus on being with family and staying comfortable. And eventually she went on hospice care. |