Four Cases with FUS/CHOP Fusion Gene Products Positive Myxoid Liposarcoma Responding Effectively to Trabectedin
Hirohito Kirishi1, Hiromichi Yamane1, Nobuaki Ochi1
1Department of General Internal Medicine 4, Kawasaki Medical School, Okayama, Japan.
Background:
Myxoid liposarcoma, a rare type of tumor, accounts for approximately 30% of all liposarcomas. Myxoid liposarcomas harboring the FUS/CHOP fusion gene have shown promising results with trabectedin in basic research and some clinical experiments. However, the efficacy and safety of trabectedin in chemotherapy-naive soft tissue sarcomas or FUS/CHOP fusion gene-positive myxoid liposarcomas have not yet been established. Therefore, we evaluated the effectiveness and safety of trabectedin monotherapy in four cases of myxoid liposarcoma harboring the FUS/CHOP fusion gene at our hospital.
Patients And Methods:
We analyzed four patients with metastatic myxoid liposarcoma who underwent surgery at Okayama University and received chemotherapy at Kawasaki Medical School. These patients had positive test results for the FUS/CHOP fusion gene as an aid to pathological diagnosis by RT-PCR. RNA was extracted from tumor tissue sliced from frozen tumor specimens. Following reverse transcription, PCR was performed using TLS/FUS-CHOP primers. The resulting products were electrophoresed, and then the nucleotide sequences were confirmed.
Case Presentation:
Case 1: A 44-year-old male started trabectedin as second-line therapy after initial chemotherapy, which included doxorubicin. To date, he has completed 9 cycles, showing a response for 6 months. Case 2: A 71-year-old male, deemed intolerant to doxorubicin, started trabectedin as his first-line treatment. He has undergone 50 cycles to date, maintaining a response for 56 months. Case 3: A 59-year-old female began trabectedin as second-line therapy after initial chemotherapy, including doxorubicin. She responded for 6 months before experiencing disease progression. Case 4: A 79-year-old male developed new lesions after one course of initial chemotherapy, including doxorubicin. He then began trabectedin and has maintained a response for 10 months to date.
Conclusion:
Compared to other chemotherapies, trabectedin demonstrated potentially higher efficacy and a favorable safety profile for patients with myxoid liposarcoma harboring the FUS/CHOP fusion gene.
Insights
Trabectedin shows promising efficacy and safety for myxoid liposarcoma with the FUS/CHOP fusion gene. This study evaluated trabectedin monotherapy in four patients, demonstrating potential as a valuable treatment option for this rare cancer.
Area of Science:
- Oncology
- Pharmacology
Background:
- Myxoid liposarcoma is a rare soft tissue sarcoma, comprising about 30% of all liposarcomas.
- The FUS/CHOP fusion gene is a key genetic marker in a subset of myxoid liposarcomas.
- Trabectedin has shown preclinical promise for FUS/CHOP-positive myxoid liposarcomas, but clinical data remains limited.
Observation:
- This study evaluated four patients with metastatic myxoid liposarcoma harboring the FUS/CHOP fusion gene.
- Patients received trabectedin monotherapy, either as first-line or second-line treatment.
- Tumor tissue was analyzed via RT-PCR to confirm the FUS/CHOP fusion gene status.
Findings:
- Trabectedin demonstrated a favorable response in patients with FUS/CHOP-positive myxoid liposarcoma.
- One patient maintained a response for 56 months on first-line trabectedin.
- Other patients showed responses ranging from 6 to 10 months, even after prior chemotherapy.
Implications:
- Trabectedin monotherapy may offer a viable and effective treatment option for myxoid liposarcoma with the FUS/CHOP fusion gene.
- The drug's safety profile appears favorable compared to traditional chemotherapies.
- Further clinical trials are warranted to establish definitive efficacy and safety in this patient population.
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