内容紹介
Effective Treatment of Metastatic Rhabdomyosarcoma with Pazopanib
Summary
Pazopanib, an oral tyrosine kinase inhibitor, is the first molecular-targeted agent approved for the treatment of advanced soft tissue sarcoma(STS). Rhabdomyosarcoma in adults is rare, accounting for less than 3% of all adult STS cases. A 57-year-old woman presented with cervical lymphadenopathy. Computed tomography revealed a heterogeneous mass in the retroperitoneum, replacing the entire right kidney. On the basis of the above findings, the patient was diagnosed with alveolar rhabdomyosarcoma. She was first treated with 4 courses of vincristine, actinomycin D, and cyclophosphamide(VAC), which resulted in a partial response. Dose reduction and delay occurred owing to hematological toxicity and febrile neutropenia. As second-line chemotherapy, the patient was administered a single daily dose of 800 mg of pazopanib. Because of an episode of hand-foot syndrome and hepatic impairment, the 800-mg daily dose of pazopanib was reduced to a daily dose of 600 mg, which had to be further reduced to a daily dose of 400 mg owing to fatigue and anorexia. The patient maintained a partial response for a total of 4.3 months when treated with pazopanib. Therefore, this drug may be a new treatment option for patients showing metastatic STS after previous chemotherapy.
要旨
pazopanibは経口マルチキナーゼ阻害薬であり,悪性軟部腫瘍に対する初めての分子標的治療薬である。横紋筋肉腫(rhabdomyosarcoma: RMS)は成人での発症は極めてまれであり,予後は不良で確立した標準治療はない。症例は57歳,女性。左頸部腫脹を自覚し受診。CT検査にて右腎臓から後腹膜領域にかけて造影不均一な腫瘤影を認め,右腎針生検にて胎児型RMSと診断された。VAC療法4コース施行後,partial response(PR)が得られたが,骨髄抑制および発熱性好中球減少症のため同療法の減量および治療間隔の延長を要したため,二次療法としてpazopanib 800 mg/dayを開始した。肝機能障害,手足症候群,倦怠感および食欲不振のため400 mg/dayまで減量を要したが,薬剤導入約2.5か月後にはPRが得られ,無増悪生存期間は約4.3か月であった。
目次
Summary
Pazopanib, an oral tyrosine kinase inhibitor, is the first molecular-targeted agent approved for the treatment of advanced soft tissue sarcoma(STS). Rhabdomyosarcoma in adults is rare, accounting for less than 3% of all adult STS cases. A 57-year-old woman presented with cervical lymphadenopathy. Computed tomography revealed a heterogeneous mass in the retroperitoneum, replacing the entire right kidney. On the basis of the above findings, the patient was diagnosed with alveolar rhabdomyosarcoma. She was first treated with 4 courses of vincristine, actinomycin D, and cyclophosphamide(VAC), which resulted in a partial response. Dose reduction and delay occurred owing to hematological toxicity and febrile neutropenia. As second-line chemotherapy, the patient was administered a single daily dose of 800 mg of pazopanib. Because of an episode of hand-foot syndrome and hepatic impairment, the 800-mg daily dose of pazopanib was reduced to a daily dose of 600 mg, which had to be further reduced to a daily dose of 400 mg owing to fatigue and anorexia. The patient maintained a partial response for a total of 4.3 months when treated with pazopanib. Therefore, this drug may be a new treatment option for patients showing metastatic STS after previous chemotherapy.
要旨
pazopanibは経口マルチキナーゼ阻害薬であり,悪性軟部腫瘍に対する初めての分子標的治療薬である。横紋筋肉腫(rhabdomyosarcoma: RMS)は成人での発症は極めてまれであり,予後は不良で確立した標準治療はない。症例は57歳,女性。左頸部腫脹を自覚し受診。CT検査にて右腎臓から後腹膜領域にかけて造影不均一な腫瘤影を認め,右腎針生検にて胎児型RMSと診断された。VAC療法4コース施行後,partial response(PR)が得られたが,骨髄抑制および発熱性好中球減少症のため同療法の減量および治療間隔の延長を要したため,二次療法としてpazopanib 800 mg/dayを開始した。肝機能障害,手足症候群,倦怠感および食欲不振のため400 mg/dayまで減量を要したが,薬剤導入約2.5か月後にはPRが得られ,無増悪生存期間は約4.3か月であった。