内容紹介
Histology-Specific Chemotherapy in Soft-Tissue Sarcomas
Summary
Soft-tissue sarcomas(STSs)are rare mesenchymal tumors, accounting for less than 1% of all adult malignancies. STSs also have diversity, with more than 50 different histological subtypes. While surgical complete resection is a definitive treatment for localized STS, chemotherapy is the treatment option for managing locally advanced and metastatic STS. Although doxorubicin±ifosfamide is still the first-line therapy for most STS subtypes, some STSs(alveolar soft part sarcoma, clear cell sarcoma, epithelioid sarcoma and extraskeletal myxoid chondrosarcoma)have been reported to have little response to these cytotoxic chemotherapies. Additionally, the treatment options after the failure of first-line therapy were very limited. The recent emergence of new therapeutic options in Japan allows us to treat patients with second-line therapy, including pazopanib, trabectedin and eribulin. However the priority and potential role of these drugs as second-line therapy are still controversial, and the outcomes with these drugs are still unsatisfactory due to the low contribution of response and survival rates. As increasing numbers of reproducible genetic alterations in STS are identified, novel molecular development has been paid attention to identify targets for the application of histology-driven therapy. We herein give an overview of histology-specific chemotherapy for STSs in Japan, highlighting first-line chemotherapy regimens, including molecular-targeted drugs other than doxorubicin-based ones, and introduce the efficacy of trabectedin and eribulin as second-line chemotherapy with consideration of liposarcoma subtypes.
要旨
軟部肉腫はいわゆる希少がんの代表である。一方で発生頻度の少ないがんでありながら,多彩な病理組織型より構成されるという特徴も持ち合わせている,極めてヘテロな集団でもある。このような軟部肉腫に対する治療開発には長年進展がみられなかった。しかしここ数年,分子標的薬をはじめとする有効な新規薬剤が複数登場した。これらの新規薬剤における個別の病理組織型に対する効果は未だに明らかではなく,専門家の間でもその適応に関しては議論がなされているのが現状である。本稿では軟部肉腫に対するドキソルビシン以外の薬剤のfirst-line治療薬としての適応,および近年承認された新規治療薬(パゾパニブ,エリブリン,トラベクテジン)における軟部肉腫に対するsecond-line以降の適応に関して述べる。
目次
Summary
Soft-tissue sarcomas(STSs)are rare mesenchymal tumors, accounting for less than 1% of all adult malignancies. STSs also have diversity, with more than 50 different histological subtypes. While surgical complete resection is a definitive treatment for localized STS, chemotherapy is the treatment option for managing locally advanced and metastatic STS. Although doxorubicin±ifosfamide is still the first-line therapy for most STS subtypes, some STSs(alveolar soft part sarcoma, clear cell sarcoma, epithelioid sarcoma and extraskeletal myxoid chondrosarcoma)have been reported to have little response to these cytotoxic chemotherapies. Additionally, the treatment options after the failure of first-line therapy were very limited. The recent emergence of new therapeutic options in Japan allows us to treat patients with second-line therapy, including pazopanib, trabectedin and eribulin. However the priority and potential role of these drugs as second-line therapy are still controversial, and the outcomes with these drugs are still unsatisfactory due to the low contribution of response and survival rates. As increasing numbers of reproducible genetic alterations in STS are identified, novel molecular development has been paid attention to identify targets for the application of histology-driven therapy. We herein give an overview of histology-specific chemotherapy for STSs in Japan, highlighting first-line chemotherapy regimens, including molecular-targeted drugs other than doxorubicin-based ones, and introduce the efficacy of trabectedin and eribulin as second-line chemotherapy with consideration of liposarcoma subtypes.
要旨
軟部肉腫はいわゆる希少がんの代表である。一方で発生頻度の少ないがんでありながら,多彩な病理組織型より構成されるという特徴も持ち合わせている,極めてヘテロな集団でもある。このような軟部肉腫に対する治療開発には長年進展がみられなかった。しかしここ数年,分子標的薬をはじめとする有効な新規薬剤が複数登場した。これらの新規薬剤における個別の病理組織型に対する効果は未だに明らかではなく,専門家の間でもその適応に関しては議論がなされているのが現状である。本稿では軟部肉腫に対するドキソルビシン以外の薬剤のfirst-line治療薬としての適応,および近年承認された新規治療薬(パゾパニブ,エリブリン,トラベクテジン)における軟部肉腫に対するsecond-line以降の適応に関して述べる。