Kikuchi Yoshinori, Nishikawa Yusuke, Amanuma Makoto, Kishimoto Yui, Takuma Kensuke, Wakayama Megumi, Shibuya Kazutoshi, Okano Naoki, Shimada Hideaki, Igarashi Yoshinori
Division of Gastroenterology and Hepatology, Department of Internal Medicine, Faculty of Medicine, Toho University, 6-11-1 Omori-nishi, Ota-ku, Tokyo, 143-8541 Japan.
Department of Surgical Pathology (Omori), Toho University, Tokyo, Japan.
Int Cancer Conf J. 2020 Oct 10;10(1):63-67. doi: 10.1007/s13691-020-00452-0. eCollection 2021 Jan.
Pancreatic leiomyosarcoma (PLMS) is an extremely rare tumor that accounts for 0.1% of pancreatic malignancies, and its chemotherapy has yet to be established. Generally, soft-tissue sarcoma chemotherapy is standard treatment with doxorubicin (DXR) alone. However, the effectiveness of gemcitabine (GEM) plus docetaxel (DOC) has been shown in uterine leiomyoma. In contrast, the GEM plus nab-paclitaxel (PTX) regimen has been established as first-line chemotherapy for unresectable pancreatic cancer. For this study, we selected the GEM plus nab-PTX regimen for patients with PLMS, achieving success in approximately 10 months. From a search on PubMed, we found only 12 cases of PLMS (including this case) that underwent chemotherapy. Our case is the first reported patient to have survived more than 2 years with chemotherapy alone. In a nude mouse model, the GEM plus DOC regimen was shown to significantly decrease tumor size when compared with DXR in leiomyosarcoma, and the GEM plus nab-PTX regimen was reported to significantly reduce necrosis when compared with DXR alone, GEM alone, DOC alone, nab-PTX alone and GEM plus DOC in soft-tissue sarcoma. GEM plus nab-PTX therapy might therefore be the first choice for soft-tissue sarcoma and leiomyosarcoma. This is the first reported case of PLMS treated with GEM plus nab-PTX.
胰腺平滑肌肉瘤（PLMS）是一种极其罕见的肿瘤，占胰腺恶性肿瘤的0.1%，其化疗方案尚未确立。一般来说，软组织肉瘤的化疗标准治疗是单独使用阿霉素（DXR）。然而，吉西他滨（GEM）联合多西他赛（DOC）在子宫平滑肌瘤中的有效性已得到证实。相比之下，GEM联合纳米白蛋白结合型紫杉醇（PTX）方案已被确立为不可切除胰腺癌的一线化疗方案。在本研究中，我们为PLMS患者选择了GEM联合纳米白蛋白结合型紫杉醇方案，取得了约10个月的成功。通过在PubMed上搜索，我们仅发现12例（包括本病例）接受化疗的PLMS病例。我们的病例是首例报道的仅通过化疗存活超过2年的患者。在裸鼠模型中，与平滑肌肉瘤中的DXR相比，GEM联合DOC方案显示可显著减小肿瘤大小，并且与单独使用DXR、单独使用GEM、单独使用DOC、单独使用纳米白蛋白结合型紫杉醇以及软组织肉瘤中的GEM联合DOC相比，GEM联合纳米白蛋白结合型紫杉醇方案据报道可显著减少坏死。因此，GEM联合纳米白蛋白结合型紫杉醇治疗可能是软组织肉瘤和平滑肌肉瘤的首选。这是首例报道的用GEM联合纳米白蛋白结合型紫杉醇治疗的PLMS病例。
