Sasaki Masaoki, Izumi Hiroaki, Yokoyama Takaaki, Kojima Motohiro, Hosono Ako
Division of Breast and Medical Oncology, National Cancer Center Hospital East, Chiba, Japan.
Division of Psychosomatic medicine, Kinki University Hospital, Osaka, Japan.
Hematol Oncol. 2017 Dec;35(4):905-908. doi: 10.1002/hon.2364. Epub 2016 Oct 13.
Follicular dendritic cell sarcoma (FDCS) is a very rare malignant tumor derived from follicular dendritic cells. Radical resection is the standard therapy for patients with local disease, but an optimal chemotherapy regimen has not been determined for unresectable disease. We report our experience of an FDCS patient with multiorgan involvement. In the present case, disease was only located in the pancreas initially and radical resection was performed. Multiple metastasis developed after the treatment and several factors that indicated a poor prognosis were observed. The present case had a very poor prognostic disease but survived for a long time with a good performance status because of the multiple chemotherapy regimens, which follow therapeutic strategies for malignant lymphoma and soft tissue sarcoma. As far as we know, this is the first study reporting the indication of bendamustine for FDCS patients.
滤泡性树突状细胞肉瘤(FDCS)是一种非常罕见的源自滤泡性树突状细胞的恶性肿瘤。根治性切除是局部病变患者的标准治疗方法,但对于不可切除的疾病,尚未确定最佳化疗方案。我们报告了1例多器官受累的FDCS患者的治疗经验。在本病例中,疾病最初仅位于胰腺,遂行根治性切除。治疗后出现多处转移,并观察到几个提示预后不良的因素。本病例预后极差,但通过采用针对恶性淋巴瘤和软组织肉瘤的多种化疗方案,患者长期存活且生活状态良好。据我们所知,这是第一项报道苯达莫司汀对FDCS患者疗效的研究。