Masuda Toshiro, Beppu Toru, Nagayama Yasunori, Miyamoto Hideaki, Komohara Yoshihiro, Oda Eri, Karashima Ryuichi, Nakagaki Takashi, Adachi Yuki, Ishiko Takatoshi
Department of Surgery, Yamaga City Medical Center, Kumamoto, Japan.
Department of Surgery, Yamaga City Medical Center, Kumamoto, Japan;
Anticancer Res. 2025 Apr;45(4):1777-1784. doi: 10.21873/anticanres.17557.
BACKGROUND/AIM: Splenic angiosarcoma is a rare and serious malignancy. We investigated the diagnosis and the suitable treatment strategy for metastatic splenic angiosarcoma with the Kasabach-Merritt phenomenon.
A 76-year-old man was referred because of abdominal discomfort together with anemia, thrombocytopenia, and disseminated intravascular coagulopathy. Contrast-enhanced computed tomography revealed some huge, low-attenuation large splenic tumors and numerous liver tumors. Magnetic resonance imaging showed tumors with low and heterogeneous high signal intensity on T1-weighted and T2-weighted images, respectively. Several splenic tumors contained peripheral hypointense areas on T2WI that demonstrated a signal drop in in-phase compared to opposed-phase chemical-shift T1WI. A percutaneous needle biopsy of liver tumors with needle-tract radiofrequency ablation was performed without bleeding. The patient was pathologically diagnosed as splenic angiosarcoma by several immunohistochemical analyses. We started anticoagulant-fibrinolytic therapy with nafamostat mesylate; however, partial ischemia of the jejunum happened twice, and partial resections and the creation of an ileostomy were performed. The patient has been relatively stable for 3 months with paclitaxel monotherapy followed by oral pazopanib. Kasabach-Merritt phenomenon has been stable during good control of splenic angiosarcoma.
Metastatic splenic angiosarcoma with the Kasabach-Merritt phenomenon might be controlled by accurate diagnosis, precise surgical intervention for digestive organ ischemia, and effective chemotherapy. The Kasabach-Merritt phenomenon is an effective indicator of chemotherapy response. Furthermore, the development and clinical application of new chemotherapies are needed.
背景/目的：脾血管肉瘤是一种罕见且严重的恶性肿瘤。我们研究了伴有卡萨巴赫-梅里特现象的转移性脾血管肉瘤的诊断及合适的治疗策略。
一名76岁男性因腹部不适伴贫血、血小板减少和弥散性血管内凝血而前来就诊。增强计算机断层扫描显示脾脏有一些巨大的低密度大肿瘤以及众多肝脏肿瘤。磁共振成像显示肿瘤在T1加权像上呈低信号，在T2加权像上呈高信号且不均匀。几个脾脏肿瘤在T2加权像上周边呈低信号区，与反相位化学位移T1加权像相比，同相位时信号下降。对肝脏肿瘤进行了经皮穿刺活检并在穿刺道进行了射频消融，未发生出血。通过多项免疫组织化学分析，患者被病理诊断为脾血管肉瘤。我们开始使用甲磺酸萘莫司他进行抗凝-纤溶治疗；然而，空肠发生了两次局部缺血，进行了部分切除并造了回肠造口术。患者接受紫杉醇单药治疗，随后口服帕唑帕尼，病情相对稳定了3个月。在脾血管肉瘤得到良好控制期间，卡萨巴赫-梅里特现象一直稳定。
伴有卡萨巴赫-梅里特现象的转移性脾血管肉瘤可通过准确诊断、针对消化器官缺血的精确手术干预以及有效的化疗得到控制。卡萨巴赫-梅里特现象是化疗反应的有效指标。此外，需要开发新的化疗药物并将其应用于临床。
