Bilski M, Surdyka D, Paśnik I, Bilska M, Cisek P, Korona P, Szumiło J, Grzybowska-Szatkowska L
Chair and Department of Oncology, Medical University of Lublin, Lublin, Poland.
Radiation Therapy Department, Lublin Oncological Center, Lublin, Poland.
Case Rep Oncol Med. 2018 Feb 11;2018:8672407. doi: 10.1155/2018/8672407. eCollection 2018.
Spleen sarcoma is one of the most rare soft tissue malignancies. The annual incidence is 0.14-0.25/1,000,000 and the average age of diagnosis is 50 to 73 years. The incidence of this cancer has been increasing. Treatment of choice is surgical splenectomy, which rarely gives good results due to the aggressive course of the disease as well as the high potential for metastasis. Overall survival in primary spleen sarcomas as described by various authors is between 4 and 14 months. 80% of patients after spleen rupture do not survive 6 months. We report the case of a 42-year-old male diagnosed with spleen angiosarcoma. The patient underwent surgery in an emergency mode because of rapid rupture of the organ. Due to positive surgical margins, he underwent adjuvant radiochemotherapy followed by chemotherapy. Overall survival time was relatively long (23 months). The international guidelines provide information based on limited data. The role of postoperative radiotherapy in angiosarcomas remains controversial. Postoperative radiotherapy may increase local disease control, especially after nonradical operation, but this does not translate into improvement in overall survival time of these patients. The case shows that adjuvant radiotherapy as part of cancer treatment strategy may prolong the overall survival.
脾肉瘤是最罕见的软组织恶性肿瘤之一。年发病率为0.14 - 0.25/100万，平均诊断年龄为50至73岁。这种癌症的发病率一直在上升。首选治疗方法是手术切除脾脏，但由于疾病进展迅速以及转移潜力高，很少能取得良好效果。不同作者描述的原发性脾肉瘤的总体生存期在4至14个月之间。80%的脾破裂患者存活时间不超过6个月。我们报告一例42岁男性被诊断为脾血管肉瘤的病例。由于器官迅速破裂，患者接受了急诊手术。由于手术切缘阳性，他接受了辅助放化疗，随后进行化疗。总体生存时间相对较长（23个月）。国际指南所依据的数据有限。术后放疗在血管肉瘤中的作用仍存在争议。术后放疗可能会提高局部疾病控制率，尤其是在非根治性手术后，但这并未转化为这些患者总体生存时间的改善。该病例表明，辅助放疗作为癌症治疗策略的一部分可能会延长总体生存期。
