Sereno María, Merino María, Aguayo Cristina, Hernández Susana, Gutiérrez-Gutiérrez Gerardo, Zambrana Tévar Francisco, López-Gómez Miriam, Gómez Raposo César, Casado-Sáenz Enrique
Oncology Department, Infanta Sofía University Hospital, Madrid, Spain.
Tumori. 2013 Mar-Apr;99(2):e43-8. doi: 10.1177/030089161309900227.
Malignant fibrous histiocytoma is an aggressive tumor, the most common soft-tissue sarcoma of adult age. It is usually located in the extremities and retroperitoneum, and very rarely there is skeletal involvement. Surgery is the preferred treatment in early disease; in advanced disease, chemotherapy is the main therapeutic strategy. We present a 25-year-old female patient diagnosed with a vertebral mass in T5 with a severely compromised spinal cord. She underwent surgical decompression and the pathological findings were consistent with malignant fibrous histiocytoma. After several surgical treatments she had pulmonary progression and was therefore started on chemotherapy. She had a very poor response to most of the administered regimens until she initiated trabectedin 1 mg/m 2 every three weeks. She showed a significant improvement with a major response of the lung metastases. This report indicates that trabectedin is an active drug in advanced, previously treated metastatic malignant fibrous histiocytoma.
恶性纤维组织细胞瘤是一种侵袭性肿瘤,是成年期最常见的软组织肉瘤。它通常位于四肢和腹膜后,很少累及骨骼。早期疾病首选手术治疗;晚期疾病则以化疗为主要治疗策略。我们报告一例25岁女性患者,诊断为T5椎体肿物伴严重脊髓受压。她接受了手术减压,病理结果符合恶性纤维组织细胞瘤。经过多次手术治疗后,她出现了肺部进展,因此开始化疗。在开始使用曲贝替定每三周1mg/m²之前,她对大多数给药方案反应很差。她的肺转移灶出现显著改善,有明显疗效。本报告表明,曲贝替定在晚期、先前接受过治疗的转移性恶性纤维组织细胞瘤中是一种有效的药物。