Catalo Miguel, Afonso Mariana, Verdelho António, Aguiar Artur, Arantes Mavilde
Radiation Oncology, Instituto Português de Oncologia do Porto Francisco Gentil, Porto, PRT.
Pathology, Instituto Português de Oncologia do Porto Francisco Gentil, Porto, PRT.
Cureus. 2024 Nov 29;16(11):e74767. doi: 10.7759/cureus.74767. eCollection 2024 Nov.
Angiosarcoma is a rare soft tissue sarcoma, namely when it presents as a primary intradural extramedullary spinal neoplasm, with only one case of non-vertebral origin reported in the literature. We present the case of a 51-year-old woman with neurological symptoms of paraparesis and constipation who underwent a magnetic resonance imaging (MRI) that revealed a well-demarcated, predominantly homogeneous, intensely enhancing intradural extramedullary lesion in T2-weighted images. Histology, immunohistochemistry, and genetics of the lesion showed an angiosarcoma. The patient received adjuvant radiation therapy (RT) and is currently stable, showing no signs of recurrence, in the third year of follow-up. Our work demonstrates a rare case of extramedullary intradural primary spinal angiosarcoma, highlighting the challenges associated with its diagnosis. The multidisciplinary approach, including surgery and adjuvant RT, has been shown to effectively control the disease. Although the prognosis is generally poor, the presented case shows apparent success.
血管肉瘤是一种罕见的软组织肉瘤,当它表现为原发性硬脊膜内髓外脊髓肿瘤时,文献中仅报道过一例非椎体起源的病例。我们报告了一例51岁女性患者,她有双下肢轻瘫和便秘的神经症状,接受了磁共振成像(MRI)检查,结果显示在T2加权图像上有一个边界清晰、主要为均匀强化的硬脊膜内髓外病变。病变的组织学、免疫组织化学和遗传学检查显示为血管肉瘤。患者接受了辅助放射治疗(RT),目前病情稳定,在随访的第三年没有复发迹象。我们的工作展示了一例罕见的硬脊膜内髓外原发性脊柱血管肉瘤病例,突出了其诊断所面临的挑战。包括手术和辅助RT在内的多学科方法已被证明能有效控制该病。尽管总体预后较差,但所报告的病例显示出明显的成功。