Asunis Emanuela, Cini Chiara, De Robertis Mario, Griffoni Cristiana, Bandiera Stefano, Righi Alberto, Ghermandi Riccardo, Pipola Valerio, Girolami Marco, Tedesco Giuseppe, Gambarotti Marco, Gasbarrini Alessandro
Department of Spine Surgery, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy.
Department of Biomedical Sciences, Humanitas University, 20072 Pieve Emanuele, Italy.
Cancers (Basel). 2024 Jul 16;16(14):2557. doi: 10.3390/cancers16142557.
Epithelioid hemangioma is recognized by the World Health Organization as a distinct benign neoplasm; however, it is characterized by locally aggressive and rarely metastasizing behavior. Epithelioid vascular tumors are rare bony vascular lesions with varying degrees of malignant potential that remain controversial because of their rarity, unusual morphological features, and unpredictable biological behavior. The application of new molecular tools, such as massive parallel sequencing technologies, have provided new diagnostic markers and an opportunity to further refine the classification of bone vascular neoplasms. Very few cases of EH of the spine have been reported in the literature; therefore, it is difficult to make evidence-based therapeutic decisions for these patients. We report herein our experience with eleven patients suffering from EH of the spine. The study population included three males and eight females treated in our center from 2016 to the present; the average age was 44.8 years (range 14-75 years). The surgical, clinical, and radiographic data were retrospectively analyzed. The mean follow-up was 34.8 months. All patients presented lytic vertebral body lesions, six of them with pathological fracture. The majority of patients (80%) presented myelo-radicular compression. All patients were surgically treated, and preoperative embolization was performed in all cases. In light of the literature review and the clinical experience of our center, we can consider EH a locally aggressive tumor that requires surgical treatment in case of symptoms. Here, we propose a treatment algorithm that could be useful in the management of patients with this rare disease.
上皮样血管瘤被世界卫生组织认定为一种独特的良性肿瘤;然而,它具有局部侵袭性,且很少发生转移。上皮样血管肿瘤是罕见的骨血管病变,具有不同程度的恶性潜能,由于其罕见性、不寻常的形态特征和不可预测的生物学行为,一直存在争议。新分子工具的应用,如大规模平行测序技术,提供了新的诊断标志物,并为进一步完善骨血管肿瘤的分类提供了机会。文献中报道的脊柱上皮样血管瘤病例极少;因此,很难为这些患者做出基于证据的治疗决策。我们在此报告我们对11例脊柱上皮样血管瘤患者的治疗经验。研究对象包括2016年至今在我们中心接受治疗的3名男性和8名女性;平均年龄为44.8岁(范围14 - 75岁)。对手术、临床和影像学数据进行了回顾性分析。平均随访时间为34.8个月。所有患者均表现为椎体溶骨性病变,其中6例发生病理性骨折。大多数患者(80%)出现脊髓神经根受压。所有患者均接受了手术治疗,所有病例均进行了术前栓塞。根据文献综述和我们中心的临床经验,我们可以认为上皮样血管瘤是一种局部侵袭性肿瘤,出现症状时需要手术治疗。在此,我们提出一种治疗方案,可能对这种罕见疾病的患者管理有用。