Mulleman Denis, Flipo René-Marc, Assaker Richard, Maurage Claude-Alain, Chastanet Patrick, Ducoulombier Vincent, Deprez Xavier, Duquesnoy Bernard
Department of Rheumatology, Lille Teaching Hospital, Hôpital Roger Salengro, France.
Eur Spine J. 2004 May;13(3):244-8. doi: 10.1007/s00586-003-0589-9. Epub 2004 Mar 16.
Localized primary amyloidosis is a disease characterized by a single tumor and localized amyloid deposit (amyloidoma) with no evidence of generalized amyloidosis. The occurrence of an amyloidoma in the spine is rare and only three cases affecting the axis have been previously reported. We describe the case of a 79-year-old woman presenting with a mass involving the odontoid process, responsible for an acute tetraparesia. Diagnosis of local primary amyloidosis was made after surgical excision.
Despite the critical presentation, outcome was excellent after total excision of the mass. This case can be classified as a primary localized amyloidoma. The patient did not exhibit any infection, tumor or inflammatory disease, and continued investigations failed to demonstrate other amyloid deposit after one-year follow-up.
Amyloidoma must be discussed in presence of a tumor-like mass of the odontoid process and may be responsible, as in our case, for spinal cord compression.
局限性原发性淀粉样变性是一种以单个肿瘤和局限性淀粉样沉积物(淀粉样瘤)为特征的疾病,无全身性淀粉样变性的证据。脊柱淀粉样瘤的发生罕见,此前仅报道过3例累及枢椎的病例。我们描述了一名79岁女性的病例,该患者出现累及齿状突的肿块,导致急性四肢轻瘫。手术切除后诊断为局限性原发性淀粉样变性。
尽管病情危急,但肿块完全切除后预后良好。该病例可归类为原发性局限性淀粉样瘤。患者未表现出任何感染、肿瘤或炎症性疾病,一年随访期间的持续检查未发现其他淀粉样沉积物。
当出现齿状突类似肿瘤的肿块时,必须考虑淀粉样瘤,如我们的病例所示,它可能导致脊髓受压。