背景:浆细胞瘤是一种罕见的血液系统恶性肿瘤,其预后比多发性骨髓瘤更好。该病例报告集中在颅骨交界处(CVJ)的独特孤立性骨浆细胞瘤(SBP),通过手术和放疗管理。
方法:一名50岁患者表现为4个月的颈部疼痛和颈臂神经痛恶化。尽管有治疗和物理治疗,症状持续存在。临床检查显示颈部僵硬,没有运动或感觉缺陷。X线片和MRI显示浸润轴病变,无不稳定征象。CT引导下的活检结果不确定。为了解决不稳定性并建立诊断,对C2后弓进行了刮宫活检,然后使用a骨移植物进行枕骨C4融合。组织学检查证实SBP。给予辅助放疗和化疗。在四年的随访中,没有多发性骨髓瘤的进展,但是据报道颈部活动受限,在成像上观察到稳定的融合。
结论:孤立性骨浆细胞瘤主要影响轴向骨骼,罕见的上颈椎受累。诊断标准包括组织学确认,正常骨髓分析,无异常成像(原发病灶除外),并且没有与淋巴浆细胞增生性疾病相关的终末器官损伤。临床表现是非特异性的,MRI对软组织评估有价值。放射治疗是主要的治疗方法,保留特定适应症的手术。
结论:孤立性骨浆细胞瘤是一种罕见的疾病,如果及时治疗,预后良好。该病例强调了早期诊断和治疗以预防复发或多发性骨髓瘤进展的重要性。多学科方法,包括必要时的手术,对于最佳结果至关重要。
BACKGROUND: Plasmacytoma is a rare hematological malignancy with a more favorable prognosis than multiple myeloma. This case report focuses on a unique solitary bone plasmacytoma (SBP) at the craniovertebral junction (CVJ), managed through surgery and radiotherapy.
METHODS: A 50-year-old patient presented with four months of worsening neck pain and cervicobrachial neuralgia. Despite treatment and physiotherapy, symptoms persisted. Clinical examination revealed neck stiffness, with no motor or sensory deficits. Radiographs and MRI showed an infiltrating axis lesion without instability signs. A CT-guided biopsy yielded inconclusive results. To address instability and establish a diagnosis, a curettage biopsy of the C2 posterior arch was performed, followed by occipito-C4 fusion using an iliac crest graft. Histological examination confirmed SBP. Adjuvant radiotherapy and chemotherapy were administered. At four-year follow-up, there was no multiple myeloma progression, but limited neck mobility were reported, with stable fusion observed on imaging.
CONCLUSIONS: Solitary bone plasmacytoma primarily affects the axial skeleton, with rare upper cervical spine involvement. Diagnostic criteria include histological confirmation, normal bone marrow analysis, unremarkable imaging (except for the primary lesion), and absence of end-organ damage related to lymphoplasmacytic proliferative disorders. Clinical presentation is nonspecific, and MRI is valuable for soft tissue assessment. Radiotherapy is the primary treatment, with surgery reserved for specific indications.
CONCLUSIONS: Solitary bone plasmacytoma is a rare condition with a favorable prognosis when promptly managed. This case underscores the importance of early diagnosis and treatment to prevent recurrence or multiple myeloma progression. A multidisciplinary approach, including surgery when necessary, is crucial for optimal outcomes.