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枢椎孤立性骨浆细胞瘤,一例罕见且具有挑战性但预后良好的病例。

Solitary bone plasmacytoma of the axis, a rare and challenging case with good outcomes.

作者信息

Zendeoui Ahmed, Nefiss Mouadh, Bousrih Anis, Tborbi Anis, Bouzidi Ramzi, Ezzaouia Khelil

机构信息

Orthopaedic department, Mongi Slim Hospital, Tunisia.

Orthopaedic department, Mongi Slim Hospital, Tunisia.

出版信息

Int J Surg Case Rep. 2024 Jan;114:109176. doi: 10.1016/j.ijscr.2023.109176. Epub 2023 Dec 20.

Abstract

INTRODUCTION

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.

CASE PRESENTATION

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.

DISCUSSION

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.

CONCLUSION

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.

摘要

引言

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有利。本病例报告聚焦于颅颈交界区(CVJ)的一种独特的孤立性骨浆细胞瘤(SBP),通过手术和放疗进行治疗。

病例介绍

一名50岁患者出现颈部疼痛和颈臂神经痛加重4个月。尽管进行了治疗和物理治疗,症状仍持续存在。临床检查发现颈部僵硬,无运动或感觉障碍。X线片和磁共振成像(MRI)显示有一个累及枢椎的病变,无不稳定迹象。CT引导下活检结果不明确。为解决不稳定问题并明确诊断,对C2后弓进行了刮除活检,随后使用髂骨移植进行枕骨至C4融合。组织学检查确诊为SBP。给予辅助放疗和化疗。在四年随访中,无多发性骨髓瘤进展,但报告颈部活动受限,影像学检查显示融合稳定。

讨论

孤立性骨浆细胞瘤主要累及中轴骨骼,累及上颈椎者罕见。诊断标准包括组织学确诊、骨髓分析正常、影像学检查无异常(原发灶除外)以及无与淋巴浆细胞增殖性疾病相关的终末器官损害。临床表现不具特异性,MRI对软组织评估有价值。放疗是主要治疗方法,手术仅用于特定指征。

结论

孤立性骨浆细胞瘤是一种罕见疾病,及时治疗预后良好。本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,必要时包括手术,对取得最佳治疗效果至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/acac/10800741/1fc70d97d463/gr1.jpg

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