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病例报告:儿童期颈椎富含巨细胞的骨肉瘤。一种需考虑的罕见实体。

Case Report: Giant cell-rich osteosarcoma of the cervical spine in the pediatric age. A rare entity to consider.

作者信息

Egea-Gámez Rosa M, Galán-Olleros María, González-Menocal Alfonso, González-Díaz Rafael

机构信息

Spine Unit. Orthopaedic Surgery and Traumatology Department, Niño Jesús University Children's Hospital, Madrid, Spain.

Orthopaedic Surgery and Traumatology Department, Infanta Elena University Hospital, Valdemoro, Spain.

出版信息

Front Surg. 2022 Oct 14;9:1001149. doi: 10.3389/fsurg.2022.1001149. eCollection 2022.

Abstract

BACKGROUND

Although osteosarcoma is the most common primary malignant bone tumor in children, its location in the axial skeleton is rare, particularly at the cervical spine. Early diagnosis, together with multidisciplinary management, improves survival rates. Safe resection and stable reconstruction are complicated by the particular anatomy of the cervical spine, which raises the risks.

CASE PRESENTATION

A 12-year-old male patient presented with cervical pain for several months and a recent weight loss of 3 kg. The complementary workup revealed a large destructive bone lesion in C7 with vertebral body collapse, subluxation, partial involvement of C6 and T1, large associated anteroposterior soft tissue components, and spinal canal narrowing. A biopsy suggested giant cell-rich osteosarcoma (GCRO). After 10 cycles of neoadjuvant chemotherapy, surgical resection was performed through a double approach: anterior, for tumoral mass resection from C6-7 vertebral bodies and reconstruction placing a mesh cage filled with iliac crest allograft plus anterior plate fixation; and posterior, for C7 complete and C6 partial posterior arch resection, thus completing a total piecemeal spondylectomy preserving the dura intact, added to a C5-T3 posterior fusion with screws and transitional rods. Postoperative chemo and radiotherapy were administered. Clinical and radiological follow-up showed disease-free survival and no neurological involvement at 3 years.

CONCLUSION

An extensive review of the literature did not find any published cases of GCRO of the cervical spine in pediatric patients. This can be explained by the combination of three peculiar conditions: its location at the cervical spine region, the young age, and the GCRO variant.

摘要

背景

骨肉瘤是儿童最常见的原发性恶性骨肿瘤,但其位于中轴骨骼的情况较为罕见,尤其是在颈椎部位。早期诊断并结合多学科管理可提高生存率。由于颈椎特殊的解剖结构,安全切除和稳定重建较为复杂,这增加了风险。

病例介绍

一名12岁男性患者出现颈部疼痛数月,近期体重减轻3 kg。辅助检查显示C7有一个巨大的破坏性骨病变,椎体塌陷、半脱位,C6和T1部分受累,伴有较大的前后软组织成分,以及椎管狭窄。活检提示富含巨细胞的骨肉瘤(GCRO)。经过10个周期的新辅助化疗后,通过双入路进行手术切除:前路,用于切除C6 - 7椎体的肿瘤块并进行重建,置入填充髂骨异体骨的网笼并加用前路钢板固定;后路,用于完全切除C7和部分切除C6后弓,从而完成整块椎体切除术,保留硬脑膜完整,再加上C5 - T3后路螺钉及过渡棒融合术。术后进行了化疗和放疗。临床和影像学随访显示3年无病生存且无神经受累。

结论

广泛查阅文献未发现小儿颈椎GCRO的任何已发表病例。这可由三种特殊情况共同解释:其位于颈椎区域、患者年龄小以及GCRO变异型。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cd95/9614060/ea22fc48c5ef/fsurg-09-1001149-g001.jpg

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