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脊柱恶性纤维组织细胞瘤:13 例临床病例报告系列及 17 例文献回顾。

Malignant fibrous histiocytoma of the spine: a series of 13 clinical case reports and review of 17 published cases.

机构信息

Department of Spine Surgery, the First Affiliated Hospital of Wenzhou Medical College, Zhejiang, China.

出版信息

Spine (Phila Pa 1976). 2011 Oct 15;36(22):E1453-62. doi: 10.1097/BRS.0b013e318203e292.

Abstract

STUDY DESIGN

Retrospective case study of 13 primary malignant fibrous histiocytomas (MFH) of the spine.

OBJECTIVE

To analyze the clinic, radiologic, histologic, and prognostic features of 13 cases with the MFH of the spine.

SUMMARY OF BACKGROUND DATA

MFH, a soft tissue sarcoma, rarely occurs at the spine. Only sporadical cases have been reported in the English literature concerning the clinical and prognostic features of the primary MFH at the spine.

METHODS

Between January 1999 and December 2006, 13 cases with primary MFH of the spine were treated in the authors' spine center. Clinical history, radiographic, surgery resection, and pathologic features were recorded. The patients were followed up regarding their local recurrence and survivals. The 17 cases with primary MFH at the spine in the literature were reviewed.

RESULTS

Paraspinal or epidural mass at multiple spinal levels developed in 11 cases, with osteolytic destruction in all 13 cases. The tumor size averaged on 10.4 cm in greatest dimension. Metastases occurred in 10 of 13 cases. Compared with the 14 ± 0.60-months median survival of the debulking surgeries in seven cases, the median survival of the en bloc resection in six cases was 25 ± 6.12 months (P ∇ 0.009). The median survival was 8.7 months in 10 cases of the literature group, with 30% 1-year survival and 6.7% 2-year survival, respectively; while the median survival was 18.0 months in the authors' series, with 92.3% 1-year survival and 38.5% 2-year survival, respectively. The 5-year survival was between 25% and 69% in the extremities of MFH, but it was 28% in the head and neck and 26.7% in the abdominal cavity, compared with 7.7% in the spine in the authors' series.

CONCLUSION

The MFH of the spine tends to extensively invade paraspinal structures at multiple spinal levels, with aggressive osteolytic destruction in the vertebrae, resulting to local huge mass, radiculopathy, and myelopathy. Regardless of recent advancements in the diagnosis, treatment methods, and adjuvant therapies, for its biologically aggressive nature, it frequently recurs at the primary site and metastasizes. It has a worse prognosis than that of MFH in other sites.

摘要

研究设计

回顾性分析 13 例原发性脊柱恶性纤维组织细胞瘤(MFH)病例。

目的

分析 13 例原发性脊柱 MFH 的临床、影像学、组织学和预后特征。

背景资料总结

MFH 是一种软组织肉瘤,很少发生在脊柱。仅在英文文献中有散在病例报道了原发性脊柱 MFH 的临床和预后特征。

方法

1999 年 1 月至 2006 年 12 月,作者所在脊柱中心收治了 13 例原发性脊柱 MFH 患者。记录患者的临床病史、影像学、手术切除和病理特征。对患者进行局部复发和生存随访。对文献中 17 例原发性脊柱 MFH 进行了回顾性分析。

结果

11 例患者出现多个脊柱水平的脊柱旁或硬膜外肿块,13 例患者均有溶骨性破坏。肿瘤最大直径平均为 10.4cm。13 例中有 10 例发生转移。与 7 例减瘤手术的 14±0.60 个月中位生存时间相比,6 例整块切除的中位生存时间为 25±6.12 个月(P∇0.009)。文献组 10 例的中位生存时间为 8.7 个月,1 年生存率为 30%,2 年生存率为 6.7%;而作者所在系列的中位生存时间为 18.0 个月,1 年生存率为 92.3%,2 年生存率为 38.5%。MFH 四肢的 5 年生存率为 25%至 69%,而头颈部为 28%,腹腔为 26.7%,作者所在系列脊柱为 7.7%。

结论

脊柱 MFH 常广泛侵犯多个脊柱水平的脊柱旁结构,伴有椎体侵袭性溶骨性破坏,导致局部巨大肿块、神经根病和脊髓病。尽管在诊断、治疗方法和辅助治疗方面取得了最新进展,但由于其生物学侵袭性,肿瘤常在原发部位复发和转移。它的预后比其他部位的 MFH 更差。

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