脊髓样肉瘤1例。

Surgical neurology international Pub Date : 2024-11-15 eCollection Date: 2024-01-01 DOI:10.25259/SNI_640_2024
Wouter Deconinck, Sven Bamps, Thomas Steelandt, Maarten Wissels, Mark Plazier, Eric Put, Salah-Eddine Achahbar, Steven Vanvolsem, Sacha Meeuws, Sam Klein, Gert Roosen
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摘要

背景:髓系肉瘤(MS)是一种罕见的髓系外肿瘤,由髓系母细胞组成,被世界卫生组织归类为急性髓系白血病(AML)的一个亚型。孤立性、原发性或非白血病性MS的发病率为2/ 100万成人,仅占所有AML病例的0.7%。多发性硬化症的表现多种多样,很少累及脊柱。病例描述:a岁男性,表现为肩胛间疼痛,放射至右上臂/颈部,但神经系统完好。一旦确诊为孤立性脊髓MS,他接受了手术减压,随后局部照射,全身化疗和骨髓移植。然而,术后8个月,他经历了移植物抗宿主排斥反应,需要额外的治疗。结论:建立MS的诊断是具有挑战性的,通常需要组织学证实(即,骨髓母细胞和粒细胞的存在)。然而,最佳的治疗策略仍然难以捉摸;尽管放疗、化疗、骨髓移植/其他局部治疗,MS的整体长期预后仍然很差。
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A rare case of spinal myeloid sarcoma.

Background: Myeloid sarcoma (MS), a rare extramedullary tumor composed of myeloid blast cells, is classified by the World Health Organization as a subtype of acute myeloid leukemia (AML). Isolated, primary, or nonleukemic MS has an incidence of 2/1,000,000 adults and constitutes only 0.7% of all AML cases. MS presentations vary widely, with spinal involvement being rare.

Case description: A-year-old male presented with interscapular pain radiating to the right upper arm/neck but was neurologically intact. Once diagnosed with isolated spinal MS, he underwent a surgical decompression followed by local irradiation, systemic chemotherapy, and bone marrow transplantation. Eight months postoperatively, however, he experienced a graft-versus-host rejection and required additional therapies.

Conclusion: Establishing the diagnosis of MS is challenging and typically requires histological confirmation (i.e., the presence of myeloblasts and granulocytic cells). However, optimal treatment strategies remain elusive; despite radiation, chemotherapy, bone marrow transplant/other local therapies, the overall long-term prognosis for MS remains poor.

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