胸椎罕见的原发性去分化脂肪肉瘤:病例报告和文献综述。

IF 1.9 Q3 CLINICAL NEUROLOGY Brain & spine Pub Date : 2024-10-23 eCollection Date: 2024-01-01 DOI:10.1016/j.bas.2024.103920
Christine Kögler, Bernhard W Ullrich, Klaus D Schaser, Alexander C Disch, Gunther O Hofmann, Felix Göhre, Christian Mawrin, Matthias Vogt
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引用次数: 0

摘要

导言:脊柱原发性低分化脂肪肉瘤是一种罕见的肿瘤实体:我们报告了一例罕见的胸椎原发性低分化脂肪肉瘤病例。病例报告:一名 36 岁的女性,之前身体完全健康,在初次就诊前两天突然出现双腿升高性麻痹,且持续加重:计算机断层扫描和磁共振成像显示,肿瘤肿块从 T5 扩展到 T6,完全压迫硬膜囊和脊髓。经紧急后路减压、椎板切除术和椎管内肿瘤清除术后,患者的神经功能完全恢复。肿瘤经组织学分类为脊柱原发性2级低分化脂肪肉瘤(DDLPS),转诊至肉瘤中心后,患者接受了三个疗程的多化疗(多柔比星加伊福酰胺)。化疗后,患者在脊柱肿瘤中心的合作下接受了脊柱全切除术。随后,患者还接受了放射治疗:结果:患者仍在接受有序的肿瘤术后护理,肿瘤切除 53 个月后无肿瘤和转移:讨论和结论:DDLPS很少发生在脊柱,明确切除肿瘤是首选治疗方法。在只能进行次全切除的病例中,手术应与其他(放射)肿瘤治疗方案同时进行。此外,将脊柱原发性肉瘤患者转诊到专门的肉瘤中心也是非常必要的,这样可以为他们提供个性化的治疗方案和有序的跨学科术后护理,以确保最佳治疗效果。
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Rare primary dedifferentiated liposarcoma of the thoracic spine: A case report and literature review.

Introduction: Primary dedifferentiated liposarcomas of the spine mark a rare tumor entity.

Research question and case description: We present a rare case of a primary dedifferentiated liposarcoma of the thoracic spine. A 36-year-old previously completely healthy woman presented with a sudden ascending paresthesia of both legs, persistently increasing over the course of two days before initial presentation.

Case report: Computed tomography and magnetic resonance imaging revealed an expansively growing tumor mass extending from T5 to T6 and absolutely compressing the dural sac and spinal cord. The patient's neurological function completely recovered after emergency posterior decompression via laminectomy with intralesional tumor debulking. The tumor was histologically classified as primary grade 2 dedifferentiated liposarcoma (DDLPS) of the spine and after referral to a sarcoma center, the patient was treated with three courses of polychemotherapy (doxorubicin plus ifosfamide). Chemotherapy was followed by aggressive resection by en-bloc spondylectomy in cooperation with a spine tumor center. Subsequently, the patient also underwent radiation therapy.

Results: The patient still undergoes structured tumor aftercare and is tumor- and metastasis-free 53 months after tumor resection.

Discussion and conclusion: DDLPS rarely occur in the spine, with definitive resection of the tumor being the treatment of choice. Surgery should be accompanied by other (radio-) oncological treatment options in cases where only subtotal resection is possible. Also, referral of patients with primary sarcomas of the spine to specialized sarcoma centers is essential, so they can be provided with individual treatment options and structured interdisciplinary aftercare, that ensure the best possible outcome.

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来源期刊
Brain & spine
Brain & spine Surgery
CiteScore
1.10
自引率
0.00%
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0
审稿时长
71 days
期刊最新文献
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