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黏液样脂肪肉瘤:43 例患者的局部复发和转移模式。

Myxoid liposarcoma: local relapse and metastatic pattern in 43 patients.

机构信息

Musculoskeletal Oncology, Department of Orthopaedic Surgery, Physical Medicine and Rehabilitation, Campus Grosshadern, University Hospital, LMU Munich, Marchioninistr. 15, 81377, Munich, Germany.

Institute of Radiology, Campus Grosshadern, University Hospital, LMU Munich, Marchioninistr. 15, 81377, Munich, Germany.

出版信息

BMC Cancer. 2018 Mar 20;18(1):304. doi: 10.1186/s12885-018-4226-8.

Abstract

BACKGROUND

Liposarcomas are the second most common type of soft tissue sarcomas, 30-50% of these are of myxoid subtype. The aim of this retrospective study was to analyze the local control rate, the metastatic pattern and survival of patients in a consecutive single-institution series.

METHODS

From 1983 to 2015, 43 patients with myxoid liposarcoma of the extremities and trunk wall underwent resections. The margin was defined as R0 (wide) or R1 (marginal). Patients were followed for evidence of local recurrence or distant metastasis. Overall and recurrence-free survival was calculated.

RESULTS

The mean age was 48.6 years. The lower extremity was involved in 40 cases, the mean tumour size was 12 cm. In 31 cases a wide and in 12 cases a marginal resection was performed. Grading was G1 in 14, G2 in 25 and G3 in 4 cases. Nine patient died in follow-up, 4 of them with metastatic disease, all nonpulmonary. 5-year local recurrence (LR) free survival was 82%. 4 (9.3%) patients developed LR (all R1). Overall survival (OS) was 81% after 5 and 72% after 10 years. In multivariate analysis age and Grading proved to be significant on OS. According to univariate analysis, only age over 48 years and distant metastasis had a significant impact on overall survival.

CONCLUSIONS

Patients with myxoid liposarcomas have a good prognosis. Myxoid liposarcoma has a distinct pattern of nonpulmonary metastatic disease. Therefore, patients with high-risk extremity myxoid liposarcoma should undergo imaging studies of the chest, abdomen, spine and pelvis as part of their staging and follow-up examinations preferably with whole body MRI, or CT scans and MRI of the spine and pelvic region for detection of suspected metastatic disease.

摘要

背景

脂肪肉瘤是第二常见的软组织肉瘤类型,其中 30-50%为黏液样亚型。本回顾性研究的目的是分析连续单机构系列中患者的局部控制率、转移模式和生存率。

方法

1983 年至 2015 年,43 例肢体和躯干壁黏液样脂肪肉瘤患者行切除术。切缘定义为 R0(广泛)或 R1(边缘)。随访患者以确定局部复发或远处转移的证据。计算总生存率和无复发生存率。

结果

平均年龄为 48.6 岁。40 例累及下肢,平均肿瘤大小为 12cm。31 例行广泛切除术,12 例行边缘切除术。分级为 G1 级 14 例,G2 级 25 例,G3 级 4 例。9 例患者在随访中死亡,其中 4 例死于转移性疾病,均非肺部。5 年局部无复发生存率为 82%。4(9.3%)例患者发生局部复发(均为 R1)。5 年和 10 年总生存率(OS)分别为 81%和 72%。多因素分析显示年龄和分级对 OS 有显著影响。根据单因素分析,只有年龄超过 48 岁和远处转移对总生存率有显著影响。

结论

黏液样脂肪肉瘤患者预后良好。黏液样脂肪肉瘤有独特的非肺部转移模式。因此,高危肢体黏液样脂肪肉瘤患者应进行胸部、腹部、脊柱和骨盆的影像学检查,作为分期和随访检查的一部分,最好采用全身 MRI 或胸部、骨盆 CT 扫描和 MRI 检测可疑的转移性疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f18c/5859402/a3da71d6e736/12885_2018_4226_Fig2_HTML.jpg

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