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外周部位非典型性脂肪肿瘤和去分化脂肪肉瘤患者的临床转归。

Clinical outcome in patients with peripherally-sited atypical lipomatous tumours and dedifferentiated liposarcoma.

机构信息

Department of HistopathologyRoyal National Orthopaedic Hospital NHS TrustStanmoreMiddlesexUK; Department(s) of HistopathologyRadiologySurgery and OncologyLondon Sarcoma ServiceRoyal National Orthopaedic Hospital NHS Trust and University College London Hospitals Foundation TrustLondonUK.

Department of HistopathologyRoyal National Orthopaedic Hospital NHS TrustStanmoreMiddlesexUK; Department(s) of HistopathologyRadiologySurgery and OncologyLondon Sarcoma ServiceRoyal National Orthopaedic Hospital NHS Trust and University College London Hospitals Foundation TrustLondonUK; Research Department of PathologyUCL Cancer InstituteHuntley StreetLondonUK.

出版信息

J Pathol Clin Res. 2015 Mar 16;1(2):106-12. doi: 10.1002/cjp2.12. eCollection 2015 Apr.

Abstract

The reported incidence of local recurrence of peripheral atypical lipomatous tumours is highly variable and is likely to reflect the different inclusion criteria of cases, and the design of previous studies. We aimed to study the incidence of local recurrence of 90 cases of atypical lipomatous tumours and an additional 18 cases of de novo dedifferentiated liposarcoma. All tumours were diagnosed on the basis of MDM2 amplification: all patients had their first treatment in the same specialist sarcoma unit and were followed for a minimum of 60 months. The tumours were diagnosed between 1997 and 2009 and followed until the end of 2014. Seventy cases (78%) of atypical lipomatous tumours were located in the thigh (mean size 195 mm on presentation). Eight atypical lipomatous tumours (8.9%) recurred locally, of which 50% recurred after 60 months. The only two tumours with intralesional excisions recurred. Seven of the eight recurrent tumours were detected by the patient by self-examination. One case recurred a second time as a dedifferentiated liposarcoma. Seventeen per cent of the de novo dedifferentiated liposarcomas recurred within 60 months of presentation. Extending the study period revealed that atypical lipomatous tumour could recur up to 40 years after the first surgery. Furthermore, of 26 tumours that recurred in the extended study, 27% recurred more than once, and three of the seven that recurred more than once transformed into a dedifferentiated liposarcoma. We recommend that, following post-operative wound care, patients with atypical lipomatous tumour are referred back to their general practitioner for follow up, but that in the event of a suspected recurrence they have rapid access back to the specialist unit using a 'supported discharge' scheme. In the event of an intralesional excision and if a lesion recurs, patients are followed in a specialist unit at regular intervals: whether MRI scanning is a valuable means of monitoring such patients is unclear and requires an evidence base.

摘要

外周性非典型脂肪肉瘤局部复发的报道发生率差异很大,这可能反映了病例的不同纳入标准和既往研究的设计。我们旨在研究 90 例非典型脂肪肉瘤和另外 18 例新发去分化脂肪肉瘤的局部复发发生率。所有肿瘤均基于 MDM2 扩增诊断:所有患者均在同一肉瘤专科治疗单位接受首次治疗,并至少随访 60 个月。肿瘤诊断时间为 1997 年至 2009 年,随访至 2014 年底。70 例(78%)非典型脂肪肉瘤位于大腿(初次就诊时的平均大小为 195mm)。8 例非典型脂肪肉瘤局部复发,其中 50%在 60 个月后复发。仅 2 例接受肿瘤内切除术的肿瘤复发。8 例复发肿瘤中有 7 例为患者自检发现。1 例复发为去分化脂肪肉瘤。17%的新发去分化脂肪肉瘤在初次就诊后 60 个月内复发。延长研究时间后发现,非典型脂肪肉瘤在首次手术后 40 年内仍可能复发。此外,在延长研究中复发的 26 例肿瘤中,27%复发不止一次,7 例复发不止一次中有 3 例转化为去分化脂肪肉瘤。我们建议,在术后伤口护理后,将非典型脂肪肉瘤患者转介给他们的全科医生进行随访,但如果怀疑复发,应通过“支持性出院”计划迅速转回专科治疗单位。如果进行肿瘤内切除术且肿瘤复发,患者应在专科治疗单位定期随访:MRI 扫描是否是监测此类患者的有价值手段尚不清楚,需要循证医学证据。

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