METHODS: We performed a 15-year retrospective review of our pathology database to identify cases of DDLPS in the skin or subcutaneous tissue. Diagnosis of primary sDDLPS required evidence of non-lipogenic sarcoma in the skin or subcutis, with concurrent ALT/WDLPS and/or MDM2 amplification.
RESULTS: We identified 14 cases of DDLPS involving skin or subcutis: 7 primary sDDLPS and 7 secondary lesions (3 from recurrent deep DDLPS and 4 from metastasis). Primary sDDLPS cases (4 females, 3 males; median age: 74) mainly presented as undifferentiated spindle cell or pleomorphic sarcoma. Tumor grades were grade 2 (5 cases) and grade 3 (2 cases), with three cases also showing grade 1 areas. MDM2 amplification was confirmed in 6 sDDLPSs for which FISH was successfully performed. Follow-up available for 6 sDDLPS patients showed 2 local recurrences, treated with re-excision and radiation therapy, with all disease-free at last follow-up (5-126 months). Of the 7 secondary cases, 2 had ongoing disease after multiple recurrences, 1 was disease-free, and all 4 with cutaneous metastasis died of disease (follow-up range: 24-263 months).
CONCLUSIONS: These findings emphasize the importance of distinguishing between primary sDDLPS and secondary lesions due to their distinct prognoses. Metastasis or superficial extensions from deep DDLP correlate with a considerably worse prognosis than those originating in superficial tissues.
方法:我们对我们的病理数据库进行了15年的回顾性审查,以确定皮肤或皮下组织中的DDLPS病例。原发性sDDLPS的诊断需要皮肤或皮下组织非脂源性肉瘤的证据,同时ALT/WDLPS和/或MDM2扩增。
结果:我们确定了14例涉及皮肤或皮下组织的DDLPS:7例原发性sDDLPS和7例继发性病变(3例来自复发性深DDLPS,4例来自转移)。原发性sDDLPS病例(4名女性,3名男性;中位年龄:74)主要表现为未分化的梭形细胞或多形性肉瘤。肿瘤分级为2级(5例)和3级(2例),三个案例也显示了一级地区。在6个sDDLPS中证实了MDM2扩增,已成功执行FISH。6例sDDLPS患者随访显示2例局部复发,再切除和放射治疗,在最后一次随访(5-126个月)时全部无病。在7个次要案例中,2在多次复发后患有持续疾病,1还活着,4例皮肤转移均死亡(随访24~263个月)。
结论:这些发现强调了区分原发性sDDLPS和继发性病变的重要性,因为它们的预后不同。深部DDLP的转移或浅表延伸与源自浅表组织的预后差很多。