Fiore Marco, Grosso Federica, Lo Vullo Salvatore, Pennacchioli Elisabetta, Stacchiotti Silvia, Ferrari Andrea, Collini Paola, Lozza Laura, Mariani Luigi, Casali Paolo G, Gronchi Alessandro
Department of Surgery, National Cancer Institute, Milan, Italy.
Cancer. 2007 Jun 15;109(12):2522-31. doi: 10.1002/cncr.22720.
The objective of this study was to investigate prognostic factors and clinical outcome of myxoid/round cell and pleomorphic liposarcoma.
Three hundred twenty-nine patients with localized myxoid/round cell or pleomorphic liposarcoma who underwent surgery at the Istituto Nazionale per lo Studio e la Cura dei Tumori (Milan, Italy) over 25 years were reviewed. The rates of local recurrence, distant metastases, and survival were studied.
Two hundred fourteen patients presented with primary disease, and 115 patients had locally recurrent tumors. The disease-specific survival rate was 75% at 10 years, and the local recurrence and distant metastases incidence were 25% and 15%, respectively. Presentation with recurrent disease, tumor size (>10 cm), tumor grade (French Federation of Cancer Centers grade II or III vs grade I), and positive surgical margins were independent predictors of death. Tumor site and radiation therapy also played a role, mostly related to their effect on local outcome. Pathologic grade and histologic subtype influenced distant metastases. Extrapulmonary metastases were associated with poorer postmetastatic disease-specific survival.
Myxoid/round cell liposarcomas shared similar prognostic factors with other soft tissue sarcomas and had a relatively good clinical outcome. The presence of >5% of round cell component singled out a group of patients at greater risk of metastases and death but with a broad spectrum of disease aggressiveness. Extrapulmonary metastases were a peculiar pattern of myxoid/round cell liposarcoma that require special consideration for treatment and prognosis.
本研究的目的是调查黏液样/圆形细胞和多形性脂肪肉瘤的预后因素及临床结局。
回顾了25年间在意大利米兰国立肿瘤研究所接受手术的329例局限性黏液样/圆形细胞或多形性脂肪肉瘤患者。研究了局部复发率、远处转移率和生存率。
214例患者为原发性疾病，115例患者有局部复发性肿瘤。10年疾病特异性生存率为75%，局部复发率和远处转移率分别为25%和15%。复发性疾病、肿瘤大小（>10 cm）、肿瘤分级（法国癌症中心联合会II级或III级与I级）以及手术切缘阳性是死亡的独立预测因素。肿瘤部位和放疗也起作用，主要与其对局部结局的影响有关。病理分级和组织学亚型影响远处转移。肺外转移与转移后较差的疾病特异性生存率相关。
黏液样/圆形细胞脂肪肉瘤与其他软组织肉瘤具有相似的预后因素，临床结局相对较好。圆形细胞成分>5%的患者转移和死亡风险更高，但疾病侵袭性范围较广。肺外转移是黏液样/圆形细胞脂肪肉瘤的一种特殊模式，在治疗和预后方面需要特别考虑。
