Chaney A W, Pollack A, McNeese M D, Zagars G K, Pisters P W, Pollock R E, Hunt K K
Division of Radiation Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas 77030, USA.
Cancer. 2000 Oct 1;89(7):1502-11. doi: 10.1002/1097-0142(20001001)89:7<1502::aid-cncr13>3.0.co;2-p.
Cystosarcoma phyllodes is a rare sarcoma of the breast. Although surgical removal is the mainstay of treatment, the extent of surgery required (excision vs. mastectomy) and the need for additional local therapy, such as radiotherapy, are unclear. The current study evaluated the rate of local and distant failure, as well as potential prognostic factors, to better define appropriate treatment strategies.
One hundred one patients treated primarily for cystosarcoma phyllodes of the breast were evaluated. These tumors were classified histologically into benign (58%), indeterminate (12%), and malignant (30%) based on well defined criteria. Stromal overgrowth (29%) was considered separately. Surgery was comprised of local excision with breast conservation (47%) or mastectomy (53%). Microscopic surgical margins were negative in 99% of cases. Six patients received adjuvant radiotherapy.
Overall survival for the 101 patients was 88%, 79%, and 62% at 5, 10, and 15 years, respectively. For patients with nonmalignant (benign or indeterminate) and malignant cystosarcoma phyllodes, the overall survival was 91% and 82%, respectively, at 5 years, and 79% and 42%, respectively, at 10 years. Similar rates were observed based on the presence or absence of stromal overgrowth. Local recurrence occurred in 4 patients, with an actuarial 10-year rate of 8%. Eight patients developed distant metastases, with an actuarial 10-year rate of 13%. Multivariate analysis using Cox proportional hazards regression revealed stromal overgrowth to be the only independent predictor of distant failure.
Local failure in this group of largely margin negative patients with cystosarcoma phyllodes of the breast was low, showing that breast-conserving surgery with appropriate margins is the preferred primary therapy. The current study data do not support the use of adjuvant radiotherapy for patients with adequately resected disease. Patients with stromal overgrowth, particularly when the tumor size was > 5 cm, were found to have a high rate of distant failure; such patients merit consideration of a trial that examines the efficacy of systemic therapy.
叶状囊肉瘤是一种罕见的乳腺肉瘤。虽然手术切除是主要的治疗方法，但所需的手术范围（切除还是乳房切除术）以及是否需要额外的局部治疗，如放疗，尚不清楚。本研究评估了局部和远处复发率以及潜在的预后因素，以更好地确定合适的治疗策略。
对101例主要接受乳腺叶状囊肉瘤治疗的患者进行评估。根据明确的标准，这些肿瘤在组织学上分为良性（58%）、不确定（12%）和恶性（30%）。单独考虑间质过度生长（29%）情况。手术包括保乳局部切除（47%）或乳房切除术（53%）。99%的病例显微镜下手术切缘阴性。6例患者接受了辅助放疗。
101例患者的5年、10年和15年总生存率分别为88%、79%和62%。非恶性（良性或不确定）和恶性叶状囊肉瘤患者的5年总生存率分别为91%和82%，10年分别为79%和42%。根据间质过度生长情况观察到类似的比率。4例患者发生局部复发，10年精算复发率为8%。8例患者发生远处转移，10年精算转移率为13%。使用Cox比例风险回归进行多变量分析显示，间质过度生长是远处复发的唯一独立预测因素。
在这组大部分切缘阴性的乳腺叶状囊肉瘤患者中，局部复发率较低，表明具有合适切缘的保乳手术是首选的主要治疗方法。本研究数据不支持对手术切除充分的患者使用辅助放疗。发现间质过度生长的患者，特别是肿瘤大小>5 cm时，远处复发率较高；这类患者值得考虑进行一项研究全身治疗疗效的试验。
