de Visscher Sebastiaan A H J, van Ginkel Robbert J, Wobbes Theo, Veth René P H, Ten Heuvel Suzanne E, Suurmeijer Albert J H, Hoekstra Harad J
Department of Surgical Oncology, University Medical Center Groningen and University of Groningen, Groningen, the Netherlands.
Cancer. 2006 Aug 1;107(3):606-12. doi: 10.1002/cncr.22037.
Epithelioid sarcoma is a rare soft tissue sarcoma with a known high propensity for locoregional recurrence and distant metastases. The clinical behavior and prognostic factors that influence the survival of patients with epithelioid sarcoma were studied.
Twenty-three patients, including 16 men (70%) and 7 women (30%), who were treated for epithelioid sarcoma between 1979-2003 at the University Medical Center Groningen and Radboud University Nijmegen Medical Center, were reviewed retrospectively. The median age at diagnosis was 22 years (range, 1-54 years). At the time of diagnosis, 11 patients (48%) had metastases. Six patients with distant metastasis and 1 patient with an unresectable tumor received palliative treatment (30%). The remaining 16 patients underwent surgical treatment of local disease (11 patients) or locoregional disease (5 patients). Five patients in that group received isolated limb perfusion with tumor necrosis factor and melphalan.
The 5-year and 10-year disease-free survival rates for all patients were 34% and 17%, respectively; for the 16 patients who received curative treatment, both rates were 56%. In the latter group, 8 patients developed local recurrence (50%) after a median follow-up of 4 months (range, 1-14 months). Nine patients were disease free after a median follow-up of 50 months (range, 6-290 months). Tumor size >5 cm (P < .0026) at diagnosis and local recurrence (P < .0008) were significant predictors of survival.
The prognosis for patients with epithelioid sarcoma is poor, because a substantial number of patients present with extensive disease, lymph node metastases, and/or distant metastases. Treatment consists of radical surgical excision of the tumor and, if indicated, therapeutic lymph node dissection. In patients who have large tumors, isolated limb perfusion may be useful.
上皮样肉瘤是一种罕见的软组织肉瘤，已知其局部区域复发和远处转移的倾向很高。对影响上皮样肉瘤患者生存的临床行为和预后因素进行了研究。
回顾性分析了1979年至2003年间在格罗宁根大学医学中心和拉德堡德大学奈梅亨医学中心接受上皮样肉瘤治疗的23例患者，其中男性16例（70%），女性7例（30%）。诊断时的中位年龄为22岁（范围1至54岁）。诊断时，11例患者（48%）已有转移。6例远处转移患者和1例无法切除肿瘤的患者接受了姑息治疗（30%）。其余16例患者接受了局部疾病（11例患者）或局部区域疾病（5例患者）的手术治疗。该组中有5例患者接受了肿瘤坏死因子和美法仑的隔离肢体灌注治疗。
所有患者的5年和10年无病生存率分别为34%和17%；接受根治性治疗的16例患者的这两个比率均为56%。在后一组中，8例患者在中位随访4个月（范围1至14个月）后出现局部复发（50%）。9例患者在中位随访50个月（范围6至290个月）后无疾病。诊断时肿瘤大小>5 cm（P < .0026）和局部复发（P < .0008）是生存的重要预测因素。
上皮样肉瘤患者的预后较差，因为大量患者存在广泛病变、淋巴结转移和/或远处转移。治疗包括肿瘤的根治性手术切除，如有指征，进行治疗性淋巴结清扫。对于肿瘤较大的患者，隔离肢体灌注可能有用。
