Prognostic Value of Relevant Clinicopathologic Variables in Epithelioid Sarcoma: A Multi-Institutional Retrospective Study of 44 Patients.
Prognostic Value of Relevant Clinicopathologic Variables in Epithelioid Sarcoma: A Multi-Institutional Retrospective Study of 44 Patients.
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DOI：
10.1245/s10434-014-4294-1
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发表时间：
2015-08
影响因子：
3.7
通讯作者：
Kawai A
中科院分区：
文献类型：
--
作者：
Asano N;Yoshida A;Ogura K;Kobayashi E;Susa M;Morioka H;Iwata S;Ishii T;Hiruma T;Chuman H;Kawai A
上皮样肉瘤（ES）是一种极为罕见的软组织肉瘤。最近，近端变异被报道为更具侵袭性的亚型；然而，由于大多数ES的报告涉及小病例系列，实际的预后影响尚不清楚。我们研究了ES患者的临床病理特征，以确定影响生存的预后因素。我们回顾性分析了1991年至2011年间在我院治疗的44例ES患者的临床病理特征。在这些患者中，26例经组织学诊断为经典型ES，其余18例为近端型ES。33例无远处转移的患者接受了根治性手术，其余11例有远处转移（M1）的患者接受了姑息性治疗。近端亚型与肿瘤的近端位置、远处转移、横纹肌细胞的存在、较高的肿瘤分级和血管侵犯显著相关。44例患者5年总生存率（OS）为45%。浅表肿瘤位置和出现时淋巴结转移（N1）独立预测局部无复发生存（LRFS）， N1和M1肿瘤分别独立预测远处无转移生存和OS。近端亚型与不利的LRFS和OS相关，尽管没有统计学意义。近端型ES的临床病理特征明显比经典型ES更具侵袭性，淋巴结或远处转移对预后的影响最为关键。
Epithelioid sarcoma (ES) is an extremely rare soft tissue sarcoma. Recently, the proximal variant has been reported to be a more aggressive subtype; however, as most reports of ES have involved small case series, the actual prognostic implications remain unclear. We investigated the clinicopathological features of patients with ES to identify the prognostic factors that influence survival. We retrospectively analyzed the clinicopathological features of 44 patients with ES who had been treated at our institutions between 1991 and 2011. Among these patients, 26 were diagnosed histologically as having classic-type ES, whereas the remaining 18 had proximal-type ES. Thirty-three of the patients, all without distant metastases, underwent curative surgery, and the remaining 11 with distant metastases (M1) received palliative treatment. The proximal subtype was significantly correlated with a proximal tumor location, distant metastases at presentation, presence of rhabdoid cells, a higher tumor grade, and vascular invasion. The overall survival (OS) rate at 5 years for the 44 patients was 45 %. A superficial tumor location and lymph node metastases (N1) at presentation were independently predictive of local recurrence-free survival (LRFS), and N1 and M1 tumors were independently predictive of distant metastasis-free survival and OS, respectively. The proximal subtype was associated with unfavorable LRFS and OS, although not to a statistically significant degree. Proximal-type ES has significantly more aggressive clinicopathological features than classic-type ES, and lymph node or distant metastasis has the most critical impact on prognosis.