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肌腱和腱膜透明细胞肉瘤:75例患者的研究

Clear cell sarcoma of tendons and aponeuroses: a study of 75 patients.

作者信息

Kawai Akira, Hosono Ako, Nakayama Robert, Matsumine Akihiko, Matsumoto Seiichi, Ueda Takafumi, Tsuchiya Hiroyuki, Beppu Yasuo, Morioka Hideo, Yabe Hiroo

机构信息

Division of Orthopedic Surgery, National Cancer Center Hospital, Tokyo, Japan.

出版信息

Cancer. 2007 Jan 1;109(1):109-16. doi: 10.1002/cncr.22380.

Abstract

BACKGROUND

Clear cell sarcoma (CCS) of tendons and aponeuroses (malignant melanoma of soft parts) is a rare melanocytic soft tissue sarcoma. The objective of this study was to determine the clinical features, prognostic factors, and optimal treatment policy for patients with this rare disease.

METHODS

Seventy-five consecutive patients with histologically confirmed CCS who received treatment between 1980 and 2004 were analyzed retrospectively.

RESULTS

There were 41 men and 34 women, and the median age was 36 years. Sixty-five tumors were located in the extremities, and 10 tumors were located in the trunk. The median tumor size was 4 cm. Seventy-one patients underwent surgical excision, and 56 patients received chemotherapy. Sixteen patients developed local recurrences, and 52 patients developed metastasis. The overall patient survival rates was 47% at 5 years and 36% at 10 years. Univariate analysis showed that sex (P = .018), tumor size (P = .001), tumor depth (P = .002), TNM classification (P = .001), and surgical margin (P = .042) were significant prognostic factors. Among the 52 patients who presented with localized disease, sex (P = .023), tumor size (P = .002), tumor depth (P = .011), TNM classification (P = .004), and chemotherapy (P = .032) were identified as significant prognostic factors. Multivariate analysis showed that tumor size remained an independent prognostic factor in both groups.

CONCLUSIONS

The current results supported the contention that early diagnosis and initial wide excision are essential for a favorable outcome of CCS. The role of chemotherapy for CCS should be investigated further.

摘要

背景

腱膜透明细胞肉瘤(软组织恶性黑色素瘤)是一种罕见的黑素细胞性软组织肉瘤。本研究的目的是确定这种罕见疾病患者的临床特征、预后因素和最佳治疗策略。

方法

回顾性分析了1980年至2004年间连续接受治疗的75例经组织学确诊的腱膜透明细胞肉瘤患者。

结果

男性41例,女性34例,中位年龄为36岁。65个肿瘤位于四肢,10个肿瘤位于躯干。肿瘤中位大小为4厘米。71例患者接受了手术切除,56例患者接受了化疗。16例患者出现局部复发,52例患者发生转移。患者5年总生存率为47%,10年为36%。单因素分析显示,性别(P = .018)、肿瘤大小(P = .001)、肿瘤深度(P = .002)、TNM分类(P = .001)和手术切缘(P = .042)是显著的预后因素。在52例表现为局限性疾病的患者中,性别(P = .023)、肿瘤大小(P = .002)、肿瘤深度(P = .011)、TNM分类(P = .004)和化疗(P = .032)被确定为显著的预后因素。多因素分析显示,肿瘤大小在两组中均为独立的预后因素。

结论

目前的结果支持以下观点,即早期诊断和初次广泛切除对于腱膜透明细胞肉瘤取得良好预后至关重要。化疗在腱膜透明细胞肉瘤中的作用应进一步研究。

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