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排除胃肠道间质瘤的原发性局限性消化道高级别肉瘤患者:来自法国肉瘤研究组的一项回顾性研究

Patients with primary localized high-grade sarcomas of the digestive tract excluding GIST : a retrospective study from the French sarcoma group.

作者信息

de Nonneville A, Toullec C, Blay J Y, Ranchere D, Stoeckle P E, Italiano A, Bonvalot S, Terrier A P, Duffaud F, Bertucci F, Cupissol D, Isambert N, Piperno Neumann S, Coindre J M, Salas S

机构信息

APHM, Hôpital de la Timone, Department of Medical Oncology, Marseille, France.

Aix Marseille Univ, Marseille, France.

出版信息

Acta Gastroenterol Belg. 2017 Oct-Dec;80(4):481-486.

Abstract

INTRODUCTION

The natural history of localized high-grade sarcomas of the digestive tract (SDT) excluding GIST has been rarely considered owing to their low incidence and heterogeneity. We describe the histoclinical characteristics of SDT and correlate them with patients' outcomes.

METHODS

We retrospectively collected medical files from a European database covering connective tissue tumors listed in Europe for about twenty years. Only untreated localized primary high-grade SDT were included. A central histological review was performed for each case. Patients' characteristics were compared and correlated with clinical outcomes.

RESULTS

A total of 45 patients were identified. Leiomyosarcomas (LMS) and undifferentiated sarcomas (UDS) were predominant, the former having better overall survival (OS) and progressionfree survival (PFS) while the latter having a worse outcome than the other histological types. Complete remission was obtained in 34 patients (75%) and was associated with male sex, age over 40 years and monofocal tumor. Complete surgery and LMS histology were associated with a better prognosis without any significant difference in baseline characteristics or in treatment modalities.

CONCLUSION

Complete surgery and histological type seem to be prognostic indicators of SDT. These results suggest the importance of treating these patients in a reference center.

摘要

引言

由于其发病率低且具有异质性,除胃肠道间质瘤(GIST)外的局限性消化道高级别肉瘤(SDT)的自然病史很少被研究。我们描述了SDT的组织临床特征,并将其与患者的预后相关联。

方法

我们回顾性收集了一个欧洲数据库中的医疗档案,该数据库涵盖了欧洲约20年列出的结缔组织肿瘤。仅纳入未经治疗的局限性原发性高级别SDT。对每个病例进行了中央组织学审查。比较了患者的特征,并将其与临床结果相关联。

结果

共识别出45例患者。平滑肌肉瘤(LMS)和未分化肉瘤(UDS)占主导地位,前者的总生存期(OS)和无进展生存期(PFS)较好,而后者的预后比其他组织学类型差。34例患者(75%)获得完全缓解,这与男性、年龄超过40岁和单灶性肿瘤有关。完整手术和LMS组织学与较好的预后相关,在基线特征或治疗方式上无显著差异。

结论

完整手术和组织学类型似乎是SDT的预后指标。这些结果表明在参考中心治疗这些患者的重要性。

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