腹膜后脂肪肉瘤：对86例脂肪肉瘤和恶性纤维组织细胞瘤进行临床病理复查后对去分化的随访分析

Retroperitoneal liposarcomas: follow-up analysis of dedifferentiation after clinicopathologic reexamination of 86 liposarcomas and malignant fibrous histiocytomas.

作者信息

Fabre-Guillevin Elizabeth, Coindre Jean-Michel, Somerhausen Nicolas de Saint Aubain, Bonichon Francoise, Stoeckle Eberhard, Bui Nguyen Binh

机构信息

Department of Medical Oncology, Hopital Europeen Georges Pompidou, Assistance Publique-Hopitaux de Paris, Paris, France.

出版信息

Cancer. 2006 Jun 15;106(12):2725-33. doi: 10.1002/cncr.21933.

DOI:10.1002/cncr.21933

PMID:16688768

Abstract

BACKGROUND

Dedifferentiated liposarcoma (DDL) juxtapose components of well-differentiated liposarcoma (WDL) and nonlipogenic sarcoma. Malignant fibrous histiocytoma (MFH) is no longer considered a homogeneous entity, but rather as the common morphologic appearance of various subtypes of sarcomas. The objectives of the current retrospective study were: 1( to analyze the relation between DDLs and tumors previously diagnosed as MFHs; 2) to trace the evolution of liposarcomas, and 3) to assess the consequences of dedifferentiation.

METHODS

Between 1974 and 2001, 86 patients with retroperitoneal liposarcoma (61 patients) or MFH (25 patients) underwent surgery at Institut Bergonie in Bordeaux, France. Histologic review was performed and tumors reclassified as WDL or DDL were retained for further clinicohistologic study. Subsequently, initial presentation and all recurrences were analyzed.

RESULTS

The 61 liposarcomas consisted of 21 WDLs and 35 DDLs; 17 MFHs were reclassified as DDL. In all, approximately half of the retroperitoneal liposarcomas and MFHs were found to be DDLs. The DDLs presented with a smaller size (20 cm vs.30 cm; P = .05) but a lower rate of complete resection (72% vs.90%; P = .015) and remission (72% vs. 100%; P = .0015). Dedifferentiated recurrence was evidenced in 7 WDLs. Ten DDLs presented with metastatic evolution. DDLs demonstrated a tendency toward lower rates of 5-year overall survival (55% vs. 82%; P = .075).

CONCLUSIONS

Most occurrences of retroperitoneal liposarcomas and MFHs are in fact DDLs and dedifferentiated recurrence of WDLs is frequent. Retroperitoneal DDLs present a lower rate of complete remission than WDLs and a risk of metastatic recurrence. Therefore, extensive histologic analysis of WDLs is required to identify an undifferentiated component and avoid misdiagnosis of DDL.

摘要

背景

去分化脂肪肉瘤（DDL）兼具高分化脂肪肉瘤（WDL）和非脂肪生成性肉瘤的成分。恶性纤维组织细胞瘤（MFH）不再被认为是一种单一的实体，而是各种肉瘤亚型的常见形态学表现。本回顾性研究的目的是：1）分析DDL与先前诊断为MFH的肿瘤之间的关系；2）追踪脂肪肉瘤的演变过程；3）评估去分化的后果。

方法

1974年至2001年间，86例腹膜后脂肪肉瘤患者（61例）或MFH患者（25例）在法国波尔多的贝贡尼研究所接受了手术。进行了组织学复查，重新分类为WDL或DDL的肿瘤被保留用于进一步的临床组织学研究。随后，对初始表现和所有复发情况进行了分析。

结果

61例脂肪肉瘤中，21例为WDL，35例为DDL；17例MFH被重新分类为DDL。总体而言，约一半的腹膜后脂肪肉瘤和MFH被发现为DDL。DDL的大小较小（20 cm对30 cm；P = 0.05），但完整切除率较低（72%对90%；P = 0.015），缓解率也较低（72%对100%；P = 0.0015）。7例WDL出现去分化复发。10例DDL出现转移进展。DDL的5年总生存率有降低的趋势（55%对82%；P = 0.075）。