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恶性纤维组织细胞瘤:200例分析

Malignant fibrous histiocytoma: an analysis of 200 cases.

作者信息

Weiss S W, Enzinger F M

出版信息

Cancer. 1978 Jun;41(6):2250-66. doi: 10.1002/1097-0142(197806)41:6<2250::aid-cncr2820410626>3.0.co;2-w.

Abstract

The clinicopathologic findings in 200 cases of malignant fibrous histiocytoma (MFH) with follow-up information are presented. This tumor occurred principally as a mass on an extremity (lower extremity 49%, upper extremity 19%) or in the abdominal cavity or retroperitoneum (16%) of adults (peak incidence 61-70 years of age). It typically involved deep fascia (19%) or skeletal muscle (59%) and only rarely was confined to the subcutis without fascial involvement (7%). The MFH had variable morphologic features and frequently showed transitions from areas having a highly ordered storiform pattern to less differentiated areas having a pleomorphic appearance. The rate of local recurrence of the tumor was 44%, and of metastasis, 42%. Metastasis was most frequently to the lung (82%) and lymph nodes (32%). Factors that influenced the rate of metastasis included depth, size, and inflammatory component of the tumor. Tumors that were small, superficially located, or had a prominent inflammatory component metastasized less frequently than larger, more deeply located tumors. In our experience the MFH is the most common soft tissue sarcoma of late adult life, and many tumors previously diagnosed as pleomorphic variants of liposarcoma, fibrosarcoma, or rhabdomyosarcoma are probably examples of MFH. Although the histogenesis of this neoplasm remains controversial, we feel it is best regarded as a primitive and pleomorphic sarcoma showing partial fibroblastic and histiocytic differentiation, as reflected by collagen production and occasional phagocytosis.

摘要

本文呈现了200例伴有随访信息的恶性纤维组织细胞瘤(MFH)的临床病理特征。该肿瘤主要表现为成人肢体肿块(下肢49%,上肢19%)或腹腔及腹膜后肿块(16%)(发病高峰年龄为61 - 70岁)。它通常累及深筋膜(19%)或骨骼肌(59%),仅有少数情况局限于皮下组织而无筋膜受累(7%)。MFH具有多种形态学特征,常表现为由高度有序的席纹状结构区域向多形性外观的低分化区域过渡。肿瘤的局部复发率为44%,转移率为42%。转移最常见于肺(82%)和淋巴结(32%)。影响转移率的因素包括肿瘤的深度、大小和炎症成分。体积小、位置表浅或炎症成分显著的肿瘤转移频率低于体积大、位置深的肿瘤。根据我们的经验,MFH是成年晚期最常见的软组织肉瘤,许多先前诊断为脂肪肉瘤、纤维肉瘤或横纹肌肉瘤多形性变种的肿瘤可能是MFH的实例。尽管该肿瘤的组织发生仍存在争议,但我们认为它最好被视为一种原始的多形性肉瘤,表现出部分成纤维细胞和组织细胞分化,这可通过胶原产生和偶尔的吞噬作用得以体现。

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