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胸外科手术中的恶性纤维组织细胞瘤

Malignant fibrous histiocytoma in thoracic surgical practice.

作者信息

Venn G E, Gellister J, DaCosta P E, Goldstraw P

出版信息

J Thorac Cardiovasc Surg. 1986 Feb;91(2):234-7.

PMID:3003463
Abstract

Malignant fibrous histiocytoma is a rare, although increasingly recognized, deep-seated pleomorphic sarcoma. A primitive tumor, it arises from tissue histiocytes and typically occurs in the extremities. Primary intrathoracic tumors have been reported rarely. The presentation of malignant fibrous histiocytoma in our series of seven patients has been varied. Two cases presented as solitary primary intrapulmonary tumors, two as primary chest wall tumors, one as an anterior mediastinal mass, one as a retroperitoneal tumor extending cephalad through the diaphragm, and one as a late metastasis from a primary pelvic site. Malignant fibrous histiocytoma is aggressive, with a propensity for early local and distant spread; three of the patients in our series died of progressive disease within 17 months of operation. The histologic nature of the tumor makes diagnosis on small biopsy specimens difficult and frequently misleading. We would suggest a policy of open biopsy to obtain adequate and representative specimens for histologic study and preoperative computed tomographic scanning to augment the clinical search for metastatic disease and to facilitate planning of subsequent radical, excisional operation. The preoperative use of deep x-ray therapy or the newer chemotherapeutic agents may reduce tumor bulk and thereby facilitate radical operation, which presently appears to be the most appropriate primary modality of treatment of malignant fibrous histiocytoma.

摘要

恶性纤维组织细胞瘤是一种罕见的、但越来越被认识到的深部多形性肉瘤。它是一种原发性肿瘤,起源于组织组织细胞,通常发生在四肢。原发性胸内肿瘤报道很少。我们这组7例恶性纤维组织细胞瘤患者的表现各不相同。2例表现为孤立性原发性肺内肿瘤,2例为原发性胸壁肿瘤,1例为前纵隔肿块,1例为经膈肌向头侧延伸的腹膜后肿瘤,1例为原发性盆腔部位的晚期转移瘤。恶性纤维组织细胞瘤具有侵袭性,倾向于早期局部和远处扩散;我们这组中有3例患者在手术后17个月内因疾病进展而死亡。肿瘤的组织学性质使得从小活检标本中做出诊断很困难,而且常常会产生误导。我们建议采用开放活检的策略,以获取足够且有代表性的标本用于组织学研究,并进行术前计算机断层扫描,以加强对转移性疾病的临床检查,并便于规划后续的根治性切除手术。术前使用深部X线治疗或更新的化疗药物可能会缩小肿瘤体积,从而便于进行根治性手术,目前根治性手术似乎是治疗恶性纤维组织细胞瘤最合适的主要治疗方式。

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