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一名患有神经纤维瘤病的患者发生的胃肠道恶性纤维组织细胞瘤。

Malignant fibrous histiocytoma of the gastrointestinal tract in a patient with neurofibromatosis.

作者信息

Katz R N, Waye J D, Batzel E L, Reiner M A, Freed J S

机构信息

Mount Sinai Medical Center, New York, New York.

出版信息

Am J Gastroenterol. 1990 Nov;85(11):1527-30.

PMID:2173401
Abstract

Malignant fibrous histiocytoma (MFH) of the gastrointestinal tract is extremely rare. A case of MFH of the colon associated with neurofibromatosis is presented. MFH is a high-grade soft-tissue sarcoma of fibroblast cell origin with a strong propensity for metastasis and recurrence. Immunochemical markers help to differentiate MFH from other sarcomas. The most successful treatment of MFH is surgical extirpation. Adjuvant chemotherapy and radiotherapy have not been definitively shown to be of value.

摘要

胃肠道恶性纤维组织细胞瘤(MFH)极为罕见。本文报告1例与神经纤维瘤病相关的结肠MFH病例。MFH是一种起源于成纤维细胞的高级别软组织肉瘤,具有很强的转移和复发倾向。免疫化学标志物有助于将MFH与其他肉瘤区分开来。MFH最成功的治疗方法是手术切除。辅助化疗和放疗尚未被明确证明具有价值。

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