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肠系膜恶性纤维组织细胞瘤:一例报告

Malignant fibrous histiocytoma (MFH) of the mesentery: a case report.

作者信息

Bodner Klaus, Bodner-Adler Barbara, Mayerhofer Stephan, Grünberger Werner, Wierrani Franz, Czerwenka Klaus, Leodolter Sepp, Mayerhofer Klaus

机构信息

Department of Gynecology & Obstetrics, University of Vienna Medical School, Austria.

出版信息

Anticancer Res. 2002 Mar-Apr;22(2B):1169-70.

Abstract

BACKGROUND

Primary tumors of the mesentery are rare; only a few cases of malignant fibrous histiocytoma have been reported in the literature. This case report presents the management of a patient with malignantfibrous histiocytoma of the mesentery.

CASE REPORT

A 48-year-old woman presented with mild abdominal pain and a palpable tumor in the lower abdomen. Laparatomy revealed a 12x9 cm tumor located in the mesentery and an enlargement of the paraaortic lymph nodes. The tumor was histologically classified as a malignant fibrous histiocytoma, showing a heterologeous picture consisting of large, multinucleated cells and spindle-shaped cells forming a storiform-like growth pattern. A radical excision of the tumor and the lymphnodes was performed and the patient received adjuvant irradiation therapy. Approximately three months later she presented with a great multilobated pelvic mass infiltrating the uterus and the adnexa. The patient underwent total abdominal hysterectomy and bilateral salpingo-oopherectomy to reduce the tumor mass. Two courses of palliative cytotoxic polychemotherapy were applied, but the patient died two months later due to progression of disease.

CONCLUSION

Malignant fibrous histiocytoma of the mesentery is an extremely rare, highly malignant neoplasm with early metastatic spread. The treatment of choice is wide surgical excision, while the role of adjuvant chemotherapy and irradiation still remains controversiaL

摘要

背景

肠系膜原发性肿瘤罕见;文献中仅报道过少数几例恶性纤维组织细胞瘤。本病例报告介绍了一名肠系膜恶性纤维组织细胞瘤患者的治疗情况。

病例报告

一名48岁女性,表现为轻度腹痛,下腹部可触及肿物。剖腹探查发现一个12×9厘米的肿瘤位于肠系膜,腹主动脉旁淋巴结肿大。肿瘤组织学分类为恶性纤维组织细胞瘤,呈现出由大的多核细胞和梭形细胞组成的异源性图像,形成席纹状生长模式。对肿瘤和淋巴结进行了根治性切除,患者接受了辅助放疗。大约三个月后,她出现了一个巨大的多叶状盆腔肿物,侵犯子宫和附件。患者接受了全腹子宫切除术和双侧输卵管卵巢切除术以缩小肿瘤体积。应用了两个疗程的姑息性细胞毒性联合化疗,但患者在两个月后因疾病进展死亡。

结论

肠系膜恶性纤维组织细胞瘤是一种极其罕见、高度恶性的肿瘤,早期即可发生转移。治疗的选择是广泛的手术切除,而辅助化疗和放疗的作用仍存在争议。

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