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临床呈现为原发性卵巢肿瘤的低度恶性纤维黏液样肉瘤:一例报告

Low-grade fibromyxoid sarcoma presenting clinically as a primary ovarian neoplasm: a case report.

作者信息

Winfield Harry L, De Las Casas Luis E, Greenfield William W, Santin Alessandro D, McKenney Jesse K

机构信息

Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, Arkansas 72205, USA.

出版信息

Int J Gynecol Pathol. 2007 Apr;26(2):173-6. doi: 10.1097/01.pgp.0000228145.36807.43.

DOI:10.1097/01.pgp.0000228145.36807.43
PMID:17413985
Abstract

Low-grade fibromyxoid sarcoma is an uncommon, deceptively bland mesenchymal neoplasm that typically occurs in the deep soft tissues of the proximal extremities of young to middle-aged patients. Intra-abdominal low-grade fibromyxoid sarcomas are distinctly rare. We describe the first reported example of this sarcoma involving the ovary. The 42-year-old patient presented with progressing abdominal pain and urinary frequency. Computed tomographic imaging of the abdomen and pelvis showed a 14-cm left-sided pelvic mass. The patient underwent surgical resection and, intraoperatively, a left ovarian mass was identified that extended to both the left hypogastric artery and the left ureter. The resected specimen consisted of a 17-cm tan, fleshy mass containing grossly recognizable nodules of gelatinous, myxoid tissue and a small rim of normal ovary. Microscopic examination of the neoplasm revealed a cytologically bland spindle cell proliferation set in a collagenized stroma that abruptly transitioned to nodules of a myxoid stroma with a well-formed capillary vasculature, features characteristic of low-grade fibromyxoid sarcoma. On the basis of the diagnosis, the patient was clinically staged as a retroperitoneal sarcoma with secondary ovarian involvement. The patient has stable residual disease 11 months postoperatively. This case adds to the literature of intra-abdominal low-grade fibromyxoid sarcoma and expands the list of malignant mesenchymal neoplasms that may involve the ovary.

摘要

低度纤维黏液样肉瘤是一种罕见的、看似良性的间叶性肿瘤,通常发生于青年至中年患者近端肢体的深部软组织。腹腔内低度纤维黏液样肉瘤极为罕见。我们报告了首例累及卵巢的该型肉瘤。这位42岁的患者表现为进行性腹痛和尿频。腹部和盆腔的计算机断层扫描成像显示左侧盆腔有一个14厘米的肿块。患者接受了手术切除,术中发现左侧卵巢肿块,该肿块延伸至左下腹动脉和左输尿管。切除的标本为一个17厘米的黄褐色肉质肿块,包含肉眼可见的胶冻样、黏液样组织结节以及一小圈正常卵巢组织。对该肿瘤进行显微镜检查发现,在胶原化的基质中存在细胞学上看似良性的梭形细胞增殖,该基质突然转变为具有良好形成的毛细血管脉管系统的黏液样基质结节,这些特征是低度纤维黏液样肉瘤的典型表现。根据诊断,该患者临床分期为腹膜后肉瘤伴继发性卵巢受累。患者术后11个月残留疾病稳定。该病例丰富了腹腔内低度纤维黏液样肉瘤的文献,并扩大了可能累及卵巢的恶性间叶性肿瘤的范围。

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