摘要
低度恶性纤维黏液样肉瘤是一种特殊类型的软组织恶性肿瘤,临床少见,其组织形态温和,容易被误诊为其他良性肿瘤。现报告郑州人民医院2023年9月收治的1例肩部低度恶性纤维黏液样肉瘤患者的临床表现、病理特征及治疗和预后情况,对相关文献进行复习和总结。患者,男,32岁,肩部肿物,镜下见交替分布的纤维性和黏液样区域组成,黏液样区域内可见弧形血管,纤维化区域可见纤细的胶原纤维和菊形团结构,细胞核呈短梭形或卵圆形,轻度异型性,核分裂象少见。免疫组织化学染色示MUC4阳性,FUS基因断裂。术后未行放化疗,随访5个月无复发。低度恶性纤维黏液样肉瘤是一种少见的低度恶性肿瘤,生物学行为较为惰性,其最终判断依赖病理诊断,正确认识其形态学、免疫表型及分子生物学改变至关重要。
Low-grade fibromyxoid sarcoma(LGFMS)is a rare subtype of malignant soft tissue tumor characterized by deceptively bland histological features,often leading to misdiagnosis as a benign tumor.This paper reports a case treated at Zhengzhou People’s Hospital in September 2023,involving a 32-year-old male with a shoulder mass.Histopathology revealed alternating fibrous and myxoid areas.Myxoid zones displayed curvilinear vessels,while fibrous zones contained fine collagen fibers and rosette-like structures.Tumor cells showed short spindle-or oval-shaped nuclei with mild atypia and rare mitoses.Immunohistochemistry showed MUC 4 positivity,and FUS gene rearrangement was detected.The patient underwent surgical resection without adjuvant radiotherapy or chemotherapy and showed no recurrence after a 5-month follow-up.LGFMS is an uncommon tumor with low-grade malignant potential and indolent biological behavior.Accurate diagnosis relies on pathological evaluation,making recognition of its morphological,immunophenotypic,and molecular characteristics crucially important.
作者
王建君
徐小艳
胡晓舒
闫琛
姜黄
WANG Jianjun;XU Xiaoyan;HU Xiaoshu;YAN Chen;JIANG Huang(Department of Pathology,Fifth Clinical Medical College of Henan University of Chinese Medicine/Zhengzhou People’s Hospital,Zhengzhou 450001,China)
出处
《临床与病理杂志》
2025年第2期256-264,共9页
Journal of Clinical and Pathological Research
关键词
低度恶性纤维黏液样肉瘤
病理诊断
治疗
预后
临床特征
low-grade fibromyxoid sarcoma
pathological diagnosis
treatment
prognosis
clinical features
