胸膜弥漫性黏液性恶性纤维组织细胞瘤一例并文献复习

Diffuse myxoid malignant fibrous histiocytoma of the pleura:a case report and review of the literature

目的探讨胸膜弥漫性黏液性恶性纤维组织细胞瘤(MFH)的临床和病理特点。方法分析我院收治的1例胸膜弥漫性黏液性 MFH 患者的临床和病理资料,并分析国内外文献报道的7例胸膜 MFH 病例。结果患者为男性,65岁,表现为咳嗽、胸痛、气促伴大量血性胸腔积液。胸部 CT示右侧脏壁层胸膜弥漫性肿块影。开胸探查见脏壁层胸膜表面弥漫性灰白色半透明肿块,直径为1～8 cm。光镜下可见黏液性基质中瘤细胞弥漫分布,呈梭形和多形性。波形蛋白、CD_(68)、溶菌酶免疫组化染色瘤细胞呈阳性反应,细胞角蛋白、上皮膜抗原、结蛋白呈阴性反应。术后24 d,因上腔静脉梗阻、循环衰竭死亡。与文献报道的非弥漫性胸膜 MFH 相比,本例为胸膜弥漫性多灶性 MFH,起病急,病情进展快。结论胸膜弥漫性黏液性 MFH 是非常罕见的胸膜原发性肿瘤,恶性程度高,预后极差。

Objective To study the clinicopathologic characteristics of diffuse myxoid malignant fibrous histiocytoma （MFH） of pleura. Methods The clinical and pathological features of a patient with diffuse myxoid MFH of pleura from this hospital were analyzed, and the reported literature of 7 patients with MFH of pleura were reviewed. Results The patient was a 65-year-old male. The primary manifestations were cough, chest pain, breathlessness, and a great quantity of bloody pleural effusion. Chest CT scan showed diffuse masses on the visceral and parietal pleura. Exploratory thoracotomy exposed diffuse translucent gray-white masses on the surface of visceral and parietal pleura, with diameters of 1 - 8 cm. Microscopic findings showed that diffuse spindle-shaped and pleomorphic tumour cells were within the myxoid stroma. Tumor cells were positive for vimentin, CD68, lysozyme, and negative for CK, EMA, and desmin. The patient died of obstruction of superior vena cava, and circulatory failure at 24th postoperative day. Different from cases with single or multiple MFH of pleura reported in the literature, this case of diffuse myxoid MFH had an abrupt onset, and progressed aggressively. Conclusion Diffuse myxoid MFH of pleura is a very rare high-grade malignant tumor with very poor prognosis.