全身多发性恶性孤立性纤维性肿瘤1例及相关文献回顾

Systemic multiple malignant solitary fibrous tumors: A case report and related literature review

目的:探索恶性孤立性纤维性肿瘤(malignantsolitary fibrous tumor,MSFT)的临床病理特征、诊断、鉴别诊断、治疗及预后。方法:回顾分析1例MSFT临床病理特征,并通过检索相关数据库,复习相关文献报告。结果:此例为61岁男性患者,主因胸闷、活动后气喘,伴咳嗽、咳痰半月,原发肿瘤位于右肺下叶,双肺散在发生,且于胸前壁、口腔、左侧丘脑、心包前区、脾脏及腹膜后等可见肿瘤生长。B超引导下经皮肺穿刺活检组织瘤镜检:组织之瘤细胞呈短梭形或卵圆形,排列呈束状、席纹状、波浪状,瘤细胞丰富,密集,胞核增大、深染、异型,核分裂>4/10 HPF,部分有出血、坏死及黏液变,黏液变区有少量淋巴细胞浸润。免疫组化:肿瘤细胞CD34(+)、vimentin(+),CD99(+)、EMA(+/-),不表达S-100、Bcl-2、CKp、h-caldesmon、CK8/18、desmin,Ki67阳性细胞数50%。结论:MSFT是一种少见的间叶源性肿瘤,可发生于全身各个组织器官,临床表现缺乏特异性,确诊主要依靠形态学及免疫组化检查,常需与恶性纤维瘤、纤维肉瘤、恶性胸膜间皮瘤、血管外皮细胞肉瘤、滑膜肉瘤、脑膜瘤、恶性神经鞘瘤相鉴别。MSFT预后差,易复发和转移、孤立性病变首选手术切除,术后需长期随访,不可切除患者放化疗效果欠佳。除此之外,针对该病全身多器官发病及临床表现缺乏特异性等特点,临床医师应详细询问病史、仔细体格检查,并与患者及家属良好沟通,尽可能使诊断准确化及患者获益最大化。

Objective：To explore the clinical features,diagnosis,differential diagnosis,treatment and prognosis of malignant solitary fibrous tumor （MSFT）. Methods：One case of MSFF was retrospectively analyzed for its clinical case characteristics. And the literature was reviewed by searching the database of Pubmed and CNKI. Results： The case was on a 61 -year- old male patient who complainted chest tightness, asthma with cough and sputum last half months, the primary tumor was located in the right lower lobe, and lungs were scattered ,besides,in the chest wall, mouth,left thalamus, pericardial anterior, spleen and retroperitoneal tumor growth could be seen. Ultrasound guided percutaneous puncture biopsies tissue microscopy showed the tumor was composed of areas of alternating hypercella- larity and hypocellularity which were separated by dense collagenous fibrous stroma, the tumor cells were spindle to short - spindle shaped and arranged in fascieular or storiform pattern. The tumor cells showed marked nuclear atypia, increased mitotic activity （ 〉 4/10 HPF） , coagulative necrosis and focal hemorrhage, mucus variable area with a small amount of lymphocyte infiltration. Immunohistochemically, the tumor cells were diffusely positive staining for CD34, vi- mentin, CD99 and EMA,while negative staining for S - 100, Bcl - 2, CKp, h - caldesmon, CK8/18, desmin. Ki - 67 labelling index was 50%. Conclusion：MSFT is a rare mesenchymal tumor, which can occur in all tissues and organs of the body. The clinical manifestations are lack of specificity. The diagnosis is mainly related to morphological and immunohistochemistry. Differential diagnosis includes malignant fibroma, fibrosarcoma, malignant pleural mesothelio- ma, vascular cell sarcoma, synovial sarcoma, meningioma, malignant schwannoma. The prognosis of MSFT is poor and easy to recur and even metastasize, The major treatment for the resectable is to completely removed and long term clin- ical follow - up is necessary, while unresectable patients with poor chemotherapeutic effect. In addition, considering the disease characteristics of multiple - organs incidence and the lack of specific manifestations, clinicians should asked medical history in detail and conducted physical examination carefully, and made good communication with pa- tients and their families, so that ensured the accuracy of diagnosis and maximize the benefits of patients.