首页> 外文期刊>Medicine. >Giant solitary fibrous tumor of the pleura with high-grade sarcomatous overgrowth accompanied by lipid-rich, rhabdomyosarcomatous, and pleomorphic components: A case report
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Giant solitary fibrous tumor of the pleura with high-grade sarcomatous overgrowth accompanied by lipid-rich, rhabdomyosarcomatous, and pleomorphic components: A case report

机译:胸膜巨大孤立性纤维瘤伴高度肉瘤过度生长并伴有丰富的脂质,横纹肌肉瘤和多形性成分:一例报告

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Rationale: Solitary fibrous tumors are mesenchymal tumors presenting as fibroblastic neoplasms with prominent branching vascular patterns, which are often generated from the pleura. Most solitary fibrous tumors are benign; however, some can turn malignant. High-grade sarcomas from solitary fibrous tumors include multidirectional histopathological components. Patient concerns: We describe our experience of a giant high-grade sarcoma with mixed components generated from a solitary fibrous tumor of the pleura in a 67-year-old female patient presenting with cough and left-sided chest pain. The patient had been diagnosed with a pleural mass in the left chest by X-ray about 30 years earlier. However, the tumor was allowed to grow, without surgical intervention, for a long time. Interventions: Thoracic surgeons performed the removal of the giant pleural tumor; the tumor measured 18.0 × 14.5 × 10 cm in size, and was considered a giant tumor generated from the pleura of the left chest cavity. Diagnoses: The surgically removed tumor was solid and light brownish, and included myxoid and arabesque pattern lesions. The tumor also showed hemorrhagic and necrotic lesions. Moreover, spindle cells with less atypia, resembling fibroblasts, were noted. These spindle tumor cells were CD34- and Stat6-positive, suggesting a solitary fibrous tumor. Some of the spindle tumor cells were surrounded by thick collagenous fibers. Considering that the tumor originated from the parietal pleura, the tumor was defined as a solitary fibrous tumor in origin. The tumor also comprised high-grade sarcomatous components; these included lipid-rich, rhabdomyosarcomatous, and pleomorphic components. The high-grade sarcoma component included bizarre tumor cells with severe atypia. Outcomes: Tumor recurrence occurred in the left chest about 4 months after the surgery, and the patient died 8 months postoperatively. Lessons: The present case clearly demonstrates that a solitary fibrous tumor can develop into high-grade sarcomatous overgrowth, including lipid-rich, rhabdomyosarcoma, and pleomorphic sarcoma components, if left untreated for a prolonged period. This case provides profound insights about the natural history, histogenesis, differentiation, and malignant transformation of solitary fibrous tumors.
机译:原理:孤立性纤维性肿瘤是间充质肿瘤,表现为成纤维细胞赘生物,具有明显的分支血管形态,通常由胸膜产生。大多数孤立性纤维性肿瘤是良性的。但是,有些会变成恶性肿瘤。来自单发性纤维瘤的高级肉瘤包括多向组织病理学成分。患者的担忧:我们描述了我们的经历:一位67岁的女性患者出现咳嗽和左侧胸痛时,出现了巨大的高级别肉瘤,其混合成分由胸膜的孤立性纤维性肿瘤产生。大约30年前,该患者已通过X射线诊断为左胸腔有胸膜肿块。但是,在没有手术干预的情况下,允许肿瘤长期生长。干预措施:胸外科医师切除了巨大的胸膜肿瘤;肿瘤大小为18.0×14.5×10 cm,被认为是左胸腔胸膜产生的巨大肿瘤。诊断:手术切除的肿瘤为实性和浅褐色,包括黏液样和蔓藤花纹样病变。肿瘤还显示出血性和坏死性病变。此外,还发现非典型性较少的纺锤状细胞,类似于成纤维细胞。这些纺锤体肿瘤细胞为CD34和Stat6阳性,提示为孤立性纤维性肿瘤。一些纺锤体肿瘤细胞被厚厚的胶原纤维包围。考虑到肿瘤起源于壁层胸膜,将该肿瘤定义为起源于孤立性纤维性肿瘤。肿瘤还包括高级别的肉瘤成分。这些包括富含脂质,横纹肌肉瘤和多形性成分。高级别的肉瘤成分包括具有非典型性的奇异肿瘤细胞。结果:术后约4个月左胸部出现肿瘤复发,患者在术后8个月死亡。经验教训:本案清楚地表明,如果长期不治疗,一个孤立的纤维瘤可以发展为高度肉瘤过度生长,包括脂质丰富,横纹肌肉瘤和多形肉瘤成分。该病例提供了关于孤立纤维瘤的自然历史,组织发生,分化和恶性转化的深刻见解。

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