关键词: Solitary fibrous tumor gastrointestinal stromal tumor metastases urinary bladder

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Abstract:
Solitary fibrous tumor (SFT) of the urinary bladder has been rarely reported and malignant bladder SFT is even rarer. Here we present a case of an African-American male with SFT of the urinary bladder (intermediate risk) initially treated by cystoprostatectomy at the age of 59 years. Eight years later, he developed recurrence with widespread metastases to the liver, lungs, and abdominal cavity. He then received temozolomide and bevacizumab with good disease control. However, treatment was paused due to declining performance status. Follow-up at 1 year demonstrated growth of the metastatic lesions. Despite restarting therapy, the patient expired, 11 years after the original diagnosis. Autopsy was performed and revealed widespread metastases within the abdominal cavity (abdominal sarcomatosis) as well as liver, bilateral lung, and diaphragmatic involvement. The cause of death was determined to be metastatic SFT. A comprehensive literature review was performed. Although SFTs are commonly considered benign, a subset of SFTs of the urinary bladder behave aggressively. Risk assessment and proper follow-up for recurrence and metastasis is necessary. The patient was also found at autopsy to have two gastrointestinal stromal tumors (GISTs) in the stomach and near the gastroesophageal junction. To the best of our knowledge, this is the first reported case of a primary urinary bladder SFT resulting in death or having concurrent, multifocal GISTs, and only the second case of a bladder SFT that developed metastases after the initial diagnosis.
摘要:
膀胱孤立性纤维瘤(SFT)的报道很少,恶性膀胱SFT更是罕见。在这里,我们介绍了一名非洲裔美国男性,患有膀胱SFT(中等风险),最初在59岁时接受膀胱前列腺切除术治疗。八年后,他复发并广泛转移到肝脏,肺,和腹腔。然后他接受了替莫唑胺和贝伐单抗,疾病控制良好。然而,由于表现状态下降而暂停治疗.1年的随访显示转移性病变的生长。尽管重新开始治疗,病人过期了,最初诊断后的11年。进行尸检,发现腹腔(腹部肉瘤病)和肝脏内广泛转移,双侧肺,和膈肌受累。死亡原因被确定为转移性SFT。进行了全面的文献综述。虽然SFT通常被认为是良性的,膀胱SFT的一个子集表现出攻击性。复发和转移的风险评估和适当的随访是必要的。在尸检中还发现该患者在胃和胃食管交界处附近有两个胃肠道间质瘤(GIST)。据我们所知,这是首例原发性膀胱SFT导致死亡或并发的病例,多焦点GIST,只有第二例膀胱SFT在最初诊断后发生转移。
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