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Metastatic extrapleural malignant solitary fibrous tumor presenting with hypoglycemia (Doege–Potter syndrome)

机译:伴有低血糖的转移性胸膜外恶性孤立性纤维性肿瘤(Doege-Potter综合征)

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摘要

We report a rare case of metastatic malignant solitary fibrous tumor (SFT) that presented with hypoglycemia because of insulin growth factor-2 production. Initial workup included computed tomography imaging that revealed a large, partially necrotic liver mass, a hypervascular pancreatic head lesion, and 2 renal lesions. Following hepatic resection, pancreatic head resection and nephrectomy, all these lesions demonstrated pathological findings that were consistent with SFT. The patient also had a history of an intracranial mass that had been previously resected and treated with gamma knife therapy at an outside institution, which was found to also be SFT. Six months after initial pancreatic head resection, the patient developed a new lesion involving the pancreatic tail that was found to represent recurrent metastatic SFT. This case emphasizes the highly aggressive nature of extrapleural SFT, while rare, and the role of imaging in follow-up for disease recurrence.
机译:我们报告了罕见的转移性恶性孤立性纤维性肿瘤(SFT)的案例,由于胰岛素生长因子2的产生而出现低血糖。最初的检查包括计算机断层扫描成像,显示有较大的部分坏死的肝脏肿块,血管性胰头病变和2个肾脏病变。肝切除,胰头切除和肾切除术后,所有这些病变均表现出与SFT一致的病理学发现。该患者还具有颅内肿块的病史,该病史先前曾在外部机构进行过切除和伽马刀疗法治疗,发现也属于SFT。最初的胰头切除术后六个月,患者出现了一个新的病变,该病变累及胰尾,被发现代表复发性转移性SFT。该病例强调了胸膜外SFT的高度侵袭性(尽管罕见),以及成像在疾病复发的随访中的作用。

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