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Abstract：
BACKGROUND: Doege-Potter syndrome is a rare paraneoplastic phenomenon associated with solitary fibrous tumors of the pleura (SFTPs). It is characterized by the presence of severe, sustained, and treatment-refractory hypoglycemia. Hypoglycaemia, which may be the sole symptom at disease onset, is mediated by the secretion of high-molecular-weight insulin-like growth factor (IGF-2). Most tumors exhibit benign behavior, with a 100% survival rate at 5 years. However, 10% of these tumors may display aggressive behavior with local or metastatic recurrence. We present a clinical case of a patient with a benign solitary fibrous tumor of the pleura who presented with symptomatic hypoglycemia and required pulmonary and pleural surgical resection to control the paraneoplastic phenomenon.
METHODS: A Hispanic 46-year-old man presented with a 15-day history of transient alterations in consciousness worsened by fasting. The relevant medical history included obstructive sleep apnea treated with continuous positive air pressure (CPAP) and previous smoking. In-hospital studies revealed noninsulinemic hypoglycemia and a benign SFTP. Complete surgical resection was performed while the patient received dextrose fluids and corticosteroids perioperatively for hypoglycemia. Subsequently, the hypoglycemia resolved, and the patient was followed-up without disease recurrence.
CONCLUSIONS: Doege-Potter syndrome is challenging to recognize. However, effective treatment can be achieved with a high survival rate. Raising awareness among healthcare professionals about the recognition of this paraneoplasic syndrome patients will improve diagnostic suspicion, biochemical confirmation, the development of diagnostic and therapeutic guidelines, and the creation of predictive indices for aggressive presentations requiring closer monitoring.
METHODS: A Hispanic 46-year-old man presented with a 15-day history of transient alterations in consciousness worsened by fasting. The relevant medical history included obstructive sleep apnea treated with continuous positive air pressure (CPAP) and previous smoking. In-hospital studies revealed noninsulinemic hypoglycemia and a benign SFTP. Complete surgical resection was performed while the patient received dextrose fluids and corticosteroids perioperatively for hypoglycemia. Subsequently, the hypoglycemia resolved, and the patient was followed-up without disease recurrence.
CONCLUSIONS: Doege-Potter syndrome is challenging to recognize. However, effective treatment can be achieved with a high survival rate. Raising awareness among healthcare professionals about the recognition of this paraneoplasic syndrome patients will improve diagnostic suspicion, biochemical confirmation, the development of diagnostic and therapeutic guidelines, and the creation of predictive indices for aggressive presentations requiring closer monitoring.
摘要：
背景：Doege-Potter综合征是一种与胸膜孤立性纤维瘤（SFTP）相关的罕见副肿瘤现象。它的特点是存在严重，持续,和治疗难治性低血糖。低血糖,这可能是疾病发作时唯一的症状，是由高分子量胰岛素样生长因子（IGF-2）的分泌介导的。大多数肿瘤表现出良性行为，5年生存率为100%。然而,这些肿瘤中的10％可能表现出侵袭性行为，并伴有局部或转移性复发。我们介绍了一例胸膜良性孤立性纤维瘤患者的临床病例，该患者表现为有症状的低血糖，需要进行肺和胸膜手术切除以控制副肿瘤现象。
方法：一名46岁的西班牙裔男子因禁食而出现短暂意识改变的15天病史。相关病史包括持续正压（CPAP）治疗的阻塞性睡眠呼吸暂停和先前吸烟。住院研究显示非胰岛素性低血糖和良性SFTP。在患者因低血糖而围手术期接受葡萄糖液和皮质类固醇治疗时，进行了完整的手术切除。随后，低血糖消退，对患者进行随访，无疾病复发。
结论：Doege-Potter综合征具有挑战性。然而,有效的治疗可以实现高存活率。提高医疗保健专业人员对这种副肿瘤综合征患者的认识将改善诊断怀疑，生化确认，诊断和治疗指南的发展，以及为需要更密切监测的激进演示创建预测指数。
方法：一名46岁的西班牙裔男子因禁食而出现短暂意识改变的15天病史。相关病史包括持续正压（CPAP）治疗的阻塞性睡眠呼吸暂停和先前吸烟。住院研究显示非胰岛素性低血糖和良性SFTP。在患者因低血糖而围手术期接受葡萄糖液和皮质类固醇治疗时，进行了完整的手术切除。随后，低血糖消退，对患者进行随访，无疾病复发。
结论：Doege-Potter综合征具有挑战性。然而,有效的治疗可以实现高存活率。提高医疗保健专业人员对这种副肿瘤综合征患者的认识将改善诊断怀疑，生化确认，诊断和治疗指南的发展，以及为需要更密切监测的激进演示创建预测指数。
