Internal Medicine, Fundación Cardioinfantil-Instituto de Cardiología, Calle 163ª#13B-60, Bogotá, Colombia.
School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia.
J Med Case Rep. 2024 Aug 14;18(1):383. doi: 10.1186/s13256-024-04658-1.
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.
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.
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.
Doege-Potter 综合征是一种罕见的副瘤现象，与胸膜孤立性纤维瘤（SFTP）有关。其特征是存在严重、持续且治疗抵抗的低血糖症。低血糖症可能是疾病发作时的唯一症状，由高分子量胰岛素样生长因子（IGF-2）的分泌介导。大多数肿瘤表现出良性行为，5 年生存率为 100%。然而，这些肿瘤中有 10%可能表现出侵袭性行为，伴有局部或转移性复发。我们报告了 1 例胸膜良性孤立性纤维瘤患者的临床病例，该患者表现为症状性低血糖症，需要进行肺和胸膜外科切除术来控制副瘤现象。
一名 46 岁西班牙裔男性，因 15 天短暂意识改变病史就诊，禁食后病情加重。相关的医疗史包括阻塞性睡眠呼吸暂停，用持续气道正压通气（CPAP）治疗和既往吸烟史。住院期间研究显示非胰岛素性低血糖和良性 SFTP。在围手术期，患者接受葡萄糖液和皮质类固醇治疗低血糖的同时进行了完全手术切除。随后，低血糖得到缓解，患者无疾病复发，进行了随访。
Doege-Potter 综合征难以识别。然而，通过有效的治疗可以获得高存活率。提高医疗保健专业人员对这种副瘤综合征患者的认识，将提高对其的诊断怀疑、生化确认、诊断和治疗指南的制定以及对需要更密切监测的侵袭性表现的预测指数的创建。
