Leivaditis Vasileios, Ehle Benjamin, Papatriantafyllou Athanasios, Mulita Francesk, Koletsis Efstratios, Verras Georgios-Ioannis, Tasios Konstantinos, Antzoulas Andreas, Charokopos Nikolaos, Dahm Manfred, Katsakiori Paraskevi F, Grapatsas Konstantinos
Department of Cardiothoracic and Vascular Surgery, WestpfalzKlinikum, Kaiserslautern, Germany.
Department of Thoracic Surgery, Asklepios Lung Clinic Munich-Gauting, Gauting, Germany.
Arch Med Sci Atheroscler Dis. 2024 Feb 28;9:e33-e40. doi: 10.5114/amsad/183433. eCollection 2024.
Doege-Potter syndrome (DPS), a rare paraneoplastic phenomenon characterised by non-islet cell tumour hypoglycaemia (NICTH), presents clinicians with intricate diagnostic and therapeutic challenges. This comprehensive review consolidates current understanding, clinical presentations, diagnostic modalities, therapeutic interventions, and emerging trends in managing DPS. The pathophysiology of DPS revolves around dysregulated insulin-like growth factors (IGF), particularly IGF-2, produced by mesenchymal tumours, notably solitary fibrous tumours (SFT). Clinical manifestations encompass recurrent hypoglycaemic episodes, often distinct from typical hypoglycaemia, with implications for insulin and counterregulatory hormone levels. Diagnosis necessitates a multidisciplinary approach integrating biochemical assays, imaging studies, and histopathological confirmation of the underlying neoplasm. Surgical resection remains the cornerstone of treatment, complemented by adjunctive therapies to manage persistent hypoglycaemia. Prognosis is influenced by successful tumour resection and long-term surveillance for recurrence. A patient-centred approach, incorporating supportive services and multidisciplinary care, is essential for optimal outcomes in individuals affected by DPS.
多伊格-波特综合征(DPS)是一种罕见的副肿瘤现象,其特征为非胰岛细胞瘤低血糖症(NICTH),给临床医生带来了复杂的诊断和治疗挑战。这篇综述整合了目前对DPS的认识、临床表现、诊断方法、治疗干预措施以及治疗DPS的新趋势。DPS的病理生理学围绕间充质肿瘤(尤其是孤立性纤维性肿瘤,SFT)产生的胰岛素样生长因子(IGF)失调展开,特别是IGF-2。临床表现包括反复出现的低血糖发作,通常与典型低血糖不同,对胰岛素和反调节激素水平有影响。诊断需要多学科方法,综合生化检测、影像学检查以及对潜在肿瘤的组织病理学确认。手术切除仍然是治疗的基石,并辅以辅助治疗来控制持续性低血糖。预后受肿瘤切除是否成功以及复发的长期监测影响。以患者为中心的方法,包括支持性服务和多学科护理,对于受DPS影响的个体获得最佳治疗效果至关重要。