STATE OF THE ART PAPER
Addressing recurrent hypoglycaemia through thoracic surgical intervention: understanding Doege-Potter syndrome, a rarity in syndromes
1
Department of Cardiothoracic and Vascular Surgery, WestpfalzKlinikum, Kaiserslautern, Germany.
2
Department of Thoracic Surgery, Asklepios Lung Clinic Munich-Gauting, Gauting, Germany
3
Department of Surgery, General University Hospital of Patras, Patras, Greece
4
Department of Cardiothoracic Surgery, Patras University Hospital, Patras, Greece
5
Health Centre of Akrata, Akrata, Greece
6
Department of Thoracic Surgery and Thoracic Endoscopy, Ruhrlandklinik, West German Lung Center, University Hospital Essen, University Duisburg-Essen Essen, Germany.
Submission date: 2024-01-01
Final revision date: 2024-01-29
Acceptance date: 2024-01-29
Publication date: 2024-02-28
Corresponding author
Arch Med Sci Atheroscler Dis 2024;9(1):33-40
KEYWORDS
Doege-Potter syndromenon-islet cell tumour hypoglycaemiaparaneoplastic phenomenonrecurrent hypoglycaemia
TOPICS
ABSTRACT
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.
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| ISSN: | 2451-0629 |
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