Doege-Potter Syndrome
N Ahluwalia1, R Attia1, A Green1
1Guy's and St Thomas' NHS Foundation Trust , UK.
Abstract:
Doege-Potter syndrome is a rare paraneoplastic syndrome presenting as a hypoinsulinaemic hypoglycaemia from the ectopic secretion of a prohormone of insulin-like growth factor II (IGF-II) from a solitary fibrous tumour. Surgical resection is curative in the majority of cases. If, however, the diagnosis is not suspected and treatment is delayed, it can lead to hypoxic cerebral injury or death. The underlying tumour can be a benign or malignant pleural tumour but may be present in extrapleural sites. For a diagnosis of Doege-Potter syndrome, symptoms attributable to hypoglycaemia and low blood glucose levels should be present along with the secretion of prohormone IGF-II. We report a case of severe hypoglycaemia in a 76-year-old inpatient admitted for resection of a recurrent left-sided pleural tumour. Investigation revealed true hypoglycaemia and Doege-Potter syndrome was diagnosed. The tumour was completely resected and the patient made a full recovery with no further hypoglycaemic episodes.
Insights
Doege-Potter syndrome, a rare condition causing low blood sugar, is linked to solitary fibrous tumors secreting insulin-like growth factor II. Surgical removal of the tumor effectively cures this paraneoplastic syndrome.
Area of Science:
- Endocrinology
- Oncology
- Pathology
Background:
- Doege-Potter syndrome is a rare paraneoplastic syndrome characterized by hypoinsulinaemic hypoglycemia.
- It results from ectopic secretion of insulin-like growth factor II (IGF-II) prohormone, typically from a solitary fibrous tumor.
Observation:
- A 76-year-old inpatient presented with severe hypoglycemia.
- The patient was admitted for resection of a recurrent left-sided pleural tumor.
Findings:
- Investigations confirmed true hypoglycemia and diagnosed Doege-Potter syndrome.
- The underlying cause was identified as a solitary fibrous tumor secreting IGF-II prohormone.
Implications:
- Complete surgical resection of the tumor led to a full recovery.
- Early diagnosis and treatment are crucial to prevent hypoxic cerebral injury or death.
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