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Pancreatic somatostatinoma presenting with hypoglycaemia
Clinical Endocrinology
|June 1, 1980
Summary
A rare tumor primarily secreting somatostatin, but also insulin, caused severe hypoglycemia. Treatment with diazoxide, chlorothiazide, and 5-fluorouracil led to tumor regression.
Area of Science:
- Endocrinology
- Oncology
- Pathology
Background:
- A 33-year-old woman presented with hypoglycaemic coma.
- Initial investigations suggested a malignant insulinoma as the cause of her symptoms.
Observation:
- Immunohistological examination revealed a tumor predominantly composed of somatostatin-producing cells with sparse insulin-secreting cells.
- Elevated plasma somatostatin levels (50-200x normal) were observed, responding to arginine and diazoxide.
- The patient's hypoglycemia showed unusual sensitivity to diazoxide and chlorothiazide.
Findings:
- The tumor was diagnosed as a mixed somatostatinoma and insulinoma.
- Pharmacological agents like diazoxide and chlorothiazide demonstrated hyperglycemic effects.
- Treatment with 5-fluorouracil resulted in clinical, biochemical, and radiological tumor regression.
Implications:
- This case highlights a rare dual-hormone-secreting tumor.
- It underscores the potential for somatostatinomas to cause significant hypoglycemia.
- Successful management involved a combination of medical and chemotherapeutic interventions, suggesting a potential treatment strategy for similar rare tumors.