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Somatostatinoma syndrome. Clinical, morphological and metabolic features and therapeutic aspects
Abstract:
A case of somatostatinoma syndrome in a 30-year-old woman is presented. Basal levels of growth hormone and of pancreatic and gastric hormones were reduced and the response of growth hormone, insulin and C-peptide to stimuli such as arginine, glucose, glibenclamide and calcium was virtually abolished. Similarly, gastric acid secretion, pancreatic exocrine function and intestinal absorption were significantly reduced. On the other hand, basal and stimulated levels of adrenocorticotropic hormone (ACTH), luteinizing hormone (LH), follicle-stimulating hormone (FSH) and thyroid-stimulating hormone (TSH) were within the normal range. Plasma somatostatin-like immunoreactivity was increased to 600-2,000 pg/ml (normal: 88-140 pg/ml). Immunocytochemical studies demonstrated the presence of somatostatin immunoreactive material in the primary tumour in the head of the pancreas and in the liver metastases. In spite of two courses of chemotherapy with streptozotocin and 5-fluorouracil the patient died due to liver failure 5 months after the first admission to hospital.
Insights
This case study details a rare somatostatinoma syndrome, characterized by significantly reduced hormone levels and impaired bodily functions. Despite treatment, the patient succumbed to liver failure, highlighting the aggressive nature of this rare pancreatic tumor.
Area of Science:
- Endocrinology
- Oncology
- Gastroenterology
Background:
- Somatostatinomas are rare neuroendocrine tumors that secrete somatostatin.
- Somatostatinomas can cause a distinct clinical syndrome due to hormone hypersecretion and subsequent inhibition of other hormones.
Observation:
- A 30-year-old woman presented with symptoms consistent with somatostatinoma syndrome.
- She exhibited reduced basal levels of growth hormone, pancreatic, and gastric hormones, with blunted responses to various stimuli.
- Gastric acid secretion, pancreatic exocrine function, and intestinal absorption were also significantly diminished.
Findings:
- Elevated plasma somatostatin-like immunoreactivity (600-2,000 pg/ml) was observed.
- Immunocytochemistry confirmed somatostatin presence in the primary pancreatic tumor and liver metastases.
- Levels of adrenocorticotropic hormone (ACTH), luteinizing hormone (LH), follicle-stimulating hormone (FSH), and thyroid-stimulating hormone (TSH) remained within normal ranges.
Implications:
- This case highlights the profound physiological impact of somatostatin hypersecretion.
- The findings underscore the challenges in managing aggressive somatostatinomas, even with chemotherapy.
- Early diagnosis and understanding the syndrome's multifaceted effects are crucial for patient management.