Somatostatinoma Presented as Double-Duct Sign

Area of Science:

• Neuroendocrine Tumors
• Gastroenterology
• Oncology

Background:

• Somatostatinoma is an exceptionally rare neuroendocrine tumor with a low incidence rate.
• It frequently presents asymptomatically, discovered incidentally during imaging or surgery for other abdominal conditions.
• Common presentations include vague symptoms or a triad of glucose intolerance, steatorrhea, and achlorhydria.

Observation:

• The majority of somatostatinomas are found in the pancreatic head, followed by the duodenum.
• Prognosis is often poor, with over 77% of cases diagnosed at advanced stages with local invasion or metastasis.
• A case of a 43-year-old male with an ampullary lesion, initially with unrevealing biopsies, is presented.

Findings:

• Endoscopic retrograde cholangiopancreatography (ERCP) revealed a 2 cm ampullary lesion causing upstream obstruction.
• Histopathology after laparoscopic pancreaticoduodenectomy confirmed stage IIb somatostatinoma.
• This case highlights the diagnostic challenges and emphasizes the need for a high index of suspicion.

Implications:

• The rarity of somatostatinoma limits the development of standardized treatment protocols.
• Surgical resection remains the primary treatment for early-stage disease.
• Physicians should consider somatostatinoma in the differential diagnosis for elevated liver enzymes, especially with relevant imaging findings.