Primary pancreatic paraganglioma: a systematic review

Area of Science:

• Endocrinology
• Surgical Oncology
• Gastroenterology

Background:

• Pancreatic paraganglioma (P-PGL) is an exceptionally rare neuroendocrine tumor.
• It is often misdiagnosed due to overlapping clinical and radiological features with other pancreatic neoplasms.
• Limited evidence exists, lacking standardized diagnostic and therapeutic strategies.

Purpose of the Study:

• To systematically review published cases of P-PGL.
• To summarize clinical presentation, diagnostic approaches, management, and outcomes.
• To provide insights into this rare pancreatic tumor.

Main Methods:

• Systematic literature search in major biomedical databases.
• Inclusion of histologically confirmed P-PGL cases.
• Descriptive analysis of extracted data on demographics, clinical, biochemical, imaging, treatment, and follow-up.

Main Results:

• 50 cases identified, predominantly in middle-aged adults with a slight female predominance.
• Most tumors were non-functioning and incidentally discovered; some presented with catecholamine excess symptoms.
• Imaging findings were heterogeneous, often indistinguishable from pancreatic neuroendocrine tumors; surgical resection was primary treatment with favorable outcomes.

Conclusions:

• P-PGL presents diagnostic challenges due to nonspecific features.
• Histopathology and immunohistochemistry are crucial for definitive diagnosis.
• Surgical resection is the treatment of choice, generally yielding a good prognosis; greater awareness and reporting are needed.