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Peripancreatic paraganglioma: Lesson from a round table
Federica Petrelli1, Geri Fratini2, Andrea Sbrozzi-Vanni3
1Pathology Unit, Azienda Sanitaria Toscana Nord Ovest, Pisa 56121, Italy.
World Journal of Gastroenterology
|July 8, 2022
Summary
A rare peripancreatic paraganglioma was misdiagnosed as a pancreatic lesion due to imaging limitations. Accurate diagnosis requires careful pathological review, especially in emergency settings.
Area of Science:
- Oncology
- Pathology
- Radiology
Background:
- Peripancreatic paragangliomas (PGLs) are rare neuroendocrine tumors that can mimic pancreatic lesions.
- Accurate pre-operative diagnosis of PGLs is challenging due to their location and potential for misinterpretation on imaging.
Observation:
- A case of a peripancreatic PGL initially misdiagnosed as a pancreatic lesion is presented.
- Surgical exploration revealed a cystic mass originating from the mesocolon root, with an unremarkable pancreas.
- The misdiagnosis was attributed to imaging limitations, including the lack of a clear cleavage plane and the emergency setting.
Findings:
- Histopathological examination confirmed the diagnosis of PGL.
- Two novel pathogenetic mutations, in the KDR and JAK3 genes, were identified.
- A blinded expert panel review highlighted imaging biases, including the thin mesocolon sheet hindering border definition.
Implications:
- This case underscores the difficulties in pre-operative PGL diagnosis and the importance of multidisciplinary review.
- Endoscopic ultrasonography has limitations in diagnosing large peripancreatic masses.
- Accurate diagnosis is crucial for appropriate surgical management and patient outcomes.

