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Gangliocytic Paraganglioma of the Duodenum: A Masquerader

Background: Gangliocytic paraganglioma (GP) is a rare tumor that most commonly arises from the duodenum and is characterized pathologically by 3 cell types: epithelioid, spindle, and ganglion cells. GP is often difficult to differentiate from a neuroendocrine tumor on the basis of preoperative imagi...

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Detalles Bibliográficos
Autores principales: Jain, Vishu, Selvakumar, B, Varshney, Vaibhav Kumar, Vishwajeet, Vikarn, Taywade, Sameer, Agarwal, Lokesh, Yadav, Taruna, Pandey, Rakesh
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Academic Division of Ochsner Clinic Foundation 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10498948/
https://www.ncbi.nlm.nih.gov/pubmed/37711472
http://dx.doi.org/10.31486/toj.23.0010
Descripción
Sumario:Background: Gangliocytic paraganglioma (GP) is a rare tumor that most commonly arises from the duodenum and is characterized pathologically by 3 cell types: epithelioid, spindle, and ganglion cells. GP is often difficult to differentiate from a neuroendocrine tumor on the basis of preoperative imaging, and the diagnosis is based on final histopathologic and immunohistochemical analysis. Case Report: We report the case of a 28-year-old male who presented with pain in the abdomen, bilious vomiting, and weight loss. Imaging showed a mass involving the first and second part of the duodenum that was likely a neuroendocrine or gastrointestinal stromal tumor. He underwent robotic-assisted pancreatoduodenectomy, and the final pathology report identified GP with lymph node metastasis. The patient was doing well at 1-year follow-up. Conclusion: GP is often a histologic surprise as most cases are diagnosed in postoperative histopathology. While GP has a more benign course than a neuroendocrine tumor, radical surgical resection is warranted in cases of diagnostic dilemma, suspicion of malignancy, or lymph node metastasis. Robotic-assisted pancreatoduodenectomy is a feasible option.