SHM Converge Scientific Abstract ePoster Gallery-The Klatskin Imitator: Perihilar Neuroendocrine Tumor Masquerading as Cholangiocarcinoma in a Young Adult

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This case describes a perihilar neuroendocrine tumor (NET) that initially mimicked hilar cholangiocarcinoma, also known as a Klatskin tumor. The patient presented with a high-grade hilar stricture and dilated intrahepatic ducts, findings that strongly suggested cholangiocarcinoma on initial imaging and clinical assessment.<br /><br />ERCP was crucial in establishing the diagnosis. It demonstrated the stricture and allowed advanced tissue sampling before definitive surgery, leading to preoperative identification of the lesion as a NET rather than cholangiocarcinoma. Functional imaging further supported localized disease. The patient underwent complete macroscopic resection, and pathology showed a well-differentiated tumor with no metastatic disease.<br /><br />Because resection was complete and there was no evidence of spread, no adjuvant therapy was given. The planned follow-up includes surveillance cross-sectional imaging and endoscopic evaluation to rule out an occult gastrointestinal primary source.<br /><br />The case highlights several important points:<br />- Perihilar NETs can closely resemble cholangiocarcinoma in symptoms and imaging.<br />- ERCP with tissue biopsy, combined with functional imaging, can enable earlier and more accurate diagnosis.<br />- Early recognition may prevent unnecessary liver transplant evaluation and help streamline management.<br /><br />It also emphasizes the difference in treatment approaches: cholangiocarcinoma may require surgery, chemotherapy, radiation, and sometimes liver transplantation, whereas surgery alone can be curative for a NET.

Asset Subtitle

Sam Westpy

Meta Tag

Author List Purven Parikh, Sam Westpy, Sohil Khanna

Category Clinical Vignettes

Concept Perihilar Neuroendocrine Tumor

Concept Hilar Cholangiocarcinoma

Concept Endoscopic Retrograde Cholangiopancreatography

Concept Tissue Diagnosis

Concept Hilar Stricture

Distinguished Non-Finalist

Presenter Organization MedStar Georgetown University Hospital

Presenting Author Sam Westpy

Track Adult

Keywords

perihilar neuroendocrine tumor

hilar cholangiocarcinoma

Klatskin tumor

ERCP

hilar stricture

intrahepatic duct dilation

tissue biopsy

functional imaging

surgical resection

surveillance imaging

Perihilar Neuroendocrine Tumor

Hilar Cholangiocarcinoma

Endoscopic Retrograde Cholangiopancreatography

Tissue Diagnosis

Hilar Stricture