Florou E, Velayutham A, Zen Y, Waters J, Srinivasan P. Size-biology paradox in ampullary tumours - large-cell neuroendocrine carcinoma of the ampulla of Vater: A case report. World J Clin Cases 2026; 14(12): 120145 [DOI: 10.12998/wjcc.v14.i12.120145]
Corresponding Author of This Article
Evangelia Florou, MD, Department of Hepato-Pancreato-Biliary Surgery, King’s College Hospital, Denmark Hill, London SE5 9RS, United Kingdom. e.florou@nhs.net
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Surgery
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Apr 26, 2026 (publication date) through Apr 15, 2026
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World Journal of Clinical Cases
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Florou E, Velayutham A, Zen Y, Waters J, Srinivasan P. Size-biology paradox in ampullary tumours - large-cell neuroendocrine carcinoma of the ampulla of Vater: A case report. World J Clin Cases 2026; 14(12): 120145 [DOI: 10.12998/wjcc.v14.i12.120145]
World J Clin Cases. Apr 26, 2026; 14(12): 120145 Published online Apr 26, 2026. doi: 10.12998/wjcc.v14.i12.120145
Size-biology paradox in ampullary tumours - large-cell neuroendocrine carcinoma of the ampulla of Vater: A case report
Evangelia Florou, Abijith Velayutham, Yoh Zen, Justin Waters, Parthi Srinivasan
Evangelia Florou, Abijith Velayutham, Parthi Srinivasan, Department of Hepato-Pancreato-Biliary Surgery, King’s College Hospital, London SE5 9RS, United Kingdom
Yoh Zen, Department of Pathology, King’s College Hospital, London SE5 9RS, United Kingdom
Justin Waters, Department of Medical Oncology, Kent Oncology Centre, Maidstone and Tunbridge Wells NHS Trust, Maidstone, Kent ME16 9QQ, United Kingdom
Co-first authors: Evangelia Florou and Abijith Velayutham.
Author contributions: Florou E and Velayutham A designed the study, drafted the manuscript; Zen Y provided pathological expertise; Waters J and Srinivasan P supervised oncological management; and all authors revised and approved the final manuscript.
Informed consent statement: Written informed consent was obtained from the patient for publication of this case report and accompanying images.
Conflict-of-interest statement: All authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Evangelia Florou, MD, Department of Hepato-Pancreato-Biliary Surgery, King’s College Hospital, Denmark Hill, London SE5 9RS, United Kingdom. e.florou@nhs.net
Received: February 26, 2026 Revised: March 8, 2026 Accepted: March 19, 2026 Published online: April 26, 2026 Processing time: 57 Days and 10.6 Hours
Abstract
BACKGROUND
Ampullary adenocarcinoma is often associated with favourable outcomes due to early presentation and high resectability. Small ampullary lesions are more commonly well-differentiated neuroendocrine tumours, which typically demonstrate indolent behaviour. In contrast, poorly differentiated neuroendocrine carcinomas, including large-cell neuroendocrine carcinoma (LCNEC), are exceptionally rare and biologically aggressive, challenging prognostic assumptions based on tumour size.
CASE SUMMARY
A 69-year-old woman presented with painless obstructive jaundice. Imaging revealed a small ampullary lesion without evidence of metastatic disease. Endoscopic cytology suggested adenocarcinoma, and pancreaticoduodenectomy was performed. Histopathological examination demonstrated LCNEC characterised by large polygonal cells, extensive necrosis, high mitotic activity, and a Ki-67 index up to 70%. Immunohistochemistry was positive for synaptophysin and chromogranin A. The patient completed six cycles of adjuvant carboplatin and etoposide and remains disease-free at 8 months postoperatively.
CONCLUSION
This case highlights the size-biology paradox in ampullary tumours, where small lesions may harbour highly aggressive histology. LCNEC of the ampulla of Vater remains rare and carries a potentially poor prognosis, warranting accurate pathological diagnosis and multidisciplinary management.
Core Tip: Large-cell neuroendocrine carcinoma (LCNEC) of the ampulla of Vater is a rare and aggressive malignancy that may present as a small lesion but requires oncologically intensive management. This striking discordance between tumour size, anatomical location, and biological behaviour underpins the size-biology paradox observed in ampullary LCNEC and highlights the importance of accurate histopathological classification and multidisciplinary oncological management.
