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Revista Española de Enfermedades Digestivas

Print version ISSN 1130-0108

Rev. esp. enferm. dig. vol.109 n.3 Madrid Mar. 2017 



Unusual presentation of obstructive jaundice

Una forma poco común de presentación de ictericia obstructiva



Key words: Neuroendocrine tumor. Vater's ampolla.

Palabras clave: Tumor neuroendocrino. Ampolla de Vater.



Dear Editor,

Ampullary neuroendocrine tumors (NETs) are exceptional. Fewer than 200 cases were described in the ampulla of Vater (1). The WHO classification includes: grade 1 (G1), grade 2 (G2), and grade 3 (G3) NETs. Below we describe a case of ampullary G2 NET according to this classification (2).


Case report

A 57-year-old woman presented at our gastroenterology clinic with painless jaundice, choluria, and acholia of several weeks' duration. Laboratory test results highlighted an elevated total bilirubin at 9.1 mg/dl and direct bilirubin at 7.4 mg/dl.

Abdominal ultrasound was inconclusive, and a subsequent abdominal CT scan revealed a 2-cm pancreatic tumor adjacent to the ampulla, with adenopathies identified in the upper retroperitoneum and adjacent mesentery. A magnetic resonance cholangiopancreatography (MRCP) procedure was performed, which revealed a large choledochal dilation of 20 mm, with the duct tapering into a 2-cm mass at the papilla (Fig. 1A). A conventional endoscopic image obtained with EUS showed a prominent papilla with a friable area surrounding the orifice (Fig. 1B). The endosonographic image obtained with EUS showed a hypoechoic nodule that was punctured using a 25G needle in the presence of a pathologist, who ascertained after two passes the adequacy of the sample, which was suggestive of a NET. An endoscopic retrograde cholangiopancreatography (ERCP) procedure was also performed to deliver plastic stents. Finally, an octreoscan study (Fig. 1C) was performed after histological confirmation of biopsy findings (Fig. 1D), which showed abnormal tracer deposition at the periampullary region, with positivity for the presence of somatostatin receptors.



A CDP was undertaken, with malignancy-negative resection margins documented in the final pathology report. The final diagnosis after surgery was a grade 2 neuroendocrine tumor at the ampullary region, with the absence of metastasis both in the interaortocaval lymphatic-fatty tissue and liver hilar lymph nodes.


Alejandra Gordillo-Hernández, Ángel Nogales-Muñoz and Fernando Oliva-Mompeán
Department of General Surgery and Digestive Diseases.
Hospital Universitario Virgen Macarena. Sevilla, Spain



1. Calles LA, Iglesias Eider Etxeberría N, Goñi F, et al. Cervical adenopathy as first symptom of a neuroendocrine ampullary tumor. Endocrinol Nutr 2014;61(9):493-5. DOI: 10.1016/j.endoen.2014.09.004.         [ Links ]

2. Jernman J, Välimäki MJ, Louhimo J, et al. The novel WHO 2010 classification for gastrointestinal neuroendocrine tumors correlates well with the metastatic potential of rectal neuroendocrine tumors. Neuroendocrinol 2012;95(4):317-24. DOI: 10.1159/000333035.         [ Links ]