Endocrine Abstracts

Troisier’s sign names a visible left cervical adenopathy (Virchow’s node) as the first sign of a pancreatic tumor, mainly adenocarcinomas, by dissemination through the thoracic duct. Neuroendocrine tumors (NET) of the ampulla of Vater are extremely rare, no more than 2% of ampullary malignancies, and account for <0.3% of all gastrointestinal NETs.

A 59–year old woman presented with an enlarging painless left cervical node. Ultrasonographic guided biopsy of a round-shaped and hypervascular lymph node showed a small round cell proliferation with solid pattern positive for chromogranin A (CgA) but negative for calcitonin. Thyroid was normal in sonography. Laboratory results showed normal levels of tumour markers (CgA, gastrin, PP, somatostatin, glucagon and calcitonin), normal bilirrubin and elevated transaminases, mainly γGT(1167 U/l) and alkaline phosphatase. Abdominal CT revealed a dilated bile-duct (15 mm) with prominent papilla. A hypoechoic mass in the ampulla and periportal adenopathies were evident in endoscopic ultrasonography (EUS), both positive for NET when biopsied. She underwent the Whipple resection with extended lymph node dissection and pathological study showed an ampullar NET of 1.7×1.4 cm invading the duodenal mucosa and pancreas with lymphatic permeation in tumour and preaortic and mesenteric nodes, Ki-67<2%. One month later, a functional cervical left dissection was performed, showing 9/41 lymphatic nodes affected by the NET, the biggest reaching 4.5 cm (Ki-67<2%). At present the patient remains asymptomatic, without evidence of disease neither by CT nor Octreoscan.

Ampullary NETs are very rare tumors, frequently associated with von Recklinghausen’s disease. They usually present as obstructive jaundice without carcinoid syndrome. Our case is a new one of this uncommon tumour with a singular first complaint as a cervical node (Troisier’s sign), not previously described in ampullary NETs. We remark the vast asymptomatic lymphatic extension in spite of the low Ki-67 and size <2 cm of primary tumour.

Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.

Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.