Cervical adenopathy as first symptom of a neuroendocrine ampullary tumor.

Non-pancreatic gastrointestinal neuroendocrine tumors (NETs) include a wide group of diseases mainly located in the ileum and appendix and usually with no clinical signs of hormone hypersecretion. At least 140 cases have been reported in the ampulla of Vater, whose symptoms usually derive from ampullar obstruction and include abdominal pain, pancreatitis, jaundice, or gastrointestinal bleeding. Locoregional nodal metastatic involvement, unrelated to tumor size and not leading to a poorer prognosis, is found in half of these tumors at diagnosis. Cervical nodal involvement by occult intra-abdominal neoplasms (Troisier’s sign), particularly gastric or pancreatic adenocarcinomas, has been known for longer than a century. We report an ampullar NET in which the first sign was a metastatic cervical adenopathy. A 59-year-old woman attended the clinic for a palpable mass in the left side of the neck with no other symptoms. A computed tomography (CT) of the neck showed a 4-cm adenopathy in the left side of the neck that compressed the left thyroid lobe (Fig. 1). Biopsy showed a lymph node infiltrated by a well differentiated NET with immunohistochemistry (IHC) positive for chromogranin A (CgA) and synaptophysin and negative for calcitonin. Thyroid ultrasonography showed no changes. Laboratory tests showed normal serum levels of neuroendocrine tumor markers (CgA, gastrin, PP, somatostatin, glucagon, and calcitonin), and elevated transaminase and alkaline phosphatase levels (AST 75 U/L, NR < 32; ALT 82 U/L, NR < 32; GT 1167 U/L, NR < 33; FA 279 U/L, NR < 36) and normal bilirubin. Abdominal CT revealed a dilated bile duct (15 mm) with prominent papilla, with no tumor or luminal occupation and pancreatic duct dilation (Fig. 2). An endoscopic ultrasonography showed a hypoechoic mass in the