Introduction: The neuroendocrine tumor of the ampulla of Vater represent a very rare disease, corresponding up to 2% of the periampullary malignancies and less than 1% of gastrointestinal NET. Less than 130 patients have been reported until 2013. The biological and clinical behavior is very unpredictable especially if its associated with another tumor, like adenocarcinoma. We present a case of a fifty-one-years-old woman who complained of weight loss, jaundice, abdominal pain and anemia. MR-cholangiography revealed an ampullary ulcerated tumor with duodenal invasion, Forrest IIA and biopsy and endoscopic hemostasis was practiced. Also multiple liver tumors were described, the larger one in segment III, about 6 cm. The patient underwent a Whipple procedure and hepatectomy in segments II-III for bulky necrotic metastases. The immunohistochemical study of the neoplasia showed the expression of chromogranin A, synaptophysin, CD56 and also cytokeratin7, CEAm, CA19-9, beta-catenine with a proliferation index Ki67-12%. The TNM classification was pT3N1M1a- stage IV and by WHO was NET well differentiated G2 with adenocarcinoma associated (<10%), non-MANEC. The evolutive CT revealed incomplete thrombosis of right hepatic artery and the Octreoscan showed multiple bone metastases. The patient started chemotherapy with Capecitabine, Lanreotide and Zoledronic acid for bone metastases. Because of the elevated serum markers (chromogranin A, neuron specific enolase, carcinoembryonic antigen), postsurgical complications (acute superior digestive hemorrhage) and disseminated disease (liver, bones) the patient was recommended undergo radioisotope therapy cycle with Lutetium 171/Yttrium 90 in Europe, therapy not currently available in Romania. The particularity of this case of ampullary neuroendocrine tumor associated with adenocarcinoma make more insidious the progress and worsen prognosis of the disease, by being difficult to predict the effects of multisystemic therapy- chemotherapy, somatostatin analogs and newly, peptide receptor-targeted radiotherapy.
Conclusions: Although neuroendocrine tumors of the ampulla of Vater represents a rare disease, it has been increasingly reported in the last years. Radical surgery should be the standard approach. The presence of distant metastases is the most important prognostic factor in determining survival.
18 - 21 May 2019
European Society of Endocrinology