ECE2014 Poster Presentations Endocrine tumours and neoplasia (99 abstracts)
Neuroendocrine cancer of the ampulla of Vater: clinical course in seven patients: material of Department of Endocrinology in Szczecin, Poland
Elzbieta Andrysiak-Mamos 1 , Elzbieta Sowinska-Przepiera 1 , Jerzy Lubikowski 2 , Justyna Syrenicz 1 , Ewa Zochowska 1 , Bartosz Kiedrowicz 1 , Teresa Starzynska 3 & Anhelli Syrenicz 1
1Department of Endocrinology, Metabolic and Internal Diseases, Pomeranian Medical University, Szczecin, Poland; 2Department of Surgery, West Pomeranian Oncology Center, Szczecin, Poland; 3Department of Gastroenterology, Pomeranian Medical University, Szczecin, Poland.
Introduction: Neuroendocrine cancer of the ampulla of Vater area belongs to the most rare GEP-neuroendocrine tumors (NET). In the literature, there are few reports of extremely malignant course of this cancer. Among 242 patients with NET from the Department of Endocrinology in 2009–2013, 58 pancreatic NET were discovered, among which seven patients had neuroendocrine cancer of the ampulla of Vater. The aim of this study was to determine the clinical characteristics, staging, grading and survival rate in patients with such neuroendocrine carcinoma.
Materials/designs: Seven patients were evaluated (5F/2M) aged from 41 to 75 years (mean 58±11.5). The average age of diagnosis was 63±7.4 years.
Results: The most common clinical symptoms were unspecific abdominal pain and jaundice. The average value of Ki67 index was 44.7% (21–82%). Clinical stage was T1M1N0 in three patients, T2M1N0 in two patients and T2M1N1 in two patients. The average concentration of chromogranin A was 138±13.5 ng/ml, with no correlation with the clinical stage of the tumor. All patients had high expression of somatostatin receptors in somatostatin receptor scintigraphy (SRS) and 68Ga-labeled DOTA-TATE PET. The primary focal lesion was revealed in the ampulla of Vater area in the EUS examination with the size range 1.8–3.5 cm (mean 2.4 cm). In three patients that underwent total surgery there was no recurrence of the cancer for 3 years. Four patients did not qualify for radical surgery and therefore underwent chemotherapy with subsequent somatostatin analogues treatment. Next two patients underwent radionuclide treatment due to progression of the disease. The current median survival of these patients is 3.2±0.7 years.
Conclusion: Patients with neuroendocrine carcinoma of the ampulla of Vater demonstrate a high degree of staging and histopathological grading system with scanty and late occurring symptoms. They require long-term treatment and monitoring.
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