Keywords:
Neuroendocrine tumor; somatostatinoma; papillary tumor; ampullary tumor; endoscopy/treatment; endosonography.
Somatostatinomas are rare neuroendocrine tumors usually associated with Von Recklinghausen’s disease, multiple endocrine neoplasia, or Von Hippel-Lindau syndrome. Nevertheless, it may be associated with sporadic occurrence in healthy patients11. Nesi G, Marcucci T, Rubio CA, Brandi ML, Tonelli F. Somatostatinoma: clinico-pathological features of three cases and literature reviewed. J Gastroenterol Hepatol. 2008;23:521-6.. Approximately 55% of somatostatinomas are pancreatic tumors and the remainder arises in ampullary and periampullary region or, rarely, in jejunum22. Soga J, Yakuwa Y. Somatostatinoma/inhibitory syndrome: a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas. J Exp Clin Cancer Res. 1999;18:13-22.. A patient with pancreatic somatostatinoma usually presents a triad of steatorrhea, weight loss and diabetes mellitus, due to inhibition of gastrointestinal peptides11. Nesi G, Marcucci T, Rubio CA, Brandi ML, Tonelli F. Somatostatinoma: clinico-pathological features of three cases and literature reviewed. J Gastroenterol Hepatol. 2008;23:521-6.,33. Yoshida T, Matsumoto T, Morii Y, Shibata K, Fujii K, Aramaki M, et al. Carcinoid somatostatinoma of the papilla of Vater: a case report. Hepatogastroenterology. 1998;45:451-3.. In duodenal papillary somatostatinomas (DPS), these symptoms occur in less than 10%. Endoscopic retrograde cholangio-pancreatography (ERCP), magnetic resonance or computed tomography can aid in diagnosis, which is only confirmed by psammoma bodies on histologic studies, a rare finding in pancreatic tumors44. Williamson JM, Thorn CC, Spalding D, Williamson RC. Pancreatic and peripancreatic somatostatinomas. Ann R Coll Surg Engl. 2011;93:356-60.. Surgical resection (duodenopancreatectomy) is the treatment of choice. The authors present a case of DPS with cholestasis, endoscopic treated with excellent results and follow-up for 7 years.
44-year-old woman with biliary colic for 1 month and evolving with 1-week jaundice. Bilirubin =14 mg/dL (conjugated =12 mg/ dL), AST, ALT and GGT increased and chromogranin A =6 U/l. ERCP revealed choledochal dilatation, presence of spontaneous choledocoduodenal fistula and a papillary tumor. A 10F plastic prosthesis was inserted through the fistulous orifice and biopsy was performed. The pathology report revealed neuroendocrine tumor. Magnetic Resonance Imaging unveiled a tumor on duodenal papilla (2.0 cm) and three peri duodenal lymph nodes (LN). PET/CT (FDG) revealed hypermetabolic area on duodenal papilla without LN involvement (Figure 1). Endosonography revealed a lesion confined to duodenal papilla without invasion of adjacent structures (uT1) and 3 LN (Figure 2). EUS-FNA of the LN and endoscopic papillectomy were performed for whitish gallstones extraction (Figure 3, E-VIDEO* * E-VIDEO: https://youtu.be/7_Y3LyhPBpQ and Figure 4). The LN pathology was negative for metastasis and papillectomy specimen revealed neuroendocrine tumor of 2.1x1.7x1.3 cm, free margins with vascular invasion. Immunohistochemistry showed a G1 neuroendocrine neoplasia with CD56 (+), synaptophysin (+), chromogranin A (+), Ki-67 of 0.75 and anti-somatostatin antibodies (+). After 7 years of follow-up the patient is in good condition without recurrence.
Abdominal magnetic resonance imaging shows a mass (black arrows) inside the duodenal lumen on topography of duodenal papilla (a, b and c). PET/CT (FDG) shows hypermetabolic area (yellow arrow) on topography of duodenal papilla (descending part of duodenum) (d).
Endoscopic ultrasound. Hypoechoic and homogeneous area with precise limits (white arrows) in duodenal papilla measuring 1.82x0.73 (major axes) (a, b and c). In the image d the yellow arrow points to a perilesional lymph node that was biopsied with a 22G needle, just before the endoscopic papillectomy.
Endoscopic images. (a) plastic prosthesis inserted by spontaneous choledocoduodenal fistula. (b) Apprehension moment of the papilla tumor with polypectomy loop. (c) Image immediately after remove the entire tumor in monoblock. (d) Multiple stones extraction after enlarging the choledocean orifice with the papillotome.
REFERENCES
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1Nesi G, Marcucci T, Rubio CA, Brandi ML, Tonelli F. Somatostatinoma: clinico-pathological features of three cases and literature reviewed. J Gastroenterol Hepatol. 2008;23:521-6.
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2Soga J, Yakuwa Y. Somatostatinoma/inhibitory syndrome: a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas. J Exp Clin Cancer Res. 1999;18:13-22.
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3Yoshida T, Matsumoto T, Morii Y, Shibata K, Fujii K, Aramaki M, et al. Carcinoid somatostatinoma of the papilla of Vater: a case report. Hepatogastroenterology. 1998;45:451-3.
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4Williamson JM, Thorn CC, Spalding D, Williamson RC. Pancreatic and peripancreatic somatostatinomas. Ann R Coll Surg Engl. 2011;93:356-60.
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*
E-VIDEO: https://youtu.be/7_Y3LyhPBpQ
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Disclosure of funding: no funding received
Publication Dates
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Publication in this collection
22 Oct 2021 -
Date of issue
Jul-Sep 2021
History
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Received
04 Feb 2021 -
Accepted
18 Mar 2021