Poorly-Differentiated Signet-Ring Cell Carcinoma of the Ampulla of Vater: Report of a Rare Malignancy
Abstract
Context Signet-ring cell carcinoma (SRCC) of the ampulla of Vater is a very rare clinical entity, which is infrequently reported in medical literature. Case report A 78-year-old woman was admitted with jaundice, pruritus and postprandial vomiting. Abdominal ultrasound and computed tomography scanning demonstrated gross dilatation of the common bile and pancreatic ducts with gallbladder calculi. Endoscopic retrograde cholangiopancreatography suggested a duodenal tumour at the ampulla. The patient underwent Whipple’s procedure with cholecystectomy. Immunohistopathological examination confirmed poorly-differentiated SRCC of the ampulla of Vater. The tumour had infiltrated the duodenal muscularis propria and pancreatic parenchyma, but local lymph nodes were clear (T3N0M0). The patient was disease-free at 6-month follow-up. Conclusions We here report a case of poorly-differentiated SRCC of the Ampulla of Vater. The majority of patients with such tumours undergo pancreaticoduodenectomy, which affords good outcomes in early disease. However, owing to the rarity of cases, the exact prognosis of ampullary SRCC remains as yet undetermined.
Image: Axial slice of the head of the pancreas.
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Gao JM, Tang SS, Fu W, Fan R. Signet-ring cell carcinoma of the ampulla of Vater: contrast-enhanced ultrasound findings. World J Gastroenterol. 2009;15:888-91. [PMID: 19230055]
Sekoguchi T, Mizumoto R. Clinicopathological study of papilla of Vater. Geka Chiryo. 1979;41:1-5.
Gheza F, Ceryi E, Pulcini G, Villanacci V, Giulini SM, Schiavo-Lena M, Ferrari AB, et al. Signet ring cell carcinoma of the ampulla of Vater: demonstration of pancreatobiliary origin. Pancreas. 2011;40:791-3. [PMID: 21673543]
Li L, Chen QH, Sullivan JD, Breuer FU. Signet-ring cell carcinoma of the ampulla of Vater. Ann Clin Lab Sci. 2004;34:471-5. [PMID: 15648791]
Akatsu T, Aiura K, Takahashi S, Kameyama K, Kitajima M, Kitagawa Y. Signet-ring cell carcinoma of the ampulla of Vater : report of a case. Surg Today. 2007;37:1110-4. [PMID: 18030577]
Purohit RC, Kant K, Bhargava N, Kothari N, Purohit V. Signet ring cell carcinoma of ampulla of Vater in a young adult. Indian J Gastroenterol. 2005;24:222-3. [PMID: 16361773]
Taş A, Ozer E, Köklü S, Kocak E. Signet ring cell carcinoma of the ampulla of vater: rare cause of acute pancreatitis. Scand J Gastroenterol. 2011;46:126-7. [PMID: 20950208]
Nabeshima S, Kishihara Y, Nabeshima A, Yamaga S, Kinjo M, Kashiwagi S, Hayashi J. Poorly differentiated adenocarcinoma with signet-ring cells of the Vater’s ampulla, without jaundice but with disseminated carcinomatosis. Fukuoka Igaku Zasshi. 2003;94:235-40. [PMID: 14509231]
Paplomata E, Wilfong L. Signet ring cell carcinoma of the ampulla of Vater with leptomeningeal metastases: a case report. J Clin Oncol. 2011;29:e627-9. [PMID: 21606434]
Ishibashi Y, Ito Y, Omori K, Wakabayashi K. Signet ring cell carcinoma of the ampulla of Vater. A case report. JOP. 2009;10:690-3. [PMID: 19890196]
Ramia JM, Mansilla A, Villar J, Muffak K, Garrote D, Ferron JA. Signet-ring cell carcinoma of the Vater’s ampulla. JOP. 2004;5:495-7. [PMID: 15536289]
Eriguchi N, Aoyagi S, Jimi A. Signet-ring cell carcinoma of the ampulla of Vater: report of a case. Surg Today. 2003;33:467-9. [PMID: 12768376]
Hara T, Kawashima H, Ishigooka M, Kashiyama M, Takanahi S, Hosokawa Y. Signet-ring-cell carcinoma of the ampulla of Vater: a case report. Hepatogastroenterology. 2002;49:561-3. [PMID:11995497]
Valeri S, Caricato M, Ripetti V, Crucitti P, Ausania F, Garberini A, et al. Signet-ring cell carcinoma of the Vater’s ampulla: report of a clinical case. Suppl Tumori. 2005;4:S61. [PMID: 16437905]
Tran KT, Smeenk HG, van Eijck CH, Kazemier G, Hop WC, Greve JC, Terpstra OT et al. Pylorus-preserving pancreaticoduodenectomy versus standard Whipple procedure: a prospective, randomized, multicenter analysis of 170 patients with pancreatic and periampullary tumours. Ann Surg. 2004;240:738-45. [PMID: 15492552]
Diener MK, Fitzmaurice C, Schwarzer G, Seiler CM, Antes G, Knaebel HP, Büchler MW. Pylorus-preserving pancreaticoduodenectomy (pp Whiple) versus pancreaticoduodenectomy (classic Whipple) for surgical treatment of periampullary and pancreatic carcinoma. Cochrane Database Syst Rev. 2011 May 11;(5):CD006053. [PMID 21563148]
Terada T. Primary signet-ring cell carcinoma of the ampulla of Vater: a case report with an immunohistochemical study. Appl Immunohistochem Mol Morphol. 2012;20:427-8. [PMID 22710820]
Zhou J, Hsu CC, Winter JM, Pawlik TM, Laheru D, Hughes MA, et al. Adjuvant chemoradiation versus surgery alone for adenocarcinoma of the ampulla of Vater. Radiother Oncol. 2009;92:244-8. [PMID 19541379]
Narang AK, Miller RC, Hsu CC, Bhatia S, Pawlik TM, Laheru D, et al. Evaluation of adjuvant chemoradiation therapy for ampullary adenocarcinoma: the Johns Hopkins Hospital-Mayo Clinic collaborative study. Radiat Oncol. 2011;28:126. [PMID 21951377]
Kim DI, Park SW, Lee GS, Jung GY, Jung HJ, Moon HC, et al. A Case of Signet-ring Cell Carcinoma of the Ampulla of Vater. Korean J Gastroint Endosc. 2010;41:251-4.
Lesquereux-Martínez L, Fernández-Pérez A, Bustamante-Montalvo M. Signet ring cell adenocarcinoma of the ampulla of Vater: A rare pathology. Rev Esp Enferm Dig. 2012;104:501-2. [PMID 23130863]
Daoudi K, El Haoudi K, Bouyahia N, Benlemlih A, Arifi S, Mellas N, et al. Signet Ring Cell Carcinoma of the Vater’s Ampulla: A Very Rare Malignancy. Case Rep Oncol Med. 2012;2012:402798. [PMID 23056971]
Gardner HA, Matthews J, Ciano PS. A signet-ring cell carcinoma of the ampulla of Vater. Arch Pathol Lab Med. 1990;114:1071-2. [PMID 2171451]
Arnal Monreal FM, Lorenzo Patiño MJ, Sacristán F, Ghanimé Saide G. Signet ring cell carcinoma of the Vater’s ampulla. Rev Esp Enferm Dig. 1994;85:391–3. [PMID 8049111]
Casella R, Rittmann WW, Meier R, Wegmann W, Widmer MK, Hunger T. Signet ring cell carcinoma of Vater’s papilla: a very rare malignancy. Helv Chir Acta. 1994;60:987–90. [PMID 7876027]
Tseng LJ, Jao YT, Mo LR. Signet ring cell carcinoma of major papilla. Gastrointest Endosc. 2002;56:733. [PMID 12397285]
Fang CL, Chu JS, Hsieh MC, Wu MS. Signet-ring cell carcinoma of the ampulla of Vater. J Formos Med Assoc. 2004;103:793–6. [PMID 15490032]
Zhou H, Schaefer N, Wolff M, Fischer HP. Carcinoma of the ampulla of Vater: comparative histologic/immunohistochemical classification and follow-up. Am J Surg Pathol. 2004;28:875-82. [PMID 15223956]
Bloomston M, Walker M, Frankel WL. Radical resection in signet ring carcinoma of the ampulla of Vater: report of an 11-year survivor. Am Surg. 2006;72:193-5. [PMID 16536256]
García AB, Arranz EM, Sanz RR, Serrano EM, Arranz MD, Sanz-Agero PG, et al. Signet ring cell carcinoma of the ampulla of Vater. Gastroenterol Hepatol. 2011;34:141-6. [PMID 21376425]
Maekawa H, Sakurada M, Orita H, Sato K. Signet-ring cell carcinoma co-existing with adenocarcinoma of the ampulla of vater. A case report. JOP. 2011;12:162-6. [PMID 23186645]
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