Open Access

A case of carcinoma of the papilla of Vater in a young man after subtotal colectomy for familial adenomatous polyposis

  • Shuji Komori1Email authorView ORCID ID profile,
  • Masahiko Kawai1,
  • Toyoo Nitta1,
  • Yusuke Murase1,
  • Keita Matsumoto1,
  • Chika Shinoda1,
  • Masashi Kuno1,
  • Yuki Sasaguri1,
  • Masahiro Fukada1,
  • Yoshimi Asano1,
  • Shigeru Kiyama1,
  • Chihiro Tanaka1,
  • Yasuko Nagao1,
  • Narutoshi Nagao1 and
  • Katsuyuki Kunieda1
World Journal of Surgical Oncology201614:47

https://doi.org/10.1186/s12957-016-0806-8

Received: 22 August 2015

Accepted: 17 February 2016

Published: 24 February 2016

Abstract

Background

Carcinoma and adenoma of the duodenum, including the papilla of Vater, are problematic diseases in patients with familial adenomatous polyposis (FAP).

Case presentation

A 36-year-old man underwent a periodic medical examination for early colon cancer originating from FAP for which laparoscopic-assisted subtotal colectomy with a J-shaped ileal pouch-rectal anastomosis was performed 3 years earlier. A tumor was detected at the papilla of Vater along with elevation of total bilirubin and hepatobiliary enzymes. Although cytology did not determine the tumor to be an adenocarcinoma, we suspected adenocarcinoma due to its hypervascularity shown by contrast-enhanced computed tomography. Pylorus-preserving pancreaticoduodenectomy with modified Imanaga reconstruction and regional lymph node dissection (D2) was performed. The pathological study showed that the tumor was a papillary and moderately differentiated tubular adenocarcinoma. The patient is currently in good health without recurrence, weight loss, or severe diarrhea at 12 months after surgery.

Conclusions

Awareness of biliary-pancreatic symptoms and periodic gastroduodenoscopy might contribute both to the early detection of duodenal or periampullary polyps and cancer and to the radical treatment of FAP. Modified Imanaga reconstruction has the potential to become one of the more effective procedures for providing good quality of life to FAP patients with duodenal or periampullary cancer.

Keywords

Familial adenomatous polyposisDuodenal and ampullary cancerModified Imanaga reconstructionPancreaticoduodenectomy

Background

Carcinoma and adenoma of the duodenum, including the papilla of Vater, are problematic diseases in patients with familial adenomatous polyposis (FAP), especially as duodenal polyps were detected in 65 % of FAP patients with a median age of 38 years [1]. Duodenal adenoma changes to adenocarcinoma via the adenoma-carcinoma sequence, but the incidence rate is only approximately 5 % of all polyps [24]. The establishment of endoscopic management and treatment of patients with FAP based on the Spigelman scoring system contributes to surveillance alone or to endoscopic mini-invasive treatment of stage 0–III patients, although even now, almost all patients with stage IV disease and cancer require short-term surveillance or endoscopic treatment or surgery [5].

For stage IV patients, several treatments, including endoscopic mucosal resection, ampullectomy, and pancreas-preserving duodenectomy (PpD)/pylorus-preserving pancreaticoduodenectomy (PpPD), have been discussed and recommended [57]; however, the patients managed with endoscopic resection of adenomas continue to be at substantial risk of developing recurrent adenomas [7]. For patients with duodenal or ampullary cancer, PpD/PpPD is selected in most cases [6, 8, 9].

We report a case of the onset of cancer of the papilla of Vater in a young man after subtotal colectomy for FAP. The available diagnostic and therapeutic strategies for surgical treatment are also discussed in light of the experience with the present case and in reference to previously reported cases.

Case presentation

A 36-year-old man underwent a periodic medical examination for early colon cancer originating from classical FAP (adenomatous polyposis coli (APC) gene in 5q21 analysis, codon 1–1800: exon 15, codon 795, 1 bp deletion of C: CTC→CT) for which laparoscopic-assisted subtotal colectomy with a J-shaped ileal pouch-rectal anastomosis was performed 3 years earlier. His mother also has FAP and the same gene mutation as with his subtype and gene and underwent the same operation 13 years earlier and resection of an intra-abdominal desmoid tumor 10 years earlier (Fig. 1).
Fig. 1

The patient’s family tree

Enhanced computed tomography (CT) showed a hypervascular tumor, 10 mm in diameter, at the papilla of Vater and dilation of the common bile duct (CBD) and the intrahepatic bile duct (Fig. 2ac), and therefore, detailed studies were performed. Results of laboratory blood tests showed high values of aspartate aminotransferase (169 IU/l), alanine aminotransferase (164 IU/l), alkaline phosphatase (755 IU/l), γ-glutamyltransferase (452 IU/l), total bilirubin (1.96 mg/dl), and pancreatic amylase (382 IU/l).
Fig. 2

Enhanced CT image showed a hypervascular tumor, 10 mm in diameter, at the papilla of Vater (arrow) (a) and dilation of the common and intrahepatic bile ducts (arrow) (b, c)

Magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography (MRCP) showed the tumor as a defect present from the papilla of Vater to the lower CBD (Fig. 3a, b). Gastroduodenoscopy showed a large number of gastric and duodenal polyps and a duodenal ulcer nearby the papilla of Vater, which were suspected to be tumor invasion (Fig. 4a, b). Moreover, endoscopic retrograde cholangiopancreatography detected the same findings as those of the MRI and MRCP, and intraductal ultrasonography (IDUS) detected a torose lesion at the lower CBD (Fig. 5a, b). Although a biopsy of the lesion did not reveal any apparent cancer cells, we suspected the tumor to be a malignant tumor of the papilla of Vater because of the hypervascularity shown by CT. No regional lymph node swelling, metastases, or direct invasion to adjacent organs was detected on the chest and abdominal CT images, and there were no other FAP-related findings in other organs. PpPD with modified Imanaga reconstruction, which consists of end-to-side duodenojejunostomy, end-to-side pancreatojejunostomy and choledochojejunostomy, and regional lymph node dissection (D2), were performed.
Fig. 3

An MRI and MRCP showed the tumor as a defect from the papilla of Vater to the lower CBD (arrow) (a, b)

Fig. 4

Gastroduodenoscopy revealed a duodenal ulcer nearby the papilla of Vater (arrow), which was suspected as the site of tumor invasion (a), and a large number of duodenal polyps (dotted arrows) (b)

Fig. 5

Endoscopic retrograde cholangiopancreatography detected findings similar to those of the MRI and MRCP (arrow) (a), and IDUS detected a torose lesion (dotted arrow) at the lower CBD (b)

The specimen obtained showed a circular infiltrating papillary tumor 10 mm in diameter with a shallow ulcer at the papilla of Vater and a large number of polyps in the duodenal second portion (Fig. 6a). The pathological study indicated that the tumor was a papillary and moderately differentiated tubular adenocarcinoma (pStage: II, invasion level: duodenum alone (T2), lymphatic duct invasion: mild, vessel invasion: mild, neural invasion: not detected) (Fig. 6b). Although a large number of adenomas were found in the portion of the duodenum, regional lymph node, liver, and distant metastasis and peritoneal dissemination were not noted (pN0, H0, M0, P0). The patient is currently in good health without recurrence, weight loss, or severe diarrhea at 12 months after surgery.
Fig. 6

The resected specimen showed a 10-mm circular infiltrating papillary tumor (arrow) with a shallow ulcer (dotted arrow) at the papilla of Vater and polyps (arrowheads) in the duodenal second portion (a). The pathological study showed that the tumor was a papillary and moderately differentiated tubular adenocarcinoma (b)

Discussion

Cancer of the duodenum and the papilla of Vater affects the prognosis of patients with FAP. Many of these patients have adenomatous polyps in the second and third portions of the duodenum, and although the polyps remain adenomatous in many cases, 5 % of the polyps change from adenoma to carcinoma via the adenoma-carcinoma sequence [2, 4, 8, 10]. Interestingly, Kadmon et al. reported that no clear association between the number of colon polyps and the number of upper gastrointestinal polyps has been identified [11], whereas the presence of colorectal cancer is related to the increase in duodenal or periampullary cancer in patients with FAP [12]. Therefore, patients with FAP independently need to undergo periodic gastroduodenoscopy and side-viewing endoscopy such as colonoscopy.

Polyps in the periampullary area can cause obstruction of the biliary and pancreatic ducts, resulting in elevation of bilirubin and hepatobiliary pancreatic enzymes, jaundice, or pancreatitis as in our case [10], which suggests that awareness of biliary-pancreatic symptoms yields an opportunity to check for periampullary polyps in patients with FAP along with periodic gastroduodenoscopy. However, this does not diminish the primary importance of periodic medical surveillance for FAP patients.

According to the Spigelman scoring system, several treatments, among which are endoscopic mucosal resection, endoscopic ampullectomy, and prophylactic PpD/PpPD, are applicable to stage IV patients; however more invasive treatments, such as surgical procedures including PpPD, are applicable to cancer patients [58]. The risk of recurrence of both stage IV disease and cancer remains substantial. Especially, less invasive treatments result in high rates of recurrence of adenomas/cancer: rates following ampullectomy for early ampullary cancer range from 10 to 12.8 %, and that following PpD for duodenal adenomas/cancer is 50 % [7, 1214]. Therefore, individual patient characteristics need to be carefully considered when adopting a less invasive treatment.

For duodenal or periampullary cancer, there would be no objection to performing pancreaticoduodenectomy (PD), and thus in many cases, PpPD is performed. Several authors have reported no difference in perioperative morbidity, long-term survival, and other factors between PD and PpPD. However, except for the incidence of delayed gastric emptying, PpPD is better than PD in terms of operating time, blood loss, nutritional status, and capacity to work at 6 months after surgery [1519]. The Whipple or child reconstruction procedure is usually performed to avoid complications of pancreatic fistula (PF), abdominal abscess, or hemorrhage. However, these procedures do not create a physiological route; the modified Imanaga reconstruction is a physiological procedure that offers good long-term nutritional status and allows easier postoperative observation and treatment of the pancreatic/biliary duct [20]. The weak point of the modified Imanaga reconstruction is that it occasionally intensifies the severity of PF or cholangitis more than that by the Whipple or child procedure, and it delays ingestion when PF occurs. Nevertheless, there are at least two benefits of the modified Imanaga reconstruction in FAP: ease of endoscopic intestinal observation/treatment and maintenance of nutritional status after subtotal colectomy for colorectal lesions of FAP. Therefore, we selected the modified Imanaga procedure for our patient. However, after performing the PpPD in our patient, we experienced a patient with duodenal cancer arising from the remaining duodenum after PpPD for ampullary cancer in FAP [21], suggesting that resection of the pylorus and the duodenal bulb with pylorus-resecting PD (PrPD) can remove all of the duodenum and contributes to the prevention of any remaining duodenal cancer in patients with FAP. Moreover, long-term outcomes of PrPD are similar to those of PpPD, and PrPD reduces the incidence of delayed gastric emptying compared with PpPD [2224]. Thus, PrPD with the modified Imanaga procedure might be a better option in patients with FAP. Further evaluation with high-quality prospective studies is necessary.

Conclusions

In the future, expanded oncological, pathological, and surgical knowledge of duodenal or periampullary polyps and cancer associated with FAP might bring many FAP patients more relevant treatment than they receive at present. Although more study is necessary, PrPD with the modified Imanaga reconstruction has the potential to become one of the more effective procedures for the treatment of FAP.

Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Abbreviations

CBD: 

common bile duct

CT: 

computed tomography

FAP: 

familial adenomatous polyposis

IDUS: 

intraductal ultrasonography

MRCP: 

magnetic resonance cholangiopancreatography

MRI: 

magnetic resonance imaging

PD: 

pancreaticoduodenectomy

PF: 

pancreatic fistula

PpD: 

pancreas-preserving duodenectomy

PpPD: 

pylorus-preserving pancreaticoduodenectomy

PrPD: 

pylorus-resecting pancreaticoduodenectomy

Declarations

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

Authors’ Affiliations

(1)
Department of Surgery, Gifu Prefectural General Medical Center

References

  1. Bülow S, Björk J, Christensen IJ, Fausa O, Järvinen H, Moesgaard F, et al. Duodenal adenomatosis in familial adenomatous polyposis. Gut. 2004;53:381–6.PubMed CentralView ArticlePubMedGoogle Scholar
  2. Nugent KP, Spigelman AD, Phillips RK. Life expectancy after colectomy and ileorectal anastomosis for familial adenomatous polyposis. Dis Colon Rectum. 1993;36:1059–62.View ArticlePubMedGoogle Scholar
  3. Nakatsubo N, Kashiwagi H, Okumura M, Kamoshida T, Takahashi A, Spigelman AD. Malignant change in a duodenal adenoma in familial adenomatous polyposis: report of a case. Am J Gastroenterol. 1998;93:1566–8.PubMedGoogle Scholar
  4. Spigelman AD, Talbot IC, Penna C, Nugent KP, Phillips RK, Costello C, et al. Evidence for adenoma-carcinoma sequence in the duodenum of patients with familial adenomatous polyposis. The Leeds Castle Polyposis Group (Upper Gastrointestinal Committee). J Clin Pathol. 1994;47:709–10.PubMed CentralView ArticlePubMedGoogle Scholar
  5. Groves CJ, Saunders BP, Spigelman AD, Phillips RK. Duodenal cancer in patients with familial adenomatous polyposis (FAP): results of a 10 year prospective study. Gut. 2002;50:636–41.PubMed CentralView ArticlePubMedGoogle Scholar
  6. Parc Y, Mabrut JY, Shields C, Mallorca Group. Surgical management of the duodenal manifestations of familial adenomatous polyposis. Br J Surg. 2011;98:480–4.View ArticlePubMedGoogle Scholar
  7. Johnson MD, Mackey R, Brown N, Church J, Burke C, Walsh RM. Outcome based on management for duodenal adenomas: sporadic versus familial disease. J Gastrointest Surg. 2010;14:229–35.View ArticlePubMedGoogle Scholar
  8. Skipworth JR, Morkane C, Raptis DA, Vyas S, Olde Damink SW, Imber CJ, et al. Pancreaticoduodenectomy for advanced duodenal and ampullary adenomatosis in familial adenomatous polyposis. HPB (Oxford). 2011;13:342–9.View ArticleGoogle Scholar
  9. de Castro SM, van Eijck CH, Rutten JP, Dejong CH, van Goor H, Busch OR, et al. Pancreas-preserving total duodenectomy versus standard pancreatoduodenectomy for patients with familial adenomatous polyposis and polyps in the duodenum. Br J Surg. 2008;95:1380–6.View ArticlePubMedGoogle Scholar
  10. Wallace MH, Phillips RK. Upper gastrointestinal disease in patients with familial adenomatous polyposis. Br J Surg. 1998;85:742–50.View ArticlePubMedGoogle Scholar
  11. Kadmon M, Tandara A, Herfarth C. Duodenal adenomatosis in familial adenomatous polyposis coli. A review of the literature and results from the Heidelberg Polyposis Register. Int J Colorectal Dis. 2001;16:63–75.View ArticlePubMedGoogle Scholar
  12. van Heumen BW, Nieuwenhuis MH, van Goor H, Mathus-Vliegen LE, Dekker E, Gouma DJ, et al. Surgical management for advanced duodenal adenomatosis and duodenal cancer in Dutch patients with familial adenomatous polyposis: a nationwide retrospective cohort study. Surgery. 2012;151:681–90.View ArticlePubMedGoogle Scholar
  13. Yoon YS, Kim SW, Park SJ, Lee HS, Jang JY, Choi MG, et al. Clinicopathologic analysis of early ampullary cancers with a focus on the feasibility of ampullectomy. Ann Surg. 2005;242:92–100.PubMed CentralView ArticlePubMedGoogle Scholar
  14. Kawabata Y, Ishikawa N, Moriyama I, Tajima Y. What is an adequate surgical management for pTis and pT1 early ampullary carcinoma? Hepatogastroenterology. 2014;61:12–7.PubMedGoogle Scholar
  15. Seiler CA, Wagner M, Bachmann T, Redaelli CA, Schmied B, Uhl W, et al. Randomized clinical trial of pylorus-preserving duodenopancreatectomy versus classical Whipple resection-long term results. Br J Surg. 2005;92:547–56.View ArticlePubMedGoogle Scholar
  16. Karanicolas PJ, Davies E, Kunz R, Briel M, Koka HP, Payne DM, et al. The pylorus: take it or leave it? Systematic review and meta-analysis of pylorus-preserving versus standard whipple pancreaticoduodenectomy for pancreatic or periampullary cancer. Ann Surg Oncol. 2007;14:1825–34.View ArticlePubMedGoogle Scholar
  17. Tran KT, Smeenk HG, van Eijck CH, Kazemier G, Hop WC, Greve JW, et al. Pylorus preserving pancreaticoduodenectomy versus standard Whipple procedure: a prospective, randomized, multicenter analysis of 170 patients with pancreatic and periampullary tumors. Ann Surg. 2004;240:738–45.PubMed CentralView ArticlePubMedGoogle Scholar
  18. Ogata Y, Hishinuma S. The impact of pylorus-preserving pancreatoduodenectomy on surgical treatment for cancer of the pancreatic head. J Hepatobiliary Pancreat Surg. 2002;9:223–32.View ArticlePubMedGoogle Scholar
  19. Niedergethmann M, Shang E, Farag Soliman M, Saar J, Berisha S, Willeke F, et al. Early and enduring nutritional and functional results of pylorus preservation vs classic Whipple procedure for pancreatic cancer. Langenbecks Arch Surg. 2006;391:195–202.View ArticlePubMedGoogle Scholar
  20. Ohtsuka T, Tanaka M, Miyazaki K. Gastrointestinal function and quality of life after pylorus-preserving pancreatoduodenectomy. J Hepatobiliary Pancreat Surg. 2006;13:218–24.View ArticlePubMedGoogle Scholar
  21. Murakami Y, Uemura K, Sasaki M, Morifuji M, Hayashidani Y, Sudo T, et al. Duodenal cancer arising from the remaining duodenum after pylorus-preserving pancreatoduodenectomy for ampullary cancer in familial adenomatous polyposis. J Gastrointest Surg. 2005;9:389–92.View ArticlePubMedGoogle Scholar
  22. Kawai M, Tani M, Hirono S, Miyazawa M, Shimizu A, Uchiyama K, et al. Pylorus ring resection reduces delayed gastric emptying in patients undergoing pancreatoduodenectomy: a prospective, randomized, controlled trial of pylorus-resecting versus pylorus-preserving pancreatoduodenectomy. Ann Surg. 2011;253:495–501.View ArticlePubMedGoogle Scholar
  23. Kawai M, Tani M, Hirono S, Okada K, Miyazawa M, Yamaue H. Pylorus-resecting pancreaticoduodenectomy offers long-term outcomes similar to those of pylorus-preserving pancreaticoduodenectomy: results of a prospective study. World J Surg. 2014;38:1476–83.View ArticlePubMedGoogle Scholar
  24. Yang C, Wu HS, Chen XL, Wang CY, Gou SM, Xiao J, et al. Pylorus-preserving versus pylorus-resecting pancreaticoduodenectomy for periampullary and pancreatic carcinoma: a meta-analysis. PLoS One. 2014;9:e90316.PubMed CentralView ArticlePubMedGoogle Scholar

Copyright

© Komori et al. 2016

Advertisement