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Non-familial double malignancy of the colon and ampulla of Vater: a case report and review of literature.
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MedLine Citation:
PMID:  22421722     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Apart from their association in familial and hereditary cancer syndromes, sporadic double malignancies of the gastrointestinal tract involving the ampulla of Vater and colon are extremely rare. Although synchronous resection of the two by adding a colectomy to a pancreaticoduodenctomy can be accomplished with minimal increase in the surgical morbidity, a few patients, however, are best managed by a staged resection. We report a case of sporadic double malignancy of the ampulla of Vater and right colon who despite the best attempts continued to bleed and remained malnourished and was successfully managed by staged right hemicolectomy followed by a pancreaticoduodenectomy.
Authors:
Rajesh Rajalingam; Amit Javed; Ranjana Gondal; Asit Arora; Hirdaya H Nag; Anil K Agarwal
Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology Association     Volume:  18     ISSN:  1998-4049     ISO Abbreviation:  Saudi J Gastroenterol     Publication Date:    2012 Mar-Apr
Date Detail:
Created Date:  2012-03-16     Completed Date:  2012-07-24     Revised Date:  2013-06-26    
Medline Journal Info:
Nlm Unique ID:  9516979     Medline TA:  Saudi J Gastroenterol     Country:  India    
Other Details:
Languages:  eng     Pagination:  143-5     Citation Subset:  IM    
Affiliation:
Department of Gastrointestinal Surgery and Pathology, G B Pant Hospital and Maulana Azad Medical College, New Delhi, India.
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MeSH Terms
Descriptor/Qualifier:
Adenocarcinoma / surgery*
Aged
Ampulla of Vater*
Cecal Neoplasms / pathology
Colectomy
Colonic Neoplasms / surgery*
Common Bile Duct Neoplasms / surgery*
Humans
Male
Neoplasms, Multiple Primary / surgery*
Pancreaticoduodenectomy
Parenteral Nutrition
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Journal Information
Journal ID (nlm-ta): Saudi J Gastroenterol
Journal ID (iso-abbrev): Saudi J Gastroenterol
Journal ID (publisher-id): SJG
ISSN: 1319-3767
ISSN: 1998-4049
Publisher: Medknow Publications & Media Pvt Ltd, India
Article Information
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Copyright: © Saudi Journal of Gastroenterology
open-access:
Received Day: 06 Month: 3 Year: 2011
Accepted Day: 06 Month: 7 Year: 2011
Print publication date: Season: Mar-Apr Year: 2012
Volume: 18 Issue: 2
First Page: 143 Last Page: 145
ID: 3326977
Publisher Id: SJG-18-143
PubMed Id: 22421722
DOI: 10.4103/1319-3767.93823

Non-Familial Double Malignancy of the Colon and Ampulla of Vater: A Case Report and Review of Literature
Rajesh Rajalingamaff1
Amit Javedaff1
Ranjana Gondalaff1
Asit Aroraaff1
Hirdaya H. Nagaff1
Anil K. Agarwalaff1
Departments of Gastrointestinal Surgery and Pathology, G. B. Pant Hospital and Maulana Azad Medical College, New Delhi, India
Correspondence: Address for correspondence: Dr. Anil K. Agarwal, Department of Gastrointestinal Surgery, Room No. 223, Academic Block, 2nd Floor, G. B. Pant Hospital, New Delhi - 110 002, India. E-mail: aka.gis@gmail.com

Synchronous double malignancies of the gastrointestinal tract are rare. Although ampullary tumors are reported to occur in the presence of familial cancer syndromes such as familial adenomatous polyposis (FAP), hereditary non-polyposis colon cancer (HNPCC), and Gardner's syndrome, sporadic double malignancies involving the ampulla of Vater and colon are extremely uncommon.[1] Older age, environmental factors (such as diet and smoking), and adenoma-carcinoma sequence involving genetic mutations of oncogenes and tumor suppressor genes have been implicated as possible risk factors.[2] We report a patient of synchronous adenocarcinoma of the ampulla of Vater and ascending colon who was successfully managed by staged operations.


CASE REPORT

A 72-year-old man presented to our department with complaints of painless jaundice and passage of maroon-colored stools for the past 2 months. He also complained of pruritus, decreased appetite, and significant weight loss in the same period. There was no significant past or family history of malignancy of the bowel or the biliary tract. On examination, the patient was pale, icteric, and had bilateral pitting pedal edema. Abdominal examination was unremarkable except for mild hepatomegaly. His hematological and biochemical investigations showed a hemoglobin of 6.2 g/dL, bilirubin 6.8 mg/dL, alkaline phosphatase 1589 IU/L, and serum albumin 2.2 g/dL. On imaging with ultrasound followed by contrast-enhanced CT scan of the abdomen, bilateral intrahepatic biliary radical dilatation, dilated common bile duct up to the lower end, and a non-dilated main pancreatic duct were found [Figure 1a]. There was thickening of the right colon with multiple mesocolic lymph nodes and no evidence of liver metastases [Figure 1b]. A side-viewing duodenoscope demonstrated an ulcerated and bulky ampulla of Vater, the biopsy from which revealed a well-differentiated adenocarcinoma. On colonoscopy [Figure 2], a large constricting proliferative growth was detected in the ascending colon with a small pedunculated polyp about 2 cm proximal to it. The remaining colon was normal. Biopsy of the colonic growth revealed a moderately differentiated adenocarcinoma. His CEA and CA 19-9 levels were 5 (0–5.5 ng/mL) and 77.7 (0–39 IU/mL), respectively. A diagnosis of synchronous carcinoma of the colon and ampulla of Vater was made. In view of his suboptimal nutritional status, the patient was stented (with a plastic biliary stent) and started on high-protein diet supplemented with parenteral nutrition. However, despite 2 weeks of nutritional support, the serum albumin remained low and the patient continued to require blood transfusions due to bleeding from the colonic lesion. Hence, a decision was taken to perform a staged resection. A right hemicolectomy was done as the first operation via a midline incision. Histopathology revealed a multicentric moderately differentiated adenocarcinoma of the cecum (T3) and ascending colon (T2) with a tubular adenoma close to the ascending colonic lesion. The resection margins were free and two lymph nodes showed tumor metastases. Following discharge the patient had significant improvement in his nutritional status (a weight gain of 10 kg, improved serum albumin to 4.2 g/dL, and stabilization of hemoglobin) and was readmitted 6 weeks later for the second-stage surgery. A pancreaticoduodenectomy was done via the prior midline incision and the patient had an uneventful postoperative recovery. Histopathology of the resected specimen revealed a moderately differentiated adenocarcinoma of the ampulla (pT2). All the resection margins were free from tumor with no nodal metastases. Patient was started on 5-flurouracil and oxaliplatin-based chemotherapy. At the last follow-up of 8 months, patient is asymptomatic and disease-free.


DISCUSSION

Double malignancies of the gastrointestinal tract are very rare. In a review of more than 2000 patients, Minni et al. found the incidence of a second primary tumor in gastrointestinal tract to be 4.3%.[3] This association most often occurs in patients with familial cancer syndromes. Patients with hereditary colon cancer syndromes such as FAP, its attenuated variant, and HNPCC have an increased life time risk of developing cancer of the ampulla in addition to various other sites.[4]

Sporadic variants of both colonic and ampullary adenocarcinomas occur in a step-wise fashion involving activation of oncogenes (K-ras, Her-2/neu) and suppression of tumor suppressor genes (APC, DCC, p53, DPC4, BRCA2).[5, 6] This results in a sequential transformation of the normal colonic and ampullary epithelial cells to an adenoma and finally carcinoma. The synchronous occurrence of the two malignancies in this patient may be explained by activation of a common oncogene or inhibition of a common tumor suppressor gene involved in this pathway. A recent study has also shown a higher risk of colorectal adenomas and malignancy in patients with sporadic duodenal adenomas.[7] In another analysis of the surveillance epidemiology and end results database, Das et al.[8] reported a two-fold increase in the risk for the development of colorectal cancer in a patient with ampullary malignancy and vice versa. Although a detailed genetic evaluation of this patient for MMR mutations would have been worthwhile, this facility was not available to us and hence it was not done.

To the best of our knowledge, only six cases of sporadic synchronous ampullary and colorectal malignancies have been reported till date [Table 1]. The mean age of these patients was 67.5±12.4 years with predominance of males. The first case was reported in 1990 where sigmoid colon malignancy was incidentally detected during the surgical resection of an ampullary tumor.[9] Interestingly, this patient had two adenomas near the colonic lesion similar to our patient. This might favor a common adenoma-carcinoma hypothesis. In the majority of these case reports as tabulated, the two tumors were managed by simultaneous resections (pancreaticoduodenectomy/ampullectomy with colonic resection) which can be accomplished via the same incision. Similarly in our patient the initial plan was to improve the nutritional status and perform a simultaneous resection. However, despite 2 weeks of aggressive attempts to improve the nutritional and performance status, the serum albumin continued to remain low, and he had persistent bleeding from the colonic tumor with a drop in hemoglobin necessating blood transfusions. Hence a decision was taken to do a right hemicolectomy as the first part of the staged resection as this was thought to be of lesser magnitude than a pancreaticoduodenectomy, would prevent further blood loss (from the colonic tumor), and may allow the patient to be nutritionally optimized for a subsequent pancreaticoduodenectomy. As expected, the patient had remarkable improvement in his nutritional status and gained 10 kg weight over a period of 6 weeks. He could thus be taken up for a pancreaticoduodenectomy which could have been hazardous in the first stage. Although a staged resection (pancreaticoduodenectomy followed by a right hemicolectomy 3 weeks later) was performed by Gonzalez et al. in a similar situation, we believe that a patient who can undergo a major procedure such as a pancreaticoduodenectomy at the initial operation could have also undergone an additional right hemicolectomy in the same sitting, via the same incision, without substantially increasing the morbidity of the procedure.

To conclude, sporadic double malignancies are rare but should be kept in mind while evaluating a patient with cancer. Simultaneous resection is the preferred approach in most, however a staged procedure may be done in select high-risk surgical patients.


Notes

Source of Support: Nil

Conflict of Interest: None declared.

REFERENCES
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2. Esposito I,Friess H,Büchler MW. Carcinogenesis of cancer of the papilla and ampulla: Pathophysiological facts and molecular biological mechanismsLangenbecks Arch SurgYear: 20013861637111382316
3. Minni F,Casadei R,Marrano N,Guerra E,Piccoli L,Pagogna S,et al. Second tumours in patients with malignant neoplasms of the digestive apparatus. A retrospective study on 2406 casesAnn Ital ChirYear: 2005764677216696221
4. Björk J,Akerbrant H,Iselius L,Bergman A,Engwall Y,Wahlström J,et al. Periampullary adenomas and adenocarcinomas in familial adenomatous polyposis: Cumulative risks and APC gene mutationsGastroenterologyYear: 200112111273511677205
5. Fearon ER. Vogelstein B: A genetic model for colorectal tumorigenesisCellYear: 199061759672188735
6. Wilentz RE,Iacobuzio-Donahue CA,Argani P,McCarthy DM,Parsons JL,Yeo CJ,et al. Loss of expression of DPC4 in pancreatic intraepithelial neoplasia: Evidence that DPC4 inactivation occurs late in neoplastic progressionCancer ResYear: 2000602002610766191
7. Lagarde S,Dauphin M,Delmas C,Vitry F,Bouché O,Thiéfin G,et al. Increased risk of colonic neoplasia in patients with sporadic duodenal adenomaGastroenterol Clin BiolYear: 200933441519278801
8. Das A,Neugut AI,Cooper GS,Chak A. Association of ampullary and colorectal malignanciesCancerYear: 20041005243014745868
9. Yoshida J,Morisaki T,Yamaguchi K,Itoh T,Yokohata K,Kishikawa H,et al. Carcinoma in adenoma of the ampulla of Vater synchronous with cancer of the sigmoid colonDig Dis SciYear: 19903527152406114
10. González Sánchez JA,López-Ríos Fernández F,González Conde R,Miguel Velasco JE,Casas Pinillos MS,Larrauri Martínez J. Carcinoma of the Vater's ampulla and colonic carcinoma with synchronous onsetRev Esp Enferm DigYear: 199383459618338711
11. Kunkel D,Moreau X,de Jaureguiberry JP,Pouderoux P. Ampullar, peri-ampullar and colonic tumors: An association not to be disregarded? Apropos of 3 casesGastroenterol Clin BiolYear: 19901451232365181
12. Eriguchi N,Aoyagi S,Tamae T,Nishimura K,Hamada S,Kawabata M,et al. Carcinoma of the ampulla of Vater associated with other organ malignanciesKurume Med JYear: 200148255911830924
13. Schneider AR,Seifert H,Trojan J,Stein J,Hoepffner NM. Frequency of colorectal polyps in patients with sporadic adenomas or adenocarcinomas of the papilla of vater-an age matched, controlled studyZ GastroenterolYear: 2005431123716220451

Figures

[Figure ID: F1]
Figure 1a 

Contrast-enhanced CT abdomen showing distended gallbladder with dilated intrapancreatic common bile duct and an ampullary lesion (arrow)



[Figure ID: F2]
Figure 1b 

Contrast-enhanced CT scane of the abdomen showing mural thickening in the ascending colon (arrow)



[Figure ID: F3]
Figure 2 

Colonoscopy showing ulceroproliferative lesion in the ascending colon with a polyp 2cm proximal to it (arrow)



Tables
[TableWrap ID: T1] Table 1 

Previous reports of sporadic synchronous ampullary and colorectal malignancies




Article Categories:
  • Case Report

Keywords: Ampulla of Vater, colon, neoplasms.

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