Primary duodenal adenocarcinoma is extremely rare, accounting for 0.3v0.4% of all gastrointestinal cancers. [1] It is too difficult to anatomically diagnose primary duodenal adenocarcinoma of the fourth portion. However, primary duodenal adenocarcinoma of the fourth portion can be diagnosed by recent developments in enteroscopy. Here we report a case of duodenal adenocarcinoma of the fourth portion diagnosed by double-balloon enteroscopy and resected surgically, and discuss it based on a review of literature.

A 57-year-old Japanese male was admitted to our hospital in December 2009 for evaluation of severe anemia. Past medical history included a gastric ulcer when he was 50-years-old. His family history was noncontributory. Physical examination on admission revealed a body height of 175 cm, weight of 63 kg, blood pressure of 112/61 mmHg, a regular pulse of 85/min, and body temperature of 36.8oC. There was no sign of lymphadenopathy. His abdomen was soft and flat, and no abdominal, liver, or spleen masses were palpable.

There were no abnormal laboratory findings, except evidence of anemia (hemoglobin was 6.6 g/dL) and an inflammatory reaction (white blood cell count was 8900/µg, and C-reactive protein was 3.7 mg/dL). Tumor markers, carcinoembryonic antigen (CEA) and carbohydrate antigen (CA19-9), were within normal ranges.

Abdominal computed tomography (CT) scan indicated a circumferential, thick, and unequally enhanced wall in the fourth portion of the duodenum, and showed no lymph node swelling or ascites. Upper gastrointestinal series demonstrated a 7-cm 'apple core' sign in the fourth portion of the duodenum (Figure 1). Double-balloon enteroscopy showed a circumferential tumor with ulceration in the fourth portion of the duodenum (Figure 2). The enteroscope could not pass the lesion. Biopsy specimen from the lesion disclosed poorly differentiated adenocarcinoma. Positron Emission Tomography (PET) revealed accumulation in the fourth portion of the duodenum (Figure 3). Based on these findings, the patient was diagnosed with primary duodenal adenocarcinoma of the fourth portion and underwent partial duodenectomy and partial colectomy because the marginal artery of the transverse colon was invaded by the tumor on January 22nd, 2010.

The resected tumor had deep ulceration with a round wall and measured 100×80 mm in size (Figure 4).

Histological examination disclosed that the tumor was poorly differentiated adenocarcinoma which invaded into the subserosal layer and two lymph node metastases were seen (Figure 5).

The patient had an uneventful postoperative course, and was discharged from hospital 40 days postoperatively. Combined chemotherapy of TS-1 and cisplatin was prescribed as neoadjuvant chemotherapy. TS-1 (80 mg/m²) was administered 21 days, followed by 14 days rest as one course. Cisplatin (60 mg/m²) was administered on the eight day. Six courses of this adjuvant chemotherapy were administered. The patient is alive with no recurrence for one year.

Primary duodenal adenocarcinoma is extremely rare. Most primary duodenal adenocarcinomas are in the first and second portions, with 20% in the third portion, and 10% in the fourth portion. [2] Recently, reports of primary duodenal adenocarcinoma have been increasing because of progresses in gastric enteroscopy. [3] [4] However, it is impossible to diagnose primary duodenal adenocarcinoma of the third and fourth portions by gastric enteroscopy. There are still many cases to be diagnosed by the upper gastrointestinal series. In this case, primary duodenal adenocarcinoma of the fourth portion was diagnosed by double-balloon enteroscopy. In Japan, there are no case reports of primary duodenal adenocarcinoma of the fourth portion diagnosed by double-balloon enteroscopy. In Brazil, there is only one case report. [5]

In Japan, there are eleven case reports of primary duodenal adenocarcinoma of the fourth portion resected surgically, including our case [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] [16] (Table 1). The median age was 60.5 years (range, 42–74 years). Six cases were men. Two cases invaded into the mucosal layer, three cases into the subserosal layer, and six cases went beyond the serosal layer. Seven cases had metastasis of the lymph nodes. According to these reports, there are two kinds of surgery, three cases of pylorus preserving pancreatoduodenectomy and partial duodenectomy and eight cases of partial duodenectomy with lymphadenectomy. In Kaklamanos's report, there was no significant difference in prognosis between pancreatoduodenectomy and partial duodenectomy in sixty-three cases of primary duodenal adenocarcinoma. [16] In Lowell's report, the seven cases of primary duodenal adenocarcinoma of the third or fourth portions are all alive after five years, except for those who died of another disease. [17] Five of seven cases underwent partial duodenectomy. There are many reports that partial duodenectomy is better in the case of primary duodenal adenocarcinoma in the third or fourth portions. Consequently, partial duodenectomy was thought to be a better surgery for primary distal duodenal adenocarcinoma. However, in Suzuki's report, a case of primary duodenal adenocarcinoma of the fourth portion underwent pancreatoduodenectomy. [12] There were metastases of the lymph nodes in No. 13 and No. 14 according to the Japanese classification of gastric carcinoma (The 14th Edition). That report insisted on the necessity of pancreatoduodenectomy with lymphadenectomy. Our case underwent partial duodenectomy with lymphadenectomy. There was no recurrence for one year in spite of advanced carcinoma and metastasis of the lymph nodes.

We reported an extremely rare case of primary duodenal adenocarcinoma of the fourth portion diagnosed by double-balloon enteroscopy and resected surgically.

1. Tocci A, Mazzoni G, Puma F, et al. Adenocarcinoma of the third and fourth portions of the duodenum: results of surgical treatment. Arch Surg 2003;138:80–5.   [CrossRef]   [Pubmed]    
2. Shunsuke O, Gakuto K, Yoshihisa S, et al. A Clinicopathological Study of Our 10 Cases with Primary Carcinoma of the Duodenum. The Japanese Journal of Gastroenterological Surgery 2006;39:1458–63.    
3. Rose DM, Hochwald SN, Klimstra DS, Brennan MF. Primary duodenal adenocarcinoma: a ten-year experience with 79 patients. J Am Coll Surg 1996;183:89–6.   [Pubmed]    
4. Santoro E, Sacchi M, Scutari F, Carboni F, Graziano F. Primary adenocarcinoma of the duodenum: treatment and survival in 89 patients. Hepatogastroenterology 1997;44:1157–63.   [Pubmed]    
5. Safatle-Ribeiro AV, Franzini TA, Kuqa R, et al. Double-balloon enteroscopy in the diagnosis of an adenocarcinoma of the fourth portion of the duodenum: report of a case. Clinics (Sao Paulo) 2007 Jun;62(3):353–6.   [CrossRef]   [Pubmed]    
6. Keiichi A, Shiro F, Noriyuki A. A case of an advanced duodenal cancer of the fourth portion. Clinics in Bowel Diseases 1991;4:24–7.    
7. Yasuhiko U, Tadatoshi O, Yoshihiko S, Toshio N, Naomi W, Ryutaro M. A case of an advanced duodenal cancer of the fourth portion. Surgical Diagnosis & Treatment 1996;38:1337–40.    
8. Yoshio O, Kazuo S, Fuminori A, et al. A case of duodenal adenocarcinoma of the fourth portion diagnosed. The Journal of the Hiroshima Medical Association 2000;53:414–7.    
9. Shuji S, Itaru E, Takafumi K, et al. A case of curatively resected duodenal cancer of the fourth portion. The Japanese Journal of Gastroenterological Surgery 2001;34:485–9.    
10. Yukako H, Shigehiro K, Kimiyasu Y, et al. A case of duodenal polypoid intramucosal adenocarcinoma observed for 6 years. Gastroenterological endoscopy 2003;45:1130–4.    
11. Tetsuya M, Akira T, Hironori K. A Case Report of Primary Duodenal Carcinoma of the Fourth Portion. Journal of Japanese College of Surgeons 2004;29:884–9.    
12. Ryo S, Norichika M, Kentaro N, Akimasa Y, Takahisa M, Tomoaki M. A Case of Primary Adenocarcinoma of the Fourth Portion of the Duodenum Curatively Resected by Pylorus-Preserving Pancreatoduodenectomy. The Japanese Journal of Gastroenterological Surgery 2005;38:429–34.    
13. Hiroyuki K, Katsutoshi T, Takeshi T, et al. A Resected Case of Primary Adenocarcinoma of the Fourth Portion of the Duodenum. The Japanese Journal of Gastroenterological Surgery 2006;39:1380–4.    
14. Kiichiro M, Koichi T, Yu N, Daisuke K, Mitsumasa A, Shinji T. A case of duodenal cancer of the fourth portion detected by routine gastroduodenoscopy. Gastroenterological endoscopy 2006;48:2639–44.    
15. Tetsuo I, Makoto T, Takashi O, et al. A case of early cancer of the fourth portion of the duodenum. The Japanese Journal of Gastroenterological Surgery 2008;41:516–20.    
16. Kaklamanos IG, Bathe OF, Franceschi D, Camarda C, Levi J, Livingstone AS. Extent of resection in the management of duodenal adenocarcinoma. Am J Surg 2000;179:37–41.   [CrossRef]   [Pubmed]    
17. Lowell JA, Rossi RL, Munson JL, Braasch JW. Primary adenocarcinoma of third and fourth portions of duodenum, favorable prognosis after resection. Arch Surg 1992;127:557–60.   [CrossRef]   [Pubmed]