duodenal cancer was previously viewed as a rare type of cancer . because of the rarity of this cancer , there are few reports on chemotherapy for duodenal cancer . the 5-year survival rate of duodenal cancer is reported to be less than 30% [ 1 , 2 ] . on the other hand when the tumor is resectable , pancreatoduodenostomy is the most common operative procedure , as the majority of tumors originate in the periampullary region . although cases of duodenal cancer treated with relatively noninvasive methods such as endoscopic treatment have been reported , the usefulness of chemotherapy has been reported to be low in cases of inoperable duodenal cancer , and no standard method of treatment has been established for this type of duodenal cancer . however , the establishment of a standard treatment is important because reports on this cancer have recently been increasing following improvement in the diagnosis using procedures such as computed tomography ( ct ) , magnetic resonance imaging and endoscopy including endoscopic ultrasonography , capsule endoscopy and double balloon endoscopy . s-1 is an oral anti - cancer agent , composed of tegafur , 5-chloro-2,4-dihydroxypyridine , and potassium oxonate , at a molar ratio of 1:0.4:1 . it is based on the biochemical modulation of 5-fluorouracil and reported to be effective in patients with advanced gastrocolorectal cancer [ 4 , 5 , 6 ] . we recently encountered a case of inoperable duodenal cancer where long - term survival for 6 years and 1 month was achieved by multidisciplinary treatment such as the chemotherapy mainly using s-1 . a 50-year - old female became aware of skin yellowing and consulted another hospital where she was diagnosed intraoperatively with duodenal cancer in december 2001 because of lymph node metastases around the aorta . a catheter for endoscopic nasal biliary drainage was left inserted , and the patient was referred to our hospital . two lymph nodes , each about 1 cm in diameter , were palpable in the left supraclavicular cavity , but no other noteworthy abnormality was found . in the laboratory data on admission , mild liver dysfunction with jaundice endoscopy revealed early - stage cancer ( iia + iic ) distal to the duodenal papilla ( fig . 1a ) , and biopsy allowed a diagnosis of well - differentiated adenocarcinoma ( fig . ct revealed a large number of lymph node metastases around the aorta and in the left supraclavicular cavity ( fig . this case was rated as inoperable also at our hospital , and chemotherapy was administered . in january 2002 , the patient began to receive s-1/cisplatin ( cddp ) therapy resembling a regimen used for gastric cancer . after the start of treatment , the primary lesion disappeared and lymph node metastases also markedly decreased in size ( fig . later numbness , a known adverse reaction to cddp , developed . in january 2003 , the therapy was switched to s-1/irinotecan ( cpt-11 ) therapy . in december 2003 , the patient was rated as showing complete response on the basis of findings from 2-deoxy-18f - fluoro - glucose positron emission tomography ( fdg - pet ) and ct ( fig . the treatment was then switched to uncombined s-1 therapy . thereafter no recurrence was noted and the regimen was switched to consecutive - day uft therapy in february 2005 . in september 2005 , swelling of lymph nodes in the left supraclavicular cavity was noted , and a judgment of tumor recurrence in the lymph nodes was made . the regimen was therefore switched to s-1/cpt-11 . however , the response rate was progressive disease by ct , and cea rose to 24.7 ng / ml . this therapy was therefore discontinued after the second cycle . in december 2005 , weekly paclitaxel ( ptx ) therapy was started , but lymph node swelling of the left supraclavicular cavity intensified and the therapy was switched in february 2006 to weekly docetaxel therapy with radiotherapy . the response rated by ct after this therapy was complete response . in july 2006 , cea rose again and the regimen was switched to uncombined s-1 therapy . in the same month , multiple brain metastases were detected , and gamma knife therapy of the same area was additionally applied . later , tumor recurrence and onset of new lesion were noted , and gamma knife therapy was additionally performed in february and october 2007 . in january 2007 , , regional lymph node excision was performed at the department of surgery , followed by radiotherapy ( 50 gy in total ) of the same area . in july 2007 , fdg - pet revealed multiple subcutaneous metastases , and weekly ptx therapy was resumed on the basis of the results about the sensitivity of axillary lymph nodes to anti - cancer agents . the skin metastases diminished in response to this therapy . in october 2007 , however , ct revealed multiple subcutaneous metastases and small metastases within the lung . the patient 's treatment regimen from january 2002 to october 2007 is given in table 1 . duodenal cancer is a rare cancer which accounts for only 0.3% of all gastrointestinal cancers . however , among cancers affecting the small bowel , duodenal cancer is reported to constitute about 50% of all cases . reports on this cancer have recently been increasing following improvement in the diagnostic capability thanks to development of small bowel capsule endoscopes and double balloon endoscopes . for advanced duodenal cancer , pancreatoduodenectomy is a standard operative procedure , but this procedure is applicable to about 40% of all cases , with the postoperative 5-year survival rate being about 50% . the 2-year survival rate for cases of inoperable duodenal cancer is reported to be < 20% , with the 5-year survival rate being 0% and median survival time being 7 months [ 9 , 10 ] . chemotherapy has been viewed as little useful as a means of treating inoperative duodenal cancer   and no standard therapy for inoperative duodenal cancer has been established . however , patients with inoperative duodenal cancer having survived long after active chemotherapy have recently been reported sporadically , and it is now desirable to establish standard chemotherapy for such cases . reported that a patient with adenocarcinoma of duodenal bulb origin , accompanied by multiple liver metastases , survived for 3 years and 4 months after combined uft / mitomycin therapy . witham and harnett also reported a patient with duodenal cancer with multiple liver metastases who survived for 3 years after chemotherapy with 5-fu . s-1 is a new oral anti - cancer drug comprised of tegafur , 5-chloro-2,4-dihydroxypyridine , and potassium oxonate . this drug was designed to enhance the efficacy of tegafur , a prodrug of fluorouracil , and has been reported to exert high clinical efficacy when used independently or in combination with other drugs for the treatment of gastrointestinal cancers and various other cancers . reported that treatment with s-1 resulted in survival for 1 year and 7 months in a patient with duodenal adenocarcinoma with liver metastasis . in the present case , the primary lesion assumed a form resembling early - stage cancer , but many lymph node metastases were noted , allowing a judgment of inoperable advanced duodenal cancer . chemotherapy primarily with s-1 resulted in complete response for a while when rated by diagnostic imaging . later , recurrence and distant metastasis took place , but multidisciplinary treatment resulted in long survival for 6 years and 1 month . this is a valuable case suggesting the usefulness of this therapeutic approach . in view of the fact that duodenal cancer is a relatively rare disease and the possibility that the incidence of this disease may increase in the future , it seems essential to collect additional data from multicenter prospective studies towards the goal of establishing a standard method of treatment for this disease .
a 50-year - old female became aware of skin yellowing and consulted another hospital where she was diagnosed intraoperatively with duodenal cancer because of lymph node metastases around the aorta . 
 endoscopy revealed type iia + iic cancer distal to the duodenal papilla , and biopsy allowed a diagnosis of well - differentiated adenocarcinoma . computed tomography revealed a large number of lymph node metastases around the aorta and in the left supraclavicular cavity . 
 the patient was given many regimens of chemotherapy , mainly containing s-1 , and multidisciplinary treatment , and achieved long - term survival for 6 years and 1 month . 
 this is a valuable case suggesting the usefulness of this therapeutic approach . in view of the fact that duodenal cancer is a relatively rare disease and the possibility that the incidence of this disease may increase in the future 
 , it seems essential to collect additional data from multicenter prospective studies towards the goal of establishing a standard method of treatment for this disease .