CASE REPORT
A 54-year-old woman presented in October 2006 with vomiting and constipation for 4 days. She described a sensation of epigastric fullness but denied any abdominal pain or gastrointestinal bleeding. A computed tomography (CT) scan demonstrated a proximal jejunal mass causing intussusception and partial small bowel obstruction as well as a solitary hepatic lesion and retroperitoneal lymphadenopathy.
The patient underwent an upper endoscopy, but the results were unremarkable. The patient then underwent an exploratory laparotomy with a resection of the fourth portion of the duodenum and proximal jejunum, dudeonojejunostomy, and liver biopsy. The jejunum demonstrated a moderately differentiated adenocarcinoma, infiltrating through the bowel wall and present on the serosal surface, with one of three lymph nodes positive. The biopsy of the liver lesion was consistent with metastatic adenocarcinoma (stage T4 N1 M1). At the time of diagnosis all of patient's serum tumor markers were within normal limits, including a CA19-9 of 9.7.
The patient was started on modified FOLFOX 6 (folinic acid, 5-fluorouracil, and oxaliplatin in December 2006. The patient completed 10 cycles of chemotherapy by the end of May 2007. A CT scan of chest, abdomen, and pelvis in June 2007 showed no evidence of lung metastasis or adenopathy and a decreased liver metastasis.A follow-up CT scan in September 2007 showed stable appearance of the chest, abdomen, and pelvis. Follow-up CT scans were done every 3–4 months for a year and then every 6 months. The most recent CT scan in September 2010 was free of any evidence of metastatic disease, adenopathy, or suspicious lesions. The patient is clinically stable, without evidence of recurrence for almost 4 years. The only residual side effect from the chemotherapy is a persistent grade 1 mild sensory peripheral neuropathy.
DISCUSSION
Malignancies originating in the small intestine are uncommon, accounting for only 2% of all gastrointestinal tract neoplasms and less than 0.4% of all cancers in the United States.1,2 Approximately 6,230 new cases and 1,110 patient deaths are seen annually in the United States.1 Carcinoid tumors are the most common small bowel tumors reported to the National Cancer Data Base in the year 2000. However, adenocarcinoma is the most common malignancy affecting the duodenum and jejunum (64% and 46%, respectively), and carcinoids are the most common tumors in the ileum (63%).3 Risk factors for small bowel adenocarcinoma (SBA) include familial adenomatous polyposis, hereditary nonpolyposis colorectal cancer syndrome, Crohn's disease, celiac sprue, type-1 neurofibromatosis, and Peutz-Jeghers syndrome. The majority of patients present with advanced disease, which may be due to a lack of specificity of the presenting symptoms. The prognosis for advanced SBA is poor, with the reported overall survival (OS) rate at 2 years of 5–10%.4 Limited data exist to guide the choice of chemotherapeutic regimen in cases of advanced SBA.
Here we report a case of a 58-year-old patient with metastatic adenocarcinoma of the jejunum who had a complete response to chemotherapy with folinic acid, 5-FU (5-fluorouracil), and oxaliplatin (FOLFOX). She is alive without disease progression 4 years after her initial presentation.
The prognosis for metastatic SBA is poor, with a median OS at 2 years of between 5% and 10%.4 There is no standard first-line chemotherapy for patients with advanced SBA. The choice of systemic chemotherapy for patients with advanced SBA has usually been based on the regimens used for metastatic colorectal cancer. Numerous retrospective studies have suggested that chemotherapy improves the survival of patients with advanced SBA.5,6,7
Jigyasu et al8 reported a series of 14 patients with metastatic SBA treated with chemotherapy at MD Anderson Cancer Center over a 30-year period (1950–1980), which showed that fluoropyrimidines, when used alone or in combination with other chemotherapeutic agents, resulted in tumor regression in three patients (one partial response lasting 12 weeks and two minor responses), while nine other patients had stable disease. Median OS was 9 months.
Crawley et al9 investigated the efficacy of 5-FU-based chemotherapy in eight patients with advanced SBA seen at the Royal Marsden Hospital over a 5-year period. Three patients had a response with a median OS of 13 months and progression-free survival (PFS) of 7.8 months.
Recent clinical trials have shown favorable response rates to newer chemotherapeutic regimens. Twenty patients with advanced SBA were retrospectively analyzed over a 9-year period at the Institut Gustave Roussy.10 Patients received chemotherapy with 5-FU and either cisplatin or oxaliplatin and were found to have an overall response rate of 21%, a median PFS of 8 months, and a median OS of 14 months. Moreover, one patient had a biologicalcomplete response, which made it possible for a surgical peritoneal cytoreduction and hyperthermic intraperitoneal chemotherapy. These results suggested that platinum-based regimens could be effective in advanced SBA.
A recent prospective, phase II study done at MD Anderson Cancer Center in 2009 showed that CAPOX (capecitabine 750 mg/m2 twice daily on days 1 through 14, and oxaliplatin 130 mg/m2 on day 1, every 21 days) was highly active in patients with advanced SBA and ampullary adenocarcinoma (AAC) with an overall response rate of 50% and a median OS of 20.4 months in 30 patients.11 The response rate in the subgroup of 18 patients with SBA was 61% (95% CI, 36% to 83%), and one patient with metastatic SBA had a complete response to CAPOX.11
A recent retrospective study of 93 patients with advanced SBA compared a number of chemotherapy regimens given as front-line therapy; 48 received FOLFOX.12 Overall, FOLFOX was the most effective platinum-based regimen for advanced SBA with median OS at 17.8 months and PFS of 6.9 months.12
We present a case of a patient with metastatic adenocarcinoma of the jejunum who was treated with FOLFOX and had a long-term complete response. FOLFOX has demonstrated significant activity in other studies and could represent a new standard for the treatment of advanced SBA.
Footnotes
Disclosures of Potential Conflicts of Interest
The authors indicated no potential conflicts of interest.
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