Abstract
BackgroundAlthough primary duodenal adenocarcinoma (DA) is a rare malignancy representing ~ 0.5% of all gastrointestinal cancers, the incidence of DA is more frequent in Lynch syndrome. Because of its rarity, treatment strategies or optimal chemotherapeutic regimens have not been clearly defined for advanced DA.Case presentationA 72-year-old woman with Lynch syndrome visited our hospital with a right upper abdominal pain. Computed tomography (CT) showed wall thickness with enhancement in the second portion of the duodenum and adjacent abdominal wall, which suggested direct tumor invasion to the abdominal wall. Upper gastrointestinal endoscopy (UGE) showed a large ulcerative tumor in the second portion of the duodenum, and histological analysis revealed a poorly differentiated adenocarcinoma. A cT4N0M0, cStage IIB (Union for International Control Cancer TNM staging) DA was diagnosed. After three courses of chemotherapy with S-1 and oxaliplatin (SOX), follow-up CT and UGE showed shrinkage of the duodenal tumor. Therefore, the patient underwent pancreaticoduodenectomy with lymph node dissection with curative intent. Histological examination showed a pathological complete response to SOX therapy. The postoperative course was uneventful, and the patient was discharged on postoperative day 29. The patient received no adjuvant chemotherapy, and there has been no evidence of recurrence 6 months after the operation.ConclusionsSOX therapy provided a remarkable response and can be an optimal chemotherapeutic regimen for advanced DA in Lynch syndrome.
Highlights
Primary duodenal adenocarcinoma (DA) is a rare malignancy representing ~ 0.5% of all gastrointestinal cancers, the incidence of DA is more frequent in Lynch syndrome
The incidence of DA is more frequent in Lynch syndrome [4], and cancers that develop in Lynch syndrome are associated with microsatellite instability (MSI)
We describe the case of an advanced DA in Lynch syndrome in which pathological complete response was achieved with chemotherapy with S-1 and oxaliplatin (SOX)
Summary
Primary duodenal adenocarcinoma (DA) is a rare malignancy, representing ~ 0.5% of all gastrointestinal cancers, and is often diagnosed at an advanced stage [1,2,3]. Serum levels of the tumor markers carcinoembryonic antigen and carbohydrate antigen 19–9 were within normal limits She had a history of four resections of different parts of the colon because of colon cancer associated with Lynch syndrome. Pathological evaluation revealed a pT2N0M0 pStage I tumor based on the seventh edition of the Union for International Cancer Control TNM staging. Her family history fulfilled the Amsterdam II and revised Bethesda criteria. Operation The patient underwent pancreaticoduodenectomy with combined resection of the adjacent abdominal wall and regional lymph node dissection with curative intent 3 weeks after the last administration of chemotherapy. Postoperative surveillance is being planned according to the Japanese Society for Cancer of the Colon and Rectum Guidelines 2016 for the Clinical Practice of Hereditary Colorectal Cancer [7]
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