A Rare Case of Primary Duodenal Signet Ring Cell Carcinoma

Introduction: Signet ring cell carcinoma (SRCC) is a poorly differentiated adenocarcinoma named after a histological resemblance to a signet ring. Ninety-six percent of all SRCC are located within the stomach, but may also be found in the breast, gallbladder, pancreas, urinary bladder, and large bowel. Duodenal SRCC is an extremely rare finding. A review of the literature reveals a number of cases of SRCC involving the ampulla of Vater, but only 2 reported cases of true duodenal SRCC. In this case report, we present a true duodenal SRCC in an 82-year-old female. Case Report: An 82-year-old female presented with melena off and on over a 5-month period. Past medical history included hypertension, hypothyroidism, chronic kidney disease, and previous hysterectomy. Medications included hydrochlorothiazide, synthroid, citalopram, metoprolol, candesartan, and ASA. Blood work demonstrated iron deficiency anemia with hemoglobin 86g/L, serum iron 11.3. The patient developed 2 episodes of pre-syncope after passing melena and was assessed in the ED. Her hemoglobin had dropped to 68. The patient was transfused 2 units of packed red blood cells and was sent home. An urgent outpatient gastroscopy was performed. It showed a deep, non-bleeding, clean-based ulcer (10 mm x 12 mm) in the duodenal bulb. Biopsies were taken from the ulcer edge. Pathology confirmed adenocarcinoma with signet ring pattern with vascular space invasion. Subsequent CT confirmed thickening of the duodenal wall with nodularity in the peritoneum suggestive of tumour deposit. The patient was assessed by general surgery, but given her age, comorbidities and possible peritoneal involvement surgery was not performed. She continued to pass melena and was assessed for palliative radiation therapy. The patient completed radiotherapy (3000 cGy in 10 fractions) and has remained relatively stable other than some intermittent rectal bleeding. Discussion: Small intestine tumours make up only 1-5 % of all bowel neoplasms, and of these, approximately 50% occur in the duodenum. Within the gastrointestinal tract, signet ring cell carcinoma (SRCC) are almost always located in the stomach and rarely seen in the small intestine. This illustrates that primary duodenal SRCC is exceedingly rare. Duodenal SRCC generally presents with advancedstage disease, and thus carries a poor prognosis. If caught in early stage disease, preferred treatment for those who are surgical candidates is a pancreaticoduodenectomy with dissection of regional lymph nodes. In the rare case of a suspected duodenal SRCC, the lesion may be located throughout the duodenum and should be considered even in the absence of symptoms of gastric outlet or hepatobiliary obstruction.

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