EP417 - An Obstructing Signet Ring Cell Carcinoma in an 18 year old with a History of Neuroectodermal Tumor: A Case Report

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Purpose/Background: Colorectal cancer remains one of the most common cancers in the United States, with rates of cancer rising in younger patients.1 Signet ring cell carcinoma (SRCC) is an aggressive variant that comprises less than 1% of all cases of colorectal cancer. Despite the rare nature of SRCC, it presents at a higher rate in adolescents and young adults.1,2,3 More than 80% of patients diagnosed with SRCC were found to have advanced disease at presentation.2,3 Our case uniquely presents an 18-year-old female patient diagnosed with stage IIIC (T4b N2b) signet ring cell carcinoma.

Methods/Interventions:: 18yo F with a history of primitive neuroectodermal tumor (PNET), for which she was on chemotherapy for years, and a history of neurogenic bladder and bowel secondary to PNET who presented to the ED with two weeks of constipation. She had intermittent issues with constipation as a young child, but those issues had resolved several years prior. She had no family history of colon cancer. She had continued to pass gas but had developed nausea and vomiting. Her initial CT scan showed a distended fluid filled proximal colon with a transition at the distal transverse colon compatible with a possible distal transverse large bowel obstruction. She underwent a Gastrograffin Enema with an inability to get contrast past a very large left colon stool burden. She was administered multiple enemas to clear the fecal burden. A repeat Gastrograffin Enema (Figure 1) was performed which demonstrated an inability to get contrast past the distal transverse colon.

Results/Outcomes: She was taken for a colonoscopy to delineate if this area of obstruction was a mass or fecalith . The colonoscopy did demonstrate a large occlusive mass in the distal transverse colon that could not be traversed (Figure 2). As a result, the patient was taken urgently to the operating room for a transverse colectomy with creation of end colostomy. At the time of surgery, she was noted to have several enlarged lymph nodes as well as an area of dense omental adhesions overlying the mass. She did well postoperatively and was discharged home. Her final pathology showed a T4b N2b signet ring cell carcinoma. The primary tumor measured 4.3cm with invasion through the colon wall into adjacent omentum. She had 17 positive lymph nodes of the 48 that were sampled. Both angiolymphatic and perineural invasion were present.

Conclusion/Discussion: Signet ring carcinoma is an aggressive variant of colon cancer that often presents at a late stage.1,2,3,4 More than 96% of cases occur in the stomach with the remaining 4% being found in the colon or small bowel.4 A prior cohort study identified that 80% of patients with SRCC were diagnosed at stage III or IV, compared to only 50% for colon adenocarcinoma.3,4 This case represents a unique presentation of SRCC in a young female.