Ileal Adenocarcinoma with Liver Metastasis in Patient with Crohnʼs Disease: A Case with Prolonged Survival

Abstract

: Small bowel adenocarcinoma is a rare but well-known complication of Crohn's disease. The diagnosis is often delayed and typically detected only at surgery in an advanced stage with a poor prognosis. We report a case of metastatic ileal adenocarcinoma in a patient with Crohn's disease with prolonged survival. Case report:A 31-year-old Persian male with a 6-year history of ileal Crohn's disease (CD) presented with abdominal cramping, distention, and hematochezia. Colonoscopy showed an ileal tumor involving the ileocecal valve and cecum. Biopsy revealed a moderately differentiated adenocarcinoma. He underwent resection of the terminal ileum and descending colon; histopathology showed poorly differentiated mucinous adenocarcinoma and underlying CD (Figure 1). It was classified as T4N1M1.2066_A Figure 1. Histologic section from tumor mass show normal small intestinal mucosa (blue arrowheads) and adjacent invasive adenocarcinoma (black arrows).He underwent a small bowel resection in 2008 and left hepatic lobe resection in 2009 followed by chemotherapy with FOLFOX. The patient was stable until April 2012 when he was considered to be refractory due to disease progression to peritoneal LNs, liver, and lung, and was unfit for standard treatments. Treatment began with half the standard dosage of all drugs (FOLFOX) with added gemcitabine, irinotecan (GFLIO) and nutritional support. During this time, the patient continued with fistulizing CD with infection, obstruction, and dehydration requiring hospitalization and mesenchymal stromal cells (MSC) infusion in December 2013 for the fistulizing CD. CT scan in September 2015 revealed no evidence of recurrence. PET scan done in November 2015 showed the progression of the tumor. Further treatment began with Cetuximab along with GFLIO produced a period of stable disease. In May 2016, surveillance imaging showed metastasis to lung and thoracic spine. Trastuzumab was added as the tumor was human epidermal growth factor receptor 2-positive (HER2+). This produced regression of pulmonary metastatic disease. The bony disease became stable and was technically unevaluable. In March 2018 patient was hospitalized due to recurrence of tumor at enterocutaneous fistula site with GI bleeding from peristomal varices due to portal hypertension. His condition was deemed non-surgical. A detailed treatment course with outcome is summarized in table 1. Conclusion: Our case of prolonged survival suggest a possible role for checkpoint immunotherapy. Also when there is a rare neoplastic disease with long survivorship the chemical profile of these survivors warrants identification.2066_B Figure 2. Treatment Summary and Clinical Outcome.