The patient was a 68-year-old woman with a prior hysterectomy and family history of CD who presented as a transfer from an outside hospital for the evaluation and treatment of recurrent severe lower abdominal pain.
The patient had initially presented to the outside hospital four weeks prior to this transfer with a chief complaint of constant lower abdominal pain without diarrhea, but with intermittent constipation. The patient also reported a four-pound weight loss. The patient's social history was negative for smoking, alcohol, or illicit drug use. She reported that her mother had been diagnosed with CD. An initial computed tomography (CT) scan revealed abnormal wall thickening and fat stranding within the terminal ileum consistent with an infectious or inflammatory process with no visible abscesses. The mesenteric lymph nodes in the right lower quadrant were also mildly prominent consistent with mesenteric adenitis, and neoplastic disease could not be ruled out. A follow-up colonoscopy revealed a redundant colon with normal mucosa except for superficial, linear erosions in the distal rectum, sigmoid diverticulosis, and an 8 mm polyp in the ascending colon. The terminal ileum appeared to be diffusely congested without any ulcerations or obvious inflammation.
No evidence of pathology was noted on biopsy specimens obtained from the ileocecal valve. Laboratory studies from the outside hospital included a complete blood count (CBC) that revealed slight anemia (hemoglobin at 12 g/L and hematocrit at 37.5%) with normal white blood cell (WBC; 10.2 cells/mcL) and platelet counts (338 cells/mcL). Her routine serum chemistry panel was also within normal limits. The patient had a negative T-spot for tuberculosis and a negative hepatitis panel; elevated levels of C-reactive protein (CRP; 45 mg/L) and calprotectin (408 mug/mL) levels were noted. Based on the family history, colonoscopy findings, and elevated levels of CRP and calprotectin, the patient was initially diagnosed with CD and began treatment with 60 mg methylprednisolone. Her symptoms initially improved on this regimen. She transitioned to oral prednisone and was discharged four days later.
Ten days after discharge, the patient returned to the same outside hospital with a recurrence of persistent abdominal pain. Results from a second CT scan were similar to those reported previously, including the possibility of an infectious process. The patient was hospitalized for an additional four days and was discharged with a treatment plan that included ciprofloxacin and metronidazole together with prednisone as previously prescribed. She was scheduled to complete a prednisone taper and begin treatment with 5 mg/kg infliximab on an outpatient basis.
Two days after the second hospital discharge, the patient returned with worsening abdominal pain. A third abdominal CT performed at this time revealed terminal ileal wall thickening with surrounding fat stranding. Free fluid was detected, and ileus was also documented. The CT report noted that a portion of the inflamed segment may have developed an abscess or phlegmon.
Given the ongoing complexity of this case, the patient was transferred to our tertiary care center. Upon arrival, she was hemodynamically stable (temperature, 36.7 C; pulse, 65 beats/min; blood pressure, 166/66 mmHg; SpO2, 99% on room air; and respiratory rate, 21 breaths/min). Her CBC documented slight anemia with a normal WBC count and normal serum chemistries. Liver function tests, including serum lipase, were all unremarkable. The patient continued to experience abdominal pain without nausea or diarrhea. She denied any overt gastrointestinal bleeding, fever, or chills. Her initial diagnosis was an exacerbation of CD with ileus and a potential abscess/phlegmon. The gastroenterology team performed a repeat colonoscopy, and a biopsy of the terminal ileum was obtained; the specimen revealed mild inflammatory changes and congestion. Pathological analysis of this specimen revealed a malignant process. Microscopic evaluation revealed tubulovillous adenoma with high-grade dysplasia in Figure 1. This was then confirmed with immunohistochemistry. The cells were negative for cytokeratin 7 but positive for cytokeratin 20 and CDX 2. Although signet-ring cell carcinoma of the small bowel can occur, its relative rarity combined with lack of in situ tumor favored metastatic disease. However, biopsy specimens collected from a subsequent esophagogastroduodenoscopy were grossly negative for malignancy.
The patient underwent an exploratory laparotomy which resulted in the resection of the ileocolic mass; a right-sided colectomy, ileostomy, and abdominal washout were performed, and a mucous fistula was created. The mesentery and the mass adhered to the intestinal wall. An abscess with pus drainage and significant induration were also noted, which suggested an inflammatory process and/or lymphadenopathy. The tumor was 25 cm in length and poorly differentiated and was invading the visceral peritoneum and appendix. With this final resection, the pathology team confirmed the final diagnosis but was also able to find in situ components. The tumor was noted to be arising in a background of tubulovillous adenoma of the terminal ileum, with high-grade dysplasia. Based on the characteristics of this malignancy, the patient was deemed to be a good candidate for adjuvant chemotherapy. A FOLFOX (folinic acid, fluorouracil, and oxaliplatin) regimen was initiated. She is currently on her third cycle of chemotherapy and doing well. This treatment regimen aims to eliminate the disease, reduce the risk of its recurrence, and improve overall survival.