Lymphangitic spread from the appendiceal adenocarcinoma to the ileocecal valve, mimicking Crohn’s disease

Abstract

Due to the anatomical peculiarity of the appendix, diagnosis of tumors arising from this area can be challenging by clinicoradiologic means. We report a case of a rare primary appendiceal signet ring carcinoma with an uncommon presentation. An 86-year-old woman was admitted to our hospital with subacute epigastric pain. Computed tomography demonstrated bowel wall thickening with fat stranding in the ileocecal region. The leading diagnostic consideration was inflammatory bowel disease. Upon colonoscopy, a swollen, distorted ileocecal valve was identified. The remaining colon was otherwise unremarkable. Extensive biopsy sampling of the ileocecal region and colon was performed. A lymphangitic signet ring carcinoma within the ileocecal region was diagnosed on biopsy; there was no dysplasia or carcinoma of the remaining biopsies. By cytomorphology and immunoprofile, a lymphangitic signet ring carcinoma of appendiceal origin was the primary consideration, further confirmed upon subsequent laparotomy. This case represents an unusual pattern of appendiceal tumor spread with localized, lymphangitic involvement, creating a milieu which closely simulates Crohn's disease on imaging modalities.

Progressive Primary Appendiceal Crohn’s Disease in a 21-Year-old Female

“…Given the rarity of the diagnosis, it is very uncommon to diagnose appendiceal Crohn’s disease preoperatively. Medical management of isolated appendiceal Crohn’s disease is challenging because the lumen of the appendix is not accessible for a confirmatory tissue diagnosis [5] or for topical therapy, and systemic therapy is contraindicated for such isolated disease. In the case of our patient, our suspicion for appendiceal involvement of Crohn’s disease was raised by the atypical course of appendicitis in the patient (persistent radiographic findings of appendicitis over the course of a 5-month period with intermittent bouts of severe pain) along with mild mucosal findings of Crohn’s disease in the surrounding ileal/colonic mucosa.…”

Signet Ring Carcinoma of the Appendix- A Rare Case Report

“…13,14 Essentially, lymphatic-space metastases can closely mimic inflammatory conditions. 15,16 The majority of the appendix carcinomas are identified accidentally, and many are advanced at the time of diagnosis. 17 The extent of the tumor at the time of the diagnosis is an important determinant of the patient's future prognosis.…”

Signet Ring Carcinoma of the Appendix Presenting as Crohn’s Disease in a Young Male

“…Signet ring carcinomas are considerably more likely to have extended at the time of diagnosis, as compared to other primary appendiceal tumors [ 2 , 9 ]. Essentially, lymphatic-space metastases can closely mimic inflammatory conditions [ 14 , 19 ]. The components of the ileocecal region (terminal ileum, appendix, cecum, ileocecal valve) are anatomically next to one another; so, the pathology may include more than a single anatomic area, causing difficulties in its diagnosis.…”

“…Our patient was only 22 years old at the time of the diagnosis of his tumor. A case of appendiceal adenocarcinoma spreading to the ileocecal valve presenting as Crohn's disease in an 86-year-old man has been reported [ 19 ]. In our case, the tumor was showing lesions that could be typical for Chron's disease.…”