Contributed by Hina Sheikh,MD, Uma Krishnamurti, MD, PhD and Swaminathan Rajendiran,MD
A woman in her 40s presented to the GI clinic with a seven-month history of intermittent, crampy abdominal pain. The pain was associated with diarrhea alternating with constipation, vomiting and a poor appetite, leading to an overall 50-pound weight loss. Past medical history was notable for a positive PPD, hypothyroidism and tonsillectomy. She was gravida 0 para 0 and was under treatment with Isoniazid for 6 months. Date of last menstrual period was not known. Physical examination showed an enlarged thyroid and stasis changes in lower extremities. No abdominal masses were noted. Rectal examination was declined. Gynecologic examination was not performed.
CT scan at an outside hospital showed focal ileal thickening. Colonoscopy was performed which showed a soft mass-like lesion in the cecum (Fig 1). Terminal ileum was not visualized. An enteroclysis performed at the same time, demonstrated a high-grade small bowel obstruction in the terminal ileum (Fig 2). Biopsy of the mass showed colonic mucosa with prominent lymphoid aggregates. There was no evidence of colitis or malignancy.
Due to persistence of symptoms, the patient was scheduled for surgery. Initial laparoscopic evaluation in the OR identified the ascending colon/cecal mass that was suspicious for malignancy, with massive distension of the terminal ileum. There was an adhesive band between the cecum and the sigmoid colon. The local lymph nodes were enlarged. No other abnormalities were noted.
An ileo-colonic resection was performed and sent for intra-operative evaluation. It consisted of a 35-cm segment of bowel with attached fat and dense fibrous tissue. The small bowel was dilated to a circumference of 5.5-cm due to a 4x4x3-cm mass located at the ileocecal junction. The mucosa overlying the mass was tan-pale pink and grossly unremarkable. The cut surface showed poorly demarcated dense, gray-white fibrous lesion predominantly in the muscularis propria and subserosal adipose tissue, with focal areas of recent hemorrhage (Fig 3). The remainder of the specimen showed no abnormalities. The appendix was not identified.
A frozen section of the lesion demonstrated multiple dilated glands within the muscularis propria (Figs 4, 5). The glands were lined by a single layer of columnar cells with basally located nuclei without pleomorphism, and were surrounded by an edematous spindle-cell stroma (Fig 6). Some glandular lumens had hemorrhagic contents admixed with macrophages (Fig 7). The overlying small bowel mucosa showed intact crypt architecture with mild vascular congestion (Fig 8). No granulomas were seen.
Seventeen lymph nodes were identified in the specimen. Of these, two showed foci of glandular epithelium surrounded by stroma, similar to those seen in the ileocecal mass (Fig 9). Two additional nodes showed rare cystically dilated glands, lined by low-columnar cells with cytologically bland nuclei and no appreciable stroma (Fig 10). These glands were located underneath the lymph node capsule. Some of the lining cells demonstrated cilia and concentrically lamellated calcifications were identified in one of the glandular lumens (Fig 11).