Case 1: Submitted by Dr. Meenakshi Singh | Renaissance School of Medicine at Stony Brook University

Posted June 28, 2010

Granulomatous inflammation presenting as ileocecal mass with bladder and abdominal wall involvement
Yuanming Zhang¹, Roberto Bergamaschi², Philip Kane¹ and Meenakshi Singh¹

¹Departments of Pathology and ²Division of Colon and Rectal Surgery, State University of New York at Stony Brook and Stony Brook University Medical Center, Stony Brook, New York.

This case was presented as a poster at the College of American Pathologists (CAP) annual meeting in Washington, DC, in October 2009.

ABSTRACT

We report a case of a 34-year-old male who presented with recurrent urinary tract infection and intestinal obstruction. CT scan revealed a mass involving the ileocecum, urinary bladder, and abdominal wall. Frozen sections of the ileocecal mass demonstrated transmural granulomatous inflammation that also involved the bladder and abdominal wall. Abundant foreign body type giant cells were found, some with polarizable foreign material, identified as vegetable matter, consistent with a phlegmon, secondary to long-standing bowel perforation. There was no evidence of tuberculosis, fungal infection, Crohn's disease or sarcoidosis. There had been no prior surgery. The presence of foreign body giant cells with polarizable foreign material helped establish the nature of the inflammation. Food material in the bowel wall may serve as foreign matter and an exuberant response to it can lead to a mass that mimics a tumor.

INTRODUCTION

Crohn's disease, Mycobacterium tuberculosis, atypical mycobacteria, fungal infection, parasitic infection, sarcoidosis and foreign body reaction (1) are leading causes of granulomatous inflammation of the ileocecum. Some of these conditions may mimic a tumor with complications such as small bowel obstruction, perforation, and fistula formation (2,3). Among these, Crohn’s disease and tuberculosis are more common causes of such manifestations. Correlation of clinical, endoscopic, radiologic, and histologic findings is necessary for making an accurate diagnosis. This is clinically relevant because the treatment and management of these conditions are quite different (1,4).

REPORT OF A CASE

A 34-year-old African American male presented with 1-month history of anorexia, weight loss, recurrent urinary tract infection, increasing abdominal pain, and 1-week history of progressive abdominal distension. He had no prior abdominal surgery. A colonoscopy before admission suggested Crohn's disease. No biopsy had been performed. The past medical history was significant for a seizure disorder. Physical examination showed abdominal distension, tympanism, and tenderness in the right lower quadrant. CT scan of the abdomen revealed partial small bowel obstruction and a pelvic mass in the right lower quadrant and proximal small bowel obstruction with dilated small bowel loops showing air fluid level (Fig.1). The patient was initially treated conservatively with placement of a nasogastric tube, nil per os, total parental nutrition and intravenous antibiotics. Because there was no improvement over the next 2 weeks, the patient underwent exploratory laparotomy. The ileocecal mass extended to the dome of the bladder and the abdominal wall. An en bloc ileocecal resection with partial cystectomy and abdominal wall excision was performed.

An intraoperative examination of the specimen disclosed distorted ileocecal bowel adherent to a tan-yellow, firm mass measuring 5.5x5.5x4.5 cm (Fig. 2). The bowel mucosa away from the mass was normal on gross examination. The mass extended to the dome of the bladder. Additional tissue retrieved from the extension to the abdominal wall showed a gross appearance similar to the mass. A frozen section analysis of the mass revealed granulomatous inflammation with numerous foreign-body giant cells (Fig. 3). No tumor was identified. On permanent sections, non-necrotizing granulomatous inflammation was confirmed in the intestinal mass, the bladder and the abdominal wall. There were abundant foreign-body giant cells (Fig. 4), some of which contained polarizable foreign material and other vegetable matter (Fig. 5). Special stains for acid fast bacilli and fungal organisms were negative. The scenario was consistent with a phlegmon secondary to long-standing bowel perforation. Cultures were negative.

The patient tolerated the surgical procedure well, regained bowel function, resumed diet, and was discharged.

COMMENT

Granulomatous inflammation of the bowel raises the differential diagnosis of infectious and non-infectious diseases. Mycobacterium tuberculosis, atypical mycobacteria, fungal organisms, and parasitic infestation have been reported to involve the gastrointestinal tract producing granulomatous inflammation (1, 5, 6). Tuberculosis is far more common than other infectious causes. The most common site of tuberculosis involvement of the gastrointestinal tract is the ileocecum (7,8). Ileocecal tuberculosis may present as a palpable mass in the right lower quadrant and cause complications of obstruction and perforation. Pertinent diagnostic information includes a typical clinical history, chest X-ray, positive PPD, positive culture for mycobacteria and AFB stain, caseating granulomata on histologic evaluation, elevated ascitic fluid adenosine deaminase level and a positive polymerase chain reaction (PCR) test for mycobacterium tuberculosis. Each test mentioned above has its own limitations in terms of specificity and sensitivity in detecting tuberculosis. Not infrequently, tuberculosis is diagnosed at the time of surgery (1, 2, 3, 7, 8, 9,10).

In the non-infectious category, Crohn’s disease, sarcoidosis and foreign body reaction can present with granulomatous inflammation of the bowel (1). Crohn’s disease is a far more common cause of small bowel obstruction and perforation compared to the other listed conditions. Classic gross and microscopic features aid in making the diagnosis of Crohn’s disease. The latter include segmental distribution of lesions, frequent ileal involvement, transmural inflammation, transmural lymphoid aggregates, and frequent formation of fissures, strictures, abscesses, and fistulas, in addition to granulomas. No definitive diagnostic test exists for Crohn’s disease. Instead, the diagnosis is based on a combination of history, physical examination, endoscopic findings, radiographic appearance, and pathologic evaluation (4, 11, 12).

Sarcoidosis of the gastrointestinal tract is rarely seen in patients with disseminated sarcoidosis. However, the granulomatous inflammation in sarcoidosis is seen more in the mucosa. No transmural inflammation and lymphoid aggregates are present. Corticosteroid treatment is effective (13).

A past visit to endemic areas of schistosomiasis should raise the possibility of intestinal schistosomiasis, which may also present as an ileocecal mass, as reported by Lamyman et al (5). Histologic evaluation may reveal extensive ulcers penetrating into the muscularis and transmural inflammation with lymphoid aggregates, thereby mimicking Crohn’s disease. However, the presence of a large number of calcified eggs with associated foreign-body giant cells can confirm the diagnosis of intestinal schistosomiasis.

The clinical history and physical examination in the current case revealed subacute bowel obstruction due to an ileocecal mass that raised concern for gastrointestinal malignancy along with a differential diagnosis of tuberculosis, Crohn’s disease and other conditions listed previously. Interestingly, repeat colonoscopies were normal. At exploratory laparotomy the ileocecal mass was submitted for frozen section analysis that did not reveal any malignancy but showed an exuberant granulomatous inflammation. The etiology of the granulomatous response was determined on permanent sections. There were abundant foreign-body type giant cells that on polarized light examination revealed foreign material including vegetable matter within them. A special stain was negative for acid fast bacilli (AFB). A Gomori-Grocott methenamine silver (GMS) stain was negative for fungal organisms. The inflammation extended to the dome of the bladder and the abdominal wall. This is consistent with a phlegmon, secondary to chronic bowel perforation and leakage of bowel contents. The patient had a history of seizures, raising the possibility of having swallowed some material that could have damaged the bowel wall with subsequent leakage and inflammatory response. Pathologic workup did not reveal Crohn’s disease or tuberculosis. The appropriate diagnosis prevented drug treatment for tuberculosis or Crohn’s disease.

We emphasize several lessons from this case. The presence of a mass along with anorexia and weight loss misleadingly suggested a neoplasm or tuberculosis. Granulomatous inflammation raised the differential diagnosis of tuberculosis and Crohn’s disease. Absence of necrotizing granulomas and lack of positive AFB stains and cultures helped rule out tuberculosis. Absence of transmural lymphoid aggregates and inflammation and epithelioid granulomas unrelated to crypts helped rule out Crohn’s disease. A polarized light examination was an important tool in revealing the underlying cause of the granulomatous inflammation in this case. Identification of polarizable foreign material within foreign-body type of giant cells supported the diagnosis of chronic bowel perforation with subsequent foreign-body giant cell reaction. Food material in the wall of the bowel may serve as foreign matter and an exuberant response to it can lead to a mass and mimic a tumor. A detailed history, careful histologic examination and ancillary laboratory studies should be combined to determine the etiology of granulomatous inflammation of the gastrointestinal tract.

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LEGENDS

Figure 1 . CT image of the abdominal mass (green arrow) showing its location and involvement of the abdominal wall and the dome of the bladder (red arrow points to the Foley catheter in the bladder).

Figure 2. Gross photograph of the specimen showing a tan-yellow, firm mass (green arrow) extending from the submucosa to the serosal surface of the partially adhered bowel (blue arrow).

Figure 3. Frozen section of the ileocecal mass showing granulomatous inflammation with abundant foreign-body type giant cells (hematoxylin-eosin, original magnification x20)

Figure 4. Permanent section of the ileocecal mass showing granulomatous inflammation with foreign-body type giant cell with intracytoplasmic vegetable matter (hematoxylin-eosin, original magnification x 40).

Figure 5. Same section as Figure 4 under polarized light showing polarizable intracytoplasmic vegetable matter in a foreign-body type giant cell (hematoxylin-eosin, original magnification x 40).