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Non-Hodgkin's Lymphoma

Bang Huu Huynh, MD - Case Coordinator
John Dewolfe Mackenzie, MD - Radiology Discussion
Frank Samuel David, MD, PhD - Pathology Discussion
John M Braver, MD - Attending Radiologist
Pablo R Ros, MD, MPH - Attending Radiologist

October 28, 2002

Presentation

A 32-year-old man presented with intermittent nausea and vomiting. His medical history included cardiac transplant.

Imaging Findings

Barium Study
Computed Tomography
Gross Pathology Specimen
Histology

On barium enema images, contrast is visible in the small bowel. The folding pattern of the jejunum appears to be normal, but there is apparent displacement of the small bowel on the left side that suggests the presence of a mass. On the spot view, a "coiled spring" appearance-typical of intussusception-is clearly visible in the upper left quadrant.

In a man of this age, a tumor-although rare-is the most likely cause of intussusception. This was clearly a surgical case. However, additional images were needed for staging. Because endoscopy is difficult in the mid-small bowel, the patient was sent for computed tomography (CT).

CT images show a soft tissue mass that projects into the lumen of the small bowel in the left mid-abdomen. There appears to be a focus of low attenuation that may be fat from the small bowel, illustrating the intussusception "pulling in" the mesenteric fat. The mass is well defined, and there is no apparent stranding or inflammation of the surrounding fat. The mass appears homogeneous. Even though this is a non-contrast study, the resident suggests that the mass is projecting in from the muscle wall.

Differential Diagnosis

The differential diagnosis includes leiomyoma and lymphoma. Cardiac transplant increases a patient's risk of developing localized lymphoma of the bowel; this possibility is a good match for the findings.

Diagnosis

Non-Hodgkin's lymphoma, diffuse large B-cell type

Discussion

Pathology Discussion

A section of small bowel and several mesenteric nodules were removed. The 6.5-cm pedunculated mass is shown to be a homogeneous (white-yellow) submucosal lesion. Microscopic images show that the mass, which consists of a diffuse, homogeneous infiltrate of lymphoid cells, is bordered by normal mucosa (top). At higher magnification, it is clear that the cells have prominent mitotic figures and scant cytoplasm.

Given this patient’s history, B-cell lymphoma is the most likely diagnosis. Due to a previous treatment, however, the cells did not stain positive for CD79a, a common B-cell marker. The cells were positive for CD20, another B-cell marker. Further testing with Ki-67, a proliferation marker, indicated non-Hodgkins lymphoma.

The small (up to 2.5 cm), hemorrhagic nodules from the mesentery had the same results.

Masses associated with post-transplant proliferative disorder are generally EBV-related. In this case, however, the results of in situ hybridization for EBV early mRNAs were negative.

Radiology Discussion

Intussusception is a form of obstruction that typically occurs within the ileum and colon, but can occur elsewhere in the bowel. It occurs when a portion of the bowel—the intussuscetum—"slips inside" another portion of the bowel—the intussuscepiens—like a collapsing telescope. The typical presentation is abdominal pain, vomiting, palpable abdominal mass, and "currant jelly" stools.

Radiological diagnosis of intussusception is aided by the involvement of mesenteric fat. When the intussusecptum slips inside the intussuscepiens, mesenteric fat is dragged with it. The combination of these three layers—the intussuscepiens, mesenteric fat and intussusceptum—creates characteristic signs on various imaging modalities. On ultrasound images, concentric rings can be seen: reflections from the mesenteric fat form a bright, central ring, while the two apposed and edemoutous mucosal surfaces form the hypoechoic, outer ring. The characteristic "coiled spring" appearance is seen on barium enema studies. It is formed with the column of barium cannot advance past the obstruction, but instead advances around the margins of the filling defect. On CT images, intussusception has a "target" appearance, formed by the three layers: outer intussuscepiens, mesenteric fat, and inner intussusceptum.

Intussusception is the leading cause of bowel obstruction in children. Most pediatric cases are idiopathic but are thought to be related to lymphoid hyperplasia and mucosal edema. Reduction can be attempted with air enema. In adults, the cause can generally be identified. A lead point, such as tumor or Meckel diverticulum, is often responsible for encouraging the intussusception. Lipoma and adenocarcinoma are the most common entities. Attempts at reduction of intussusception in adults are much less successful than in children; treatment usually requires surgery.

References

University of Utah Department of Pathology, www.path.utah.edu

University of Pennsylvania Department of Radiology, www.rad.upenn.edu

South Bank University, London, http://www.lsbu.ac.uk/


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