Primary gastrointestinal lymphoma - duodenum

Case contributed by Dr Hoe Han Guan

Presentation

Sudden onset of severe abdominal pain for 1 day with vomiting.

Patient Data

Age: 70 years
Gender: Male

Air under diaphragm - pneumoperitoneum. 

Technical: poor penetration, under inspiratory, mild rotation to the right.

Large heterogeneously enhancing eccentric/exophytic intraperitoneal mass located near the third part of the duodenum (D3). This mass communicates with the lumen of D3 duodenum as evidenced by extravasation of positive oral contrast from the duodenum into the mass. It encases the proximal part of the superior mesenteric artery as well as the distal part of the superior mesenteric vein resulting in short segments of non-opacification. Posteriorly, the mass abuts both inferior vena cava and abdominal aorta. Slit-like inferior vena cava. The mass displaces the surrounding bowel loops without clear evidence of infiltration.

Large amount of air pockets are seen within the mass. Large volume pneumoperitoneum. Minimal free fluid in perihepatic, perisplenic and bilateral paracolic gutters.

Liver and spleen are not enlarged. No significant enlarged pelvic lymph nodes.

No proximal bowel loops dilatation to suggest complete obstruction. 

Minimal bilateral pleural effusion. Cardiomegaly with minimal pericardial effusion.

Case Discussion

Intraperitoneal solid mass with closed proximity to the duodenum. By virtue of its large size without significant perilesional fat streakiness or local infiltration, the top two differential diagnoses are primary gastrointestinal tract lymphoma and gastrointestinal stromal tumor (GIST). Differentiating these two is challenging, where lymphadenopathy is more common in GIT lymphoma.

The intraperitoneal mass had been biopsied prior to this and histopathological examination (HPE) tissue showed extranodal diffuse large B cell lymphoma (DLBCL), non-germinal center B-cell (NGCB) subtype.

​The pneumoperitoneum is caused by the rupture of this tumor. The patient went on to have an emergency laparotomy for excision and repair.

The most common extranodal site for non-Hodgkin's lymphoma (NHL) is the gastrointestinal tract (GIT). Primary GIT lymphoma comprises only 3% to 4% of all GIT malignancies. The stomach is the most common site of PGIL, followed by the small intestine and colon.

Dawson’s criteria 1 are used for the diagnosis of primary gastrointestinal lymphoma, which include

  1. absence of palpable peripheral lymphadenopathy at the time of presentation
  2. lack of enlarged mediastinal lymph nodes
  3. normal total and differential white blood cell count
  4. predominance of bowel lesion at the time of laparotomy with only lymph nodes obviously affected in the immediate vicinity (drainage)
  5. no lymphomatous involvement of liver and spleen

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