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Lymphomatosis - abdominal and peritoneal

Case contributed by Michael P Hartung
Diagnosis certain

Presentation

Restaging of abdominal malignancy.

Patient Data

Age: 60 years
Gender: Male

Oval nodule in the anterior right pleural space. Small to medium left effusion with pleural thickening and nodularity. Trace loculated anterior pericardial effusion. Few enlarged anterior costophrenic angle lymph nodes.

Several low-attenuation liver lesions, likely cysts. Two low-attenuation splenic lesions. Left greater than right adrenal thickening. No renal lesions. Extensive periaortic/retroperitoneal soft tissue/adenopathy. Prominent gastrohepatic ligament soft tissue abuts the pancreas. Oval nodule above the transverse colon. Two small nodules in the left lower retroperitoneum.  Pelvic adenopathy. 

Cocoon/encased appearance of the small bowel within the mesentery. Very large mass centered within the mesentery. Soft tissue and peritoneal thickening with fluid surrounding the encased small bowel loops. Small amount of free pelvic fluid.

TISSUES: Lymph node 

GROSS DESCRIPTION: Specimen submitted in formalin labeled core biopsy of abdominal mass consists of 5 cylindrical cores of pale tan tissue ranging from 1.7 to 3 cm in length and 0.1 cm diameter. 

MICROSCOPIC DESCRIPTION: Sections show about a half-dozen needle biopsy fragments with a nodular and diffuse infiltrate of mostly small, cleaved centrocytes within a sclerotic background.

Special Stains Performed:
BCL-6 Positive Strong nuclear staining in areas with follicular architecture with only dim-equivocal staining in the more diffuse areas.
CD20 Positive
CyclinD1 Negative
Ki67MIB1 See comment Low proliferation fraction: <5% in most areas, but up to 20-30% focally.

FINAL DIAGNOSIS:
Follicular lymphoma, grade 1-2.

Case Discussion

Extensive abdominal and peritoneal involvement of lymphoma, with retroperitoneal nodal disease, splenic lesions, peripancreatic/pancreatic lesions, and extensive peritoneal involvement with cocooning of the small bowel. There is partially imaged nodal and pleural disease in the chest. This patient had a longstanding history of relapsing follicular lymphoma, with pronounced worsening of disease in this examination. 

The appearance can overlap with peritoneal carcinomatosis, tuberculosis and peritoneal mesothelioma.

Ultrasound guided biopsy of the mesenteric mass can be safely performed for tissue biopsy. 

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