Contributed by Joey Oakley MD and Scott Owens MD
Published on line in January 2007
PATIENT HISTORY: The patient is a 71 year old male with right flank pain radiating to the back for 3 weeks, with an unintentional ten pound weight loss. A recent bout of melena and vomiting of coffee-ground material caused him to seek care at an outside institution. His work-up revealed a single gallstone and extrahepatic bile duct dilatation to 14 mm on ultrasound, with a total bilirubin of 2.8 mg/dl and an alkaline phosphotase of 293 IU/ml. The patient had been maintained on Coumadin for pulmonary embolism prophylaxis, and had an INR of 4.5. His hematocrit was 26% at the outside institution. Cholecystectomy was performed, and the patient developed a post-operative bile leak. A JP-tube was placed, and the patient transferred to a second outside facility. This hospital performed an upper endoscopy and found a friable periampullary mass causing biliary obstruction. The patient was then sent to UPMC for evaluation. Computed tomography revealed a 5 cm periampullary mass and periaortic lymphadenopathy. Upper endoscopy revealed a circumferential mass, around which the endoscope could not be passed. Biopsies were obtained. Serum markers were obtained, and hCG, CEA, and alpha-fetoprotein were within normal limits. CA19-9 was elevated at 40.4. Lipase levels were drawn after upper endoscopy and biopsy, and were elevated at 1,850 IU/mL.
Past medical history is significant for seminoma in 1999 with orchectomy and chemotherapy complicated by peripheral neuropathy. He was "told he had a recurrence" in 2002, with unclear follow-up. The patient had not received adjuvant therapy in three years prior to presentation. In addition, the patient had deep venous thrombosis with pulmonary embolism with pulmonary thrombectomy, lifelong Coumadin prophylaxis, and Greenfield filter placement in the inferior vena cava. Mild chronic renal insufficiency was also noted. He has a 60 pack-year history of smoking.
The biopsy specimen was received as four irregular rubbery, tan-gray, and focally hemorrhagic tissue fragments ranging 0.3 to 0.4 cm. These were entirely submitted in a single cassette.
Microscopic examination revealed an infiltration of the duodenal mucosa by large cells with abundant clear to slightly foamy cytoplasm, well-delineated cell borders, with irregularly shaped nuclei with granular chromatin, and prominent, irregular nucleoli. Rare mitotic figures could be observed. No dysplasia of the intestinal glands was noted, although reactive atypia, mostly in the form of prominent nucleoli, could be identified (Figures 1 and 2). Immunohistochemical stains to characterize the large infiltrative cells were performed. Staining for CD68 and Leukocyte Common Antigen (CD45) highlighted occasional macrophages and lymphocytes respectively, but was negative in the large infiltrative cells (Figures CD68 and LCA). AE1/AE3 cytokeratins were strongly positive in the normal reactive duodenal glands, but were negative in the infiltrative large cells (Figure AE13). S-100 was negative (Figure S100). Staining with placental leukocyte alkaline phosphatase (PLAP) was positive in the cytoplasm of the large infiltrative cells, with a string-like, foamy pattern (Figures PLAP1 and PLAP2).