—  SPECIALTY CONFERENCE  — Liver Pathology Sunday, March 8, 2009, 7:30 PM Convention Center BRA Cells of the Liver Moderator: ELIZABETH M. BRUNT Washington University School of Medicine, St. Louis, MO Disclosure: In accordance with ACCME guidelines regarding disclosure, the USCAP policy requires that faculty members who have a significant financial or other relationship with a commercial company, entity, or service (which will be discussed in this Symposium) must disclose this to attendees. The Academy also requires that speakers disclose any products that are not labeled for the use under discussion. The speakers listed below have indicated they have nothing to disclose. Panelists: ZACHARY GOODMAN, Armed Forces Institute of Pathology, Washington, DC LAURA RUBBIA-BRANDT, University of Geneva, Geneva, Switzerland MAHA GUINDI, University of Toronto, Toronto Ontario, Canada MATTHEW YEH, University of Washington, Seattle, WA JAY LEFKOWITCH, Columbia University, New York, NY Clinical histories are displayed below. For the fastest viewing of virtual slides, click: under each thumbnail image below. You must have Aperio ImageScope installed on your PC. If you do not already have Aperio ImageScope, Windows users with administrator privileges may download and install a free version in order to view USCAP Virtual Slides. Click the icon on the right to get your free copy:   Or, click on slide thumbnail images to view each slide in a Web-based slide viewer, which is somewhat slower. If you have any difficulties viewing these slides, email or call George Clay at +1.724.449.1137. Case 1 Submitted by: Zachary Goodman - Armed Forces Institute of Pathology, Washington, DC Clinical Summary: The patient is a 45 year old man who was injured by an improvised explosive devise (IED) in Iraq, suffering severe blast and shrapnel wounds to both lower extremities and the right hip with open comminuted fracture of the iliac wing. He was hypotensive on arrival at the combat support hospital in Baghdad, where he was transfused with 33 units of blood and packed red cells and other blood products before and during surgery to debride the wounds and evacuate a large retroperitoneal hematoma. He developed acute renal failure, but two days later when stable he was transferred to a medical center in Germany. He spent the next three weeks in intensive care on assisted ventilation and hemodialysis with gradually improving cardiopulmonary and renal function. The abdominal, hip and lower extremity wounds were left open for washout and debridement every 2 to 3 days. Complications included at least 2 episodes of gram negative sepsis and an episode of ventilator-associated pneumonia as well as infections in the hip and lower extremity wounds, treated with multiple antibiotics, primarily levofloxacin, meropenem and vancomycin. Total parenteral nutrition was administered from the eighth to the 22nd day after injury. On the 16th day after injury an open cholecystectomy and liver biopsy were performed because of elevated bilirubin and alkaline phosphatase, fever, leukocytosis and thickened gallbladder (? ultrasound). The abdominal wound was gradually closed over the next 6 days, and he was weaned from the ventilator, dialysis and TPN. The patient continued to improve and was sent to a hospital in his home country on the 25th day after injury. Case1 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 1 - Slide 2 Click to view with ImageScope Click to view with a Web-Based Viewer Case 2 Submitted by: Laura Rubbia-Brandt - University of Geneva, Geneva, Switzerland Clinical Summary: A 65 year old man, with a rectal carcinoma since 3 years, was referred to our center for evaluation of surgical resection of metachronous liver metastases. Preoperative assessment showed absence of underlying chronic hepatic disease serologically confirmed. A CT scan revealed four hepatic metastases measuring from 3 to 7 cm in the right liver, and one metastasis of 2 cm in the left liver. Because of their number, size and location, they were initially estimated unresectable. Therefore preoperative oxaliplatin based-chemotherapy was performed. After six cycle of chemotherapy, a new CT scan showed only three metastases measuring now from 2 to 4 cm, corresponding to a radiologic response with shrinkage of three metastases and radiological disappearance of two others. Hepatic metastases were now surgically resectable. A major right hepatectomy associated to left hepatic tumorectomy was scheduled. Three weeks prior to this major liver resection, a percutaneous right portal vein embolisation was done in order to hypertrophy the left remaining liver and avoid the risk of postoperative liver insufficiency. At operation the liver was macroscopically blue and with a firm consistence. Hepatic bleeding during parenchymectomy necessitated peroperative blood transfusion. Postoperatively despite adequate remaining hepatic volume, the patient showed transitory increase in transaminases and decrease in prothrombin time of less than 50% of normal in addition to serum bilirubin more than 50 mmol/L on postoperative till day 9. The liver function tests normalized, except for GGT. He clinically did well but could be discharged only on postoperative day 14. The patient is alive 2 years after operation without tumoral recurrence. Case 2 - Figure 1 Case 2 - Figure 2 Case 2 - Figure 3 Case 2 - Figure 4 Case 2 - Figure 5 Case 3 Submitted by: Maha Guindi - University of Toronto, Toronto Ontario, Canada Clinical History: This is a previously healthy young man with a presumptive diagnosis of Ehlers Danlos syndrome - this was made on the basis of physical findings, and strong family history. He developed a biochemical hepatitis with icterus following a cruise holiday to Honduras, Grand Canyon, Mexico in March 2008. Two months later he developed a fluctuating illness characterized by nausea, pruritus, dark urine. No significant pain. No obvious drug precipitants. US- small hemangioma. MRCP normal. Viral hepatitis screen negative for A, B, C and EBV. Autoantibody screen and immunoglbulins normal. Normal ceruloplasmin. Biochemically and symptomatically whilst initially settling he began to relapse. Laboratory tests: TBili AST ALT ALP Albumin INR May 2008 72 909 2220 136 45 June 2008 37 678 1552 137 43 0.89 July 2008 12 258 752 76 44 1.93 August 2008 53 1141 2482 105 42 0.96 Case 3 - Figure 1 Case 3 - Figure 2 Case 3 - Figure 3 Case 3 - Figure 4 Case 3 - Figure 5 Case 3 - Figure 6 Case 3 - Figure 7 Case 4 Submitted by: Matthew Yeh - University of Washington, Seattle, WA Clinical Summary: A 59-year-old man with cirrhosis due to chronic hepatitis C had a solitary 2 cm liver lesion in segment 5. Three phase CT scan showed a hypervascular lesion on arterial phase with rapid washout on delayed phase. Orthotopic liver transplantation was performed. Gross and microscopic examinations show the following: Case 4 - Figure 1 Case 4 - Figure 2 Case 4 - Figure 3 Case 4 - Figure 4 Case 4 - Figure 5 Case 4 - Figure 6 Case 4 - Figure 7 Case 4 - Figure 8 Case 4 - Figure 9 Case 5 Submitted by: Jay Lefkowitch - Columbia University, New York, NY Clinical Summary: A 19 year old woman with known SLE was transferred from an outside hospital to our medical center for management of her marked transaminitis (AST = 1318 IU/L and ALT = 2375 IU/L), episodic thrombocytopenia, fever and persistently high ferritin. Her SLE had been diagnosed 4 years earlier, with positive smooth muscle and ribonucleoprotein antibodies and complications including lupus cerebritis and avascular necrosis of both hips. Six months before transfer she presented at another hospital with AST/ALT 9000 and respiratory, kidney and liver failure with uncontrolled bleeding. She was diagnosed with macrophage activation syndrome (MAS) (bone marrow biopsy failed to demonstrate erythrophagocytosis or other diagnostic features, but liver biopsy was reported positive) and responded with aggressive medical management, including immunosuppression (steroids, cyclosporine A, then rituximab-anti-CD20 antibody, and kineret-anakinra, an interleukin-1 receptor antagonist). At transfer, in addition to the marked aminotransferase elevations she had an INR of 1.29, total bilirubin of 2.9 mg/dl (direct of 1.7) and albumin of 3.7 g/dl. Her 3-week hospitalization was characterized by rising aminotransferases to >3000 IU/L, hypotensive shock with positive blood cultures for enterococcus, serum ammonia of 785, free air in the abdomen and gastrointestinal hemorrhage requiring laparotomy and segmental colectomy (intraluminal blood was found, but no perforation was identified). During her final week of life there was diffuse cerebral edema and impending transtentorial herniation, INR of 2.83 with elevated fibrinogen and D-dimers and continued marked transaminitis. She remained in liver failure, critically ill and her family authorized withdrawal of support. Case 5 - Figure 1 Case 5 - Figure 2 Case 5 - Figure 3 Case 5 - Figure 4 Case 5 - Figure 5 Case 5 - Figure 6 Case 5 - Figure 7 Case 5 - Figure 8 Case 5 - Figure 9 Case 5 - Figure 10 Case 5 - Figure 11 Case 5 - Figure 12 If there are glass slides for a conference, they will be available for study in the microscope room in the Convention Center Room 313 for participants who wish to review them prior to the evening session. Handouts for all Specialty Conferences will be accessible via the "Educational Materials" section on the homepage the morning after each respective conference. Printed copies of the handout will not be available at the meeting. 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