Disclosures Philips, consultant Case Presentations: Problem Cases - PowerPoint PPT Presentation

5/28/2016 Disclosures • Philips, consultant Case Presentations: Problem Cases from the Liver/GI Consult Service Ryan M. Gill What kind of case requires consultation? What kind of case requires consultation? 1. Common tumor, but something is unusual 1. Common tumor, but something is unusual 2. Uncommon tumor or uncertain malignant potential 3. Diagnosis and clinical scenario discordant 2. Uncommon tumor or uncertain malignant potential 4. Distinct features of two different disease processes 3. Diagnosis and clinical scenario discordant 5. Uncertainty about a new classification/reporting system 4. Distinct features of two different disease processes 6. Limited sample, non-diagnostic 7. Active liver injury, etiology uncertain 8. Pediatric liver disease 1

5/28/2016 CASE • Adult female with hepatic rupture and a 10 cm entirely necrotic hepatic mass CK7 2

5/28/2016 CD68 Fibrolamellar HCC Ross HM, Daniel HD, Vivekanandan P, Kannangai R, Yeh MM, Wu TT, Makhlouf HR, Torbenson M. Fibrolamellar carcinomas are positive for CD68. Mod Pathol. 2011, Mar;24(3):390-5. Graham RP, Jin L, Knutson DL, Kloft-Nelson SM, Greipp PT, Waldburger N, Roessler S, Longerich T, Roberts LR, Oliveira AM, Halling KC, Schirmacher P,Torbenson MS. DNAJB1-PRKACA is specific for fibrolamellar carcinoma. Mod Pathol. 2015 Jun;28(6):822-9. CASE • Adult male with a strongly enhancing 3 cm liver tumor on CT-scan • Radiologist differential includes HCC, neuroendocrine tumor, and vascular tumor • Biopsy was concerning for angiosarcoma Patient underwent resection 3

5/28/2016 4

5/28/2016 CD34 Table 1: Clinical features and outcome HSVN (n=17) AS (n=10) CH (n=6) Average age (range) 54 years (24 – 83 years) 51 years (34-69 years) 48 years (36-63 years) Gender (M:F) 13:4 4:6 3:3 Average size (range) 2.1 cm (0.2 – 5.5 cm) 6.2 cm (2.5 – 10 cm) 6.6 cm (1.1 - 15 cm) Metastasis None Lung, heart, bone None Outcome ARD(6)/ANED(6) DOD(2) ANED (4) Maximum follow up (months) 72 7 72 HSVN – Hepatic small vessel neoplasm; AS – Hepatic angiosarcoma; CH - Cavernous hemangioma; ARD – Alive with residual disease; ANE – Alive with no evidence of disease; DOD – Dead of disease 5

5/28/2016 Hepatic Small Vessel Neoplasm Gill RM, Buelow B, Mather C, Joseph NM, Alves, V, Brunt EM, Liu T, Makhlouf H, Marginean C, Nalbantoglu I, Sempoux C, Snover DC, Thung SN, Yeh MM, Ferrell LD. Hepatic Small Vessel Neoplasm, a Rare Infiltrative Vascular Neoplasm of Uncertain Malignant Potential. Human Pathology. 2016, 10.1016/j.humpath.2016.03.018. 6

5/28/2016 CASE • Adult female with a 5 cm well-circumscribed mass with CT imaging suggestive of steatosis • Radiologist favors HCC • Biopsy was performed Glutamine Synthetase 7

5/28/2016 Glutamine Synthetase Image courtesy of Linda Ferrell, MD Image courtesy of Linda Ferrell, MD 8

5/28/2016 HCC (“steatohepatitic variant”) Fatty FNH as a mimic of HCC Deniz K, Moreira R, Yeh M, Ferrell L. Steatohepatitis-Like Changes in Focal Nodular Hyperplasia, a Finding Not to Be Confused with Steatohepatitic Variant of Hepatocellular Carcinoma. Mod Path (supple 2): 418A, 2014. Image courtesy of Linda Ferrell, MD Central zone arterioles in NASH Advanced Fibrosis 9

5/28/2016 Clinical Significance • Arteries in scarred central zones and unpaired arteries in parenchyma are common in NASH and should not suggest a neoplasm Centrizonal Arteries in Non-alcoholic • Sinusoidal capillarization is common in Steatohepatitis (NASH) NASH and does not suggest neoplasia Gill RM, Belt P, Wilson L, Bass NM, Ferrell LD. Centrizonal Arteries and Microvessels in Non-Alcoholic Steatohepatitis. American Journal of Surgical Pathology. 35(9):1400-4, 2011 CASE • Adult female with fatty liver on ultrasound, mild transaminitis, elevated ALP, and hyperlipidemia • Core liver biopsy performed to rule out NASH or other process 10

5/28/2016 CK7 11

5/28/2016 CASE • Adult female with a 6 cm liver mass, stable in size since 2012, who underwent wedge NASH with primary biliary biopsy cholangitis (PBC) Gill RM and Kakar S. Non-Alcoholic Steatohepatitis: An Update on Diagnostic Challenges. Surgical Pathology Clinics, 6(2):227-257, 2013. 12

5/28/2016 LFABP 13

5/28/2016 • Well differentiated hepatocellular neoplasm Complete loss of LFABP staining is seen in ~30% with patchy reticulin fragmentation and of HCC, including well differentiated HCC LFABP loss • Recommend resection for definitive Cho SJ, Ferrell LD, Gill RM. Expression of liver fatty acid binding protein in hepatocellular carcinoma. Human Pathology. 2016, 50, 135-139. classification Another pitfall 14

5/28/2016 LFABP Arginase HMB-45 15

5/28/2016 SMA CASE • Adult female with acute myeloid leukemia, on induction chemotherapy for allogeneic BMT, presented with right lower quadrant pain and CT imaging suggestive of acute appendicitis • Laparoscopic appendectomy was performed and converted to open ileocecectomy due to necrotic appendix and ileum 16

5/28/2016 GMS GMS Suspect angio-invasive fungal infection in neutropenic patients with ischemic bowel Choi W., Chang T., Gill RM . Gastrointestinal Zygomycosis Masquerading as Acute Appendicitis. Case Reports in Gastroenterology. 2016, 10:81-=87. 17

5/28/2016 CASE • Adult male presented with fever, chills, nausea, and abdominal pain • CT showed rim enhancing liver lesions with differential between metastatic colon cancer, amoebic abscess, or bacterial abscess related to sigmoid diverticulitis • Biopsy performed, tissue culture is negative 18

5/28/2016 Fusobacterium sp. infection should be considered in culture negative hepatic abscess Buelow, B., Lambert J., Gill RM. Fusobacterium Liver Abscess. Case Reports in Gastroenterology. 2013, 7(3):482-6. 19

5/28/2016 CASE • Adult male with history of low grade fever, • Imaging: abdominal pain, weight loss, Crohns disease, – CT scan confirms massive ascites and identifies and hepatosplenomegaly. retroperitoneal lymphadenopathy. – No liver lesions • Transaminases mildly elevated • On steroids and infliximab • Transjugular liver biopsy is performed to • EBV serology positive assess the etiology of acute liver dysfunction Sinusoidal Infiltrate 20

5/28/2016 Focal Necrosis Hemophagocytosis Cytologic Atypia Mitotic Activity 21

5/28/2016 CD3 CD3 CD5 CD5 22

5/28/2016 CD56 CD56 EBER ISH Aggressive NK Cell Leukemia � Similar presentation to HSTL and fulminant course � NK cell neoplasm with a leukemic component � CD2+, cCD3+, CD56+, TIA-1+, Granzyme B + � T-cell markers negative (sCD3, CD5) � EBER positive, TCR genes germline � Hemophagocytosis 23

5/28/2016 Prolonged Transaminitis Following CD3 Acute Viral Illness EBER ISH + EBV Hepatitis 24