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

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Case Presentations: Problem Cases from the Liver/GI Consult Service

Ryan M. Gill

Disclosures

• Philips, consultant

What kind of case requires consultation?

• 1. Common tumor, but something is unusual
• 2. Uncommon tumor or uncertain malignant potential
• 3. Diagnosis and clinical scenario discordant
• 4. Distinct features of two different disease processes

What kind of case requires consultation?

• 1. Common tumor, but something is unusual
• 2. Uncommon tumor or uncertain malignant potential
• 3. Diagnosis and clinical scenario discordant
• 4. Distinct features of two different disease processes
• 5. Uncertainty about a new classification/reporting system
• 6. Limited sample, non-diagnostic
• 7. Active liver injury, etiology uncertain
• 8. Pediatric liver disease

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CASE

• Adult female with hepatic rupture and a 10

cm entirely necrotic hepatic mass

CK7

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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

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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

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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.

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CASE

• Adult female with a 5 cm well-circumscribed

mass with CT imaging suggestive of steatosis

• Radiologist favors HCC
• Biopsy was performed

Glutamine Synthetase

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Image courtesy of Linda Ferrell, MD

Glutamine Synthetase

Image courtesy of Linda Ferrell, MD

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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.

HCC (“steatohepatitic variant”)

Image courtesy of Linda Ferrell, MD

Central zone arterioles in NASH Advanced Fibrosis

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Clinical Significance

• Arteries in scarred central zones and

unpaired arteries in parenchyma are common in NASH and should not suggest a neoplasm

• Sinusoidal capillarization is common in

NASH and does not suggest neoplasia

Centrizonal Arteries in Non-alcoholic Steatohepatitis (NASH)

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
• r other process

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CK7

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NASH with primary biliary cholangitis (PBC)

Gill RM and Kakar S. Non-Alcoholic Steatohepatitis: An Update on Diagnostic Challenges. Surgical Pathology Clinics, 6(2):227-257, 2013.

CASE

• Adult female with a 6 cm liver mass, stable in

size since 2012, who underwent wedge biopsy

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LFABP

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• Well differentiated hepatocellular neoplasm

with patchy reticulin fragmentation and LFABP loss

• Recommend resection for definitive

classification Complete loss of LFABP staining is seen in ~30%

• f HCC, including well differentiated HCC

Cho SJ, Ferrell LD, Gill RM. Expression of liver fatty acid binding protein in hepatocellular carcinoma. Human Pathology. 2016, 50, 135-139.

Another pitfall

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LFABP Arginase HMB-45

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SMA CASE

• Adult female with acute myeloid leukemia,
• n 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

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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.

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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

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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.

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CASE

• Adult male with history of low grade fever,

abdominal pain, weight loss, Crohns disease, and hepatosplenomegaly.

• Transaminases mildly elevated
• On steroids and infliximab
• EBV serology positive
• Imaging:

– CT scan confirms massive ascites and identifies retroperitoneal lymphadenopathy. – No liver lesions

• Transjugular liver biopsy is performed to

assess the etiology of acute liver dysfunction

Sinusoidal Infiltrate

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Focal Necrosis Hemophagocytosis Cytologic Atypia Mitotic Activity

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CD3 CD3 CD5 CD5

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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

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CD3 EBER ISH + EBV Hepatitis