GUTC SHEILA SEGURA DANBURY HOSPITAL 4915 HPI The

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GUT-C SHEILA SEGURA DANBURY HOSPITAL 4/9/15

GUT-C SHEILA SEGURA DANBURY HOSPITAL 4/9/15

HPI • The patient is a 35 year old female who presented to her

HPI • The patient is a 35 year old female who presented to her clinician complaining of RUQ abdominal pain and history of constipation. • No significant past medical or surgical history. • Increased BMI index (54. 1). • History of oral contraceptive pill use. • An abdominal ultrasound was performed showing multiple liver nodules.

CT ABDOMEN W/ + W/O CONTRAST 09/17/2010 • Multiple hyperdense lesions throughout the liver.

CT ABDOMEN W/ + W/O CONTRAST 09/17/2010 • Multiple hyperdense lesions throughout the liver. • 1. 5 cm up to 7. 0 cm in greatest dimension; largest located in segment 6 • Fatty liver.

MRI ABDOMEN 11/02/10 • Liver with fatty infiltration. • Hypointense liver masses in all

MRI ABDOMEN 11/02/10 • Liver with fatty infiltration. • Hypointense liver masses in all lobes of the liver. • From 1 cm to 6. 9 cm, largest mass in segment 6 of the right lobe. • Peripheral high signal on the diffusion weighted images. • Findings were suspicious for metastatic disease.

DIAGNOSIS RIGHT LIVER LESION, CT-GUIDED CORE BIOPSY: • TELANGIECTATIC (INFLAMMATORY) HEPATOCELLULAR ADENOMA. • The

DIAGNOSIS RIGHT LIVER LESION, CT-GUIDED CORE BIOPSY: • TELANGIECTATIC (INFLAMMATORY) HEPATOCELLULAR ADENOMA. • The hepatocytes were diffusely positive for serum amyloid A protein. • Beta catenin was not aberrantly expressed.

Arch Pathol Lab Med-Vol 138, August 2014 • Uncommon benign neoplasm of liver, hemangioma

Arch Pathol Lab Med-Vol 138, August 2014 • Uncommon benign neoplasm of liver, hemangioma and FNH being more frequent. • Young women of child-bearing age who have a long history of estrogen based OCPs. • Rarely, HAs have been reported in men. • In women using OCPs, the estimated annual incidence is 3 to 4 per 100, 000 per year in North America.

CONT. • Usually, HAs are solitary and asymptomatic lesions. • Diagnosis is usually made

CONT. • Usually, HAs are solitary and asymptomatic lesions. • Diagnosis is usually made on needle core biopsies. • HAs are monoclonal neoplasm with unique molecular signatures. • HAs can be divided into 4 groups, based on the molecular characteristics, immunohistochemical and histological correlation.

HEPATIC ADENOMAS SUBTYPES HA subtype Frequency range % Molecular findings HA-H 35 -40 Somatic

HEPATIC ADENOMAS SUBTYPES HA subtype Frequency range % Molecular findings HA-H 35 -40 Somatic inactivating mutations (85%) of hepatocyte nuclear factor 1α (HNF 1 A) HA-B 15 -19 Activating mutations of βcatenin gene HA-I 30 -35 Gain of function mutations of IL 6 ST; 10% with coexisting β-catenin gene mutations HA-U 10 No specific mutations HA-B, β-catenin-mutated HA; HA-H, HNF 1 A HAs; HA-I, inflammatory HA; HA-U, Unclassified HAs.

RISK FACTORS • HA-H and HA-I : women with history of OCP. • HA-I:

RISK FACTORS • HA-H and HA-I : women with history of OCP. • HA-I: obesity, alcohol, hepatic steatosis, glycogen storage disease type 1, and primary sclerosing cholangitis. • HA-H : familial diabetes or adenomatosis. • HA-B: men, associated anabolic-androgenic steroids use, types I and III glycogen storage disease, Klinefelter syndrome, familial adenomatosis polyposis, and obesity. • Familial form of liver adenomatosis involves a germline mutation of the HNF 1 A gene and is associated with MODY 3.

CLINICAL FEATURES • Usually asymptomatic, with normal LFTs and no elevation in serum tumor

CLINICAL FEATURES • Usually asymptomatic, with normal LFTs and no elevation in serum tumor markers, such as alphafetoprotein. • May be discovered incidentally during diagnostic imaging. • Rupture and spontaneous intratumoral and intraperitoneal bleeding.

CLINICAL FEATURES • Risk of rupture is increased with tumor > 7 cm and

CLINICAL FEATURES • Risk of rupture is increased with tumor > 7 cm and associated oral contraceptive use. • HA-I, signs of chronic anemia or ‘‘systemic inflammatory syndrome’’ • fever, leukocytosis, abnormal LFTs, and elevated levels of acutephase reactants, such as CRP and SAA. • Multiple adenomas (range, 2– 9), with prolonged use of OCPs, type I glycogen storage disease, and obesity.

GROSS PATHOLOGY • Solitary or multiple, unencapsulated, and welldemarcated mass lesion. • Pedunculated or

GROSS PATHOLOGY • Solitary or multiple, unencapsulated, and welldemarcated mass lesion. • Pedunculated or encapsulated. • Soft and fleshy consistency, size ranging from 1 -30 cm. • HA-H, and HA-I cases may form multiple masses.

GROSS PATHOLOGY • Cut surface may be solid tan or yellow (steatosis). • Intratumoral

GROSS PATHOLOGY • Cut surface may be solid tan or yellow (steatosis). • Intratumoral hemorrhage can give rise to a soft, necrotic, red -brown lesion. • Fibrotic foci with brown discoloration (old intratumoral hemorrhage). • Subcapsular hematoma. • Background liver is typically noncirrhotic.

HA Subtype Histopathologic features Immunohistochemistry HA-H Steatosis, lack of inflammation ↓ or – L-FABP

HA Subtype Histopathologic features Immunohistochemistry HA-H Steatosis, lack of inflammation ↓ or – L-FABP neoplastic cells; patchy CD 34, focal CK 7+ HA-B Pseudoacinar pattern, cytologic atypia, steatosis, and/or lack of inflammation ↑ nuclear β-catenin, strong diffuse glutamine synthetase + HA-I Pseudoportal tracts with thick walled arteries and lack of bile ducts or veins; inflammatory inf; ductular reaction; sinusoidal dilatation; and peliosis Diffuse, strong serum amyloid A and CRP +; CD 34 + around pseudoportal tracts; diffuse glutamine synthetase + if β-catenin gene mutation HA-U No distinctive morphology No specific protein expression L-FABP, liver-type fatty acid binding protein expression.

HA-H, steatosis HA-B, diffuse and strong + of glutamate synthetase HA-H, lack of immunoexpression

HA-H, steatosis HA-B, diffuse and strong + of glutamate synthetase HA-H, lack of immunoexpression of L-FABP (liver-type fatty acid binding protein) HA-I, cytoplasmic expression of CRP

PROGNOSIS AND TREATMENT • Frequency of malignant transformation: 4. 2% for all adenomas and

PROGNOSIS AND TREATMENT • Frequency of malignant transformation: 4. 2% for all adenomas and 4. 5% for resected adenomas. • Cessation of OCPs does not abort the risk of malignant transformation in women. • High-risk group of malignant transformation • history of androgenic or anabolic steroid intake • male patients • patients with glycogen storage disease.

PROGNOSIS AND TREATMENT • HA-B: more freq. associated with development of HCC. • Surveillance

PROGNOSIS AND TREATMENT • HA-B: more freq. associated with development of HCC. • Surveillance with imaging studies at regular intervals in tumors <5 cm. • Surgical resection recommended for HAs >5 cm, those with intratumoral hemorrhage and those that increase in size.

FOLLOW-UP • MRI abdomen 4/27/12 • Unchanged hepatic lesions. • No interval growth or

FOLLOW-UP • MRI abdomen 4/27/12 • Unchanged hepatic lesions. • No interval growth or hemorrhage. • MRI abdomen w/ + w/o Contrast , 05/29/2013 • Unchanged hepatic lesions. • No new liver lesions.

THANK YOU.

THANK YOU.