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Liver and intrahepatic bile ducts - tumor

Other malignancies

Epithelioid hemangioendothelioma


Reviewers: Deepali Jain, M.D. (see Reviewers page)
Revised: 9 January 2013, last major update February 2012
Copyright: (c) 2004-2013, PathologyOutlines.com, Inc.

General
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● Endothelial derived neoplasm with unpredictable clinical course, usually intermediate between hemangioma and angiosarcoma
● Often misdiagnosed

Clinical features
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● Mean age 47 years, but occurs at any age, 60% women
● No predisposing factors; occasional cases reported in vinyl chloride workers, women on oral contraceptives, hepatitis B/C patients, following hepatic trauma
● Symptoms: abdominal pain, weight loss, hepatic venous outflow obstruction; 40% are asymptomatic
● FNA not recommended, as even small biopsies can be misleading
● Indolent and slow growing; 5 year survival of 43%
● 50% have extrahepatic involvement at diagnosis, which does not preclude long survival
● Metastases to abdominal lymph nodes, lung, omentum or peritoneum, spleen
● Radiologic calcifications in 20%; peripheral nodules with capsular retraction by CT scan

Prognostic factors
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● High cellularity is unfavorable, but histology is otherwise not prognostic

Case reports
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● 35 year old woman with multiple FNH and cavernous hemangiomas (Arch Pathol Lab Med 1999;123:846)
● 83 year old man with incidental tumor (Arch Pathol Lab Med 2002;126:225)
● Tumor with t(1;3)(p36.3;q25) (Am J Surg Pathol 2001;25:684)

Treatment
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● Resection, liver transplantation

Gross description
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● Multiple (80%), tan-gray, firm, circumscribed and focally confluent nodules up to 12 cm with infiltrative borders
● May involve venous structures as intravascular proliferations or fibrothrombotic occlusions
● Remaining liver is unremarkable

Gross images
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Mottled tumor with cavernous hemangioma


Various images

Micro description
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● Zonal pattern; periphery shows sinusoidal proliferation with tufting of tumor cells within portal vein branches
● Midzone has sinusoidal obliteration with atrophic hepatocyte plates and increased myxochondroid and sclerotic stroma
● Perivenular stroma is paucicellular and often calcified
● Epithelioid and fibromyxoid tumor cells have focal intracytoplasmic vacuoles containing red blood cells embedded in fibromyxoid matrix
● Epithelioid cells are rounded with eosinophilic cytoplasm, mild to moderately atypical nuclei with prominent nucleoli and no /rare mitotic figures
● Extramedullary hematopoiesis present; may have myxoid areas; often has infiltrative margins, inflammatory infiltrate; adjacent liver usually normal

Micro images
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Various images


Microvascular channels and intracytoplasmic vacuoles


Intracytoplasmic vacuoles and projection into vein


Epithelioid and spindle cells in fibromyxoid stroma

Positive stains
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● Factor VIII related antigen and CD34 for vacuoles
● CD31, trichrome and elastic stains accentuate obliteration of hepatic venules and hepatic vein branches
● Occasionally NSE, smooth muscle actin (25%)

Negative stains
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● Usually AE1/AE3, CK7 and CK20 [but keratin may stain trapped hepatocytes and bile ductules], alpha fetoprotein, bile, CEA, HepPar1, mucin

Cytology images
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Various images

Molecular description
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● t(1;3)(p36.3;q25) has been reported

Electron microscopy description
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● Weibel-Palade bodies, intermediate filaments

Differential diagnosis
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Angiosarcoma: infiltrative, freely anastomosing vascular channels, more atypia
Leiomyosarcoma
Scirrhous cholangiocarcinoma
Sclerosed hemangioma: well circumscribed, no venous invasion, no atypia
Sclerotic hepatocellular carcinoma
● Signet ring adenocarcinoma

End of Liver and intrahepatic bile ducts - tumor > Other malignancies > Epithelioid hemangioendothelioma


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