Liver and intrahepatic bile ducts - tumor - Epithelioid hemangioendothelioma

Home Chapter Home Jobs Conferences Fellowships Books

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
=========================================================================

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

Clinical features
=========================================================================

● 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
=========================================================================

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

Case reports
=========================================================================

● 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
=========================================================================

● Resection, liver transplantation

Gross description
=========================================================================

● 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
=========================================================================

Mottled tumor with cavernous hemangioma

Various images

Micro description
=========================================================================

● 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
=========================================================================

Various images

Microvascular channels and intracytoplasmic vacuoles

Intracytoplasmic vacuoles and projection into vein

Epithelioid and spindle cells in fibromyxoid stroma

Positive stains
=========================================================================

● 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
=========================================================================

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

Cytology images
=========================================================================

Various images

Molecular description
=========================================================================

● t(1;3)(p36.3;q25) has been reported

Electron microscopy description
=========================================================================

● Weibel-Palade bodies, intermediate filaments

Differential diagnosis
=========================================================================

● 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

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).