Hepatic Angiosarcoma

Published on 19/07/2015 by admin

Filed under Radiology

Last modified 22/04/2025

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Multifocal hypervascular masses in liver ± spleen

– May have peripheral and delayed enhancement that simulate hemangiomas

Single/multiple hepatic masses, variable necrosis

Diffuse infiltration of liver; micronodular

• Often metastatic to spleen, nodes, lungs, bones

TOP DIFFERENTIAL DIAGNOSES

• Hepatic cavernous hemangioma

Centripetal nodular enhancement that approximates density of blood on all phases

More homogeneously intense on T2WI than angiosarcoma

• Hepatic metastases and lymphoma

Hypervascular metastases: Hyperdense in late arterial phase; usually hypo- or isodense on portal venous and delayed phase

• Hepatocellular carcinoma

Heterogeneous hypervascular mass(es), vascular invasion, cirrhotic liver

PATHOLOGY

• Etiology

Environmental carcinogens and drugs

– Polyvinyl chloride, arsenicals, steroids

CLINICAL ISSUES

• Presentation

Rapid and early metastatic spread

– Spleen, lung, bone marrow, nodes, peritoneum

• Prognosis

Poor; death within 1 year

(Left) Cross section from a partial hepatectomy for angiosarcoma shows numerous cystic, blood-filled spaces. (Courtesy C. Trower, PA [ASCP] and A. Folpe, MD.)

(Right) Markedly atypical neoplastic endothelial cells line vascular spaces. Note that the normal hepatic architecture has been destroyed. (Courtesy L. Lamps, MD.)

(Left) Axial CECT shows dozens of vascular masses in the liver (and spleen) that have marked but irregular enhancement. Some portions of some lesions simulate the nodular peripheral pattern of enhancement seen with hemangiomas, but the lesions do not meet other criteria for benign lesions.

(Right) Axial CECT in the same patient shows an additional lesion with similar enhancement and morphology in the spleen . Biopsy specimen of a liver lesion confirmed the diagnosis of angiosarcoma.

(Right) Axial CECT in the same patient shows an additional lesion with similar enhancement and morphology in the spleen . Biopsy specimen of a liver lesion confirmed the diagnosis of angiosarcoma.

TERMINOLOGY

Abbreviations

• Angiosarcoma (AGS)

Synonyms

• Hemangioendothelial sarcoma, hemangiosarcoma, Kupffer cell sarcoma

IMAGING

General Features

• Best diagnostic clue

Heterogeneous, hypervascular, multifocal malignancy

• Key concepts

Highly aggressive malignancy with several morphological types

Radiographic Findings

• Radiography

If Thorotrast exposure: Localized areas of ↑ (metallic) density in patchy or circumferential pattern

CT Findings

• 3 patterns

Multifocal hypervascular masses in liver ± spleen, other organs

– May have peripheral and delayed enhancement that simulates hemangiomas

Single or multiple hepatic masses with variable necrosis

Diffuse infiltration of liver; micronodular

• Often metastatic to nodes, lungs, bones

MR Findings

• Hemodynamics and morphology similar to those on CT

• Bright on T2WI, usually less than for hemangioma

Angiographic Findings

• Conventional

Moderately hypervascular tumor, diffuse puddling of contrast material that persists into venous phase

Fed by large peripheral vessels and centripetal flow

If ruptured: Demonstrate bleeding/hemoperitoneum

Nuclear Medicine Findings

• Tagged red blood cell scan: Early as well as late persistent uptake

Nonspecific, not recommended

• Gallium scan: Increased gallium uptake

Imaging Recommendations

• Best imaging tool

Triphasic helical CT or MR

DIFFERENTIAL DIAGNOSIS

Hepatic Cavernous Hemangioma

• Centripetal nodular enhancement that approximates density of blood on all phases of enhancement

• More homogeneously intense on T2WI than AGS

• Hemangiomas are rarely as numerous as with AGS

Hepatic Metastases and Lymphoma

• Hypervascular metastases: Hyperdense in late arterial phase; usually hypo- or isodense on portal venous and delayed phase

Hepatocellular Carcinoma

• Heterogeneous hypervascular mass(es) with washout ± capsule

• Usually occurs in setting of known cirrhosis

PATHOLOGY

General Features

• Etiology

Environmental carcinogens

– Polyvinyl chloride, arsenicals, Thorotrast

Drugs: Cyclophosphamide, anabolic steroids

– Diethylstilbestrol, oral contraceptives

Post radiation (median latency: 74 months)

• Associated abnormalities

Neurofibromatosis type 1

Gross Pathologic & Surgical Features

• Nonencapsulated mass(es) containing large cystic areas filled with bloody debris and necrosis

Microscopic Features

• Malignant endothelial cells lining vascular channels

• Fibrosis and hemosiderin in solid portions of tumor

CLINICAL ISSUES

Presentation

• Most common signs/symptoms

Weakness, weight loss, abdominal pain, hepatomegaly in older patients

• Clinical profile

Rapid and early metastatic spread: Spleen (16%), lung, bone marrow, porta hepatis nodes, kidneys, peritoneum

– Portal vein invasion/hemorrhagic ascites

Lab data: Elevation of serum neuron-specific enolase; no elevation of α-fetoprotein

– Mild ↑ transaminases, bilirubin, alkaline phosphatase

Demographics

• Age

Any age

– Peak incidence in 6th-7th decades

• Gender

M:F = 4:1

• Epidemiology

Most common mesenchymal tumor of liver

Up to 2% of all primary malignant liver tumors

30x less common than hepatocellular carcinoma

Natural History & Prognosis

• Prognosis is poor; death within 1 year

Treatment

• Surgical resection: Tumor confined to 1 lobe of liver

• Systemic or hepatic arterial chemotherapy: Antiangiogenic therapy

DIAGNOSTIC CHECKLIST

Consider

• Diagnosis is rarely established by imaging alone

Image Interpretation Pearls

• Consider in any aggressive, multifocal, hypervascular tumor with variable morphology

(Left) Axial T1WI MR in a 40-year-old woman with palpable abdominal mass shows a low signal intensity mass involving both the left and right lobes. The focal areas of high signal represent vascular spaces with slow flow.

(Left) Axial T1WI MR in a 40-year-old woman with palpable abdominal mass shows a low signal intensity mass involving both the left and right lobes. The focal areas of high signal represent vascular spaces with slow flow.

(Right) Axial T2WI MR in the same patient shows that the mass is of heterogeneous high signal intensity.

(Right) Axial T2WI MR in the same patient shows that the mass is of heterogeneous high signal intensity.

(Left) Axial CECT in a 70-year-old woman with malaise and weight loss shows 1 of several multiple hepatic masses with heterogeneous enhancement.

(Left) Axial CECT in a 70-year-old woman with malaise and weight loss shows 1 of several multiple hepatic masses with heterogeneous enhancement.

(Right) Axial CECT in the same patient shows that some of the masses have a suggestion of nodular peripheral enhancement , whereas other lesions appear as nonspecific, heterogeneous, hypodense masses typically seen with metastases.

(Right) Axial CECT in the same patient shows that some of the masses have a suggestion of nodular peripheral enhancement , whereas other lesions appear as nonspecific, heterogeneous, hypodense masses typically seen with metastases.

(Left) Axial CECT in the same patient shows that some tumors are either too small to characterize or appear as heterogeneous solid masses .

(Left) Axial CECT in the same patient shows that some tumors are either too small to characterize or appear as heterogeneous solid masses .

(Right) Axial CECT in the same patient shows that other metastases have a complex cystic appearance , illustrating the spectrum of morphologic patterns seen with angiosarcoma.

(Right) Axial CECT in the same patient shows that other metastases have a complex cystic appearance , illustrating the spectrum of morphologic patterns seen with angiosarcoma.

(Left) Axial CECT in a 69-year-old man with abdominal pain and weight loss shows heterogeneous, hypervascular malignancy in multiple sites, including the spleen and an anterior rib .

(Left) Axial CECT in a 69-year-old man with abdominal pain and weight loss shows heterogeneous, hypervascular malignancy in multiple sites, including the spleen and an anterior rib .

(Right) Axial CECT in the same patient shows more of the splenic tumor and subtle, small masses in the liver .

(Right) Axial CECT in the same patient shows more of the splenic tumor and subtle, small masses in the liver .

(Left) Axial CECT in the same patient shows multifocal hypervascular tumors in the spleen , liver , and right kidney .

(Left) Axial CECT in the same patient shows multifocal hypervascular tumors in the spleen , liver , and right kidney .

(Right) Axial CECT in the same patient shows more of the multifocal malignancy in the spleen and kidney .

(Right) Axial CECT in the same patient shows more of the multifocal malignancy in the spleen and kidney .

(Left) Coronal reformatted CECT in the same patient shows multifocal malignancy within the liver , spleen , and kidney .

(Left) Coronal reformatted CECT in the same patient shows multifocal malignancy within the liver , spleen , and kidney .

(Right) Coronal CECT in the same patient shows additional tumors seen in liver , spleen , and kidney . The tumors do not fulfill the strict imaging criteria for hemangioma, including nodular peripheral enhancement that is isodense to blood vessels. Angiosarcoma is a very rare and aggressive tumor that is usually fatal within 1 year of diagnosis.

(Right) Coronal CECT in the same patient shows additional tumors seen in liver , spleen , and kidney . The tumors do not fulfill the strict imaging criteria for hemangioma, including nodular peripheral enhancement that is isodense to blood vessels. Angiosarcoma is a very rare and aggressive tumor that is usually fatal within 1 year of diagnosis.

Diagnostic Imaging_ Gastrointes - Michael P Federle