Hepatic Angiosarcoma

Published on 19/07/2015 by admin

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Last modified 22/04/2025

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 Multifocal hypervascular masses in liver ± spleen

– May have peripheral and delayed enhancement that simulate hemangiomas
image Single/multiple hepatic masses, variable necrosis
image Diffuse infiltration of liver; micronodular
• Often metastatic to spleen, nodes, lungs, bones

TOP DIFFERENTIAL DIAGNOSES

• Hepatic cavernous hemangioma

image Centripetal nodular enhancement that approximates density of blood on all phases
image More homogeneously intense on T2WI than angiosarcoma
• Hepatic metastases and lymphoma

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

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

PATHOLOGY

• Etiology

image Environmental carcinogens and drugs

– Polyvinyl chloride, arsenicals, steroids

CLINICAL ISSUES

• Presentation

image Rapid and early metastatic spread

– Spleen, lung, bone marrow, nodes, peritoneum
• Prognosis

image Poor; death within 1 year
image
(Left) Cross section from a partial hepatectomy for angiosarcoma shows numerous cystic, blood-filled spaces. (Courtesy C. Trower, PA [ASCP] and A. Folpe, MD.)

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

image
(Right) Axial CECT in the same patient shows an additional lesion with similar enhancement and morphology in the spleen image. 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

image Heterogeneous, hypervascular, multifocal malignancy
• Key concepts

image Highly aggressive malignancy with several morphological types

Radiographic Findings

• Radiography

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

CT Findings

• 3 patterns

image Multifocal hypervascular masses in liver ± spleen, other organs

– May have peripheral and delayed enhancement that simulates hemangiomas
image Single or multiple hepatic masses with variable necrosis
image 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

image Moderately hypervascular tumor, diffuse puddling of contrast material that persists into venous phase
image Fed by large peripheral vessels and centripetal flow
image If ruptured: Demonstrate bleeding/hemoperitoneum

Nuclear Medicine Findings

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

image Nonspecific, not recommended
• Gallium scan: Increased gallium uptake

Imaging Recommendations

• Best imaging tool

image 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

image Environmental carcinogens

– Polyvinyl chloride, arsenicals, Thorotrast
image Drugs: Cyclophosphamide, anabolic steroids

– Diethylstilbestrol, oral contraceptives
image Post radiation (median latency: 74 months)
• Associated abnormalities

image 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

image Weakness, weight loss, abdominal pain, hepatomegaly in older patients
• Clinical profile

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

– Portal vein invasion/hemorrhagic ascites
image Lab data: Elevation of serum neuron-specific enolase; no elevation of α-fetoprotein

– Mild ↑ transaminases, bilirubin, alkaline phosphatase

Demographics

• Age

image Any age

– Peak incidence in 6th-7th decades
• Gender

image M:F = 4:1
• Epidemiology

image Most common mesenchymal tumor of liver
image Up to 2% of all primary malignant liver tumors
image 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
image
(Left) Axial T1WI MR in a 40-year-old woman with palpable abdominal mass shows a low signal intensity mass image involving both the left and right lobes. The focal areas of high signal image represent vascular spaces with slow flow.

image
(Right) Axial T2WI MR in the same patient shows that the mass image is of heterogeneous high signal intensity.
image
(Left) Axial CECT in a 70-year-old woman with malaise and weight loss shows 1 of several multiple hepatic masses image with heterogeneous enhancement.

image
(Right) Axial CECT in the same patient shows that some of the masses have a suggestion of nodular peripheral enhancement image, whereas other lesions appear as nonspecific, heterogeneous, hypodense masses image typically seen with metastases.
image
(Left) Axial CECT in the same patient shows that some tumors are either too small to characterize or appear as heterogeneous solid masses image.

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

image
(Right) Axial CECT in the same patient shows more of the splenic tumor image and subtle, small masses in the liver image.
image
(Left) Axial CECT in the same patient shows multifocal hypervascular tumors in the spleen image, liver image, and right kidney image.

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

image
(Right) Coronal CECT in the same patient shows additional tumors seen in liver image, spleen image, and kidney image. 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.

SELECTED REFERENCES

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2. Orlando, G, et al. Hepatic hemangiosarcoma: an absolute contraindication to liver transplantation—the European Liver Transplant Registry experience. Transplantation. 2013; 95(6):872–877.

3. Chung, EM, et al. From the archives of the AFIP: Pediatric liver masses: radiologic-pathologic correlation. Part 2. Malignant tumors. Radiographics. 2011; 31(2):483–507.

4. Geramizadeh, B, et al. Hepatic angiosarcoma of childhood: a case report and review of literature. J Pediatr Surg. 2011; 46(1):e9–11.

5. Schwartz, J, et al. Education and Imaging. Hepatobiliary and pancreatic: angiosarcoma of the liver. J Gastroenterol Hepatol. 2010; 25(5):1016.

Qiu, LL, et al. Sarcomas of abdominal organs: computed tomography and magnetic resonance imaging findings. Semin Ultrasound CT MR. 2011; 32(5):405–421.

Zhou, YM, et al. Results of hepatic resection for primary hepatic angiosarcoma in adults. Med Sci Monit. 2010; 16(2):CR61–CR66.

Deyrup, AT, et al. Angiosarcomas arising in the viscera and soft tissue of children and young adults: a clinicopathologic study of 15 cases. Am J Surg Pathol. 2009; 33(2):264–269.

Maeda, T, et al. Primary hepatic angiosarcoma on coregistered FDG PET and CT images. AJR Am J Roentgenol. 2007; 188(6):1615–1617.

Kitami, M, et al. Diffuse hepatic angiosarcoma with a portal venous supply mimicking hemangiomatosis. J Comput Assist Tomogr. 2003; 27(4):626–629.

Koyama, T, et al. Primary hepatic angiosarcoma: findings at CT and MR imaging. Radiology. 2002; 222(3):667–673.

Peterson, MS, et al. Hepatic angiosarcoma: findings on multiphasic contrast-enhanced helical CT do not mimic hepatic hemangioma. AJR Am J Roentgenol. 2000; 175(1):165–170.

White, PG, et al. The computed tomographic appearances of angiosarcoma of the liver. Clin Radiol. 1993; 48(5):321–325.