Liver & intrahepatic bile ducts
Benign / nonneoplastic
Hemangioma

Editorial Board Member: Catherine E. Hagen, M.D.
Deputy Editor-in-Chief: Raul S. Gonzalez, M.D.
Michael McCarthy, M.D.
Lizhi Zhang, M.D.

Topic Completed: 15 February 2021

Minor changes: 3 May 2021

Copyright: 2002-2021, PathologyOutlines.com, Inc.

PubMed Search: Hemangioma[TI] liver[TI] free full text[sb] pathology

See also: Soft tissue topic

Michael McCarthy, M.D.
Lizhi Zhang, M.D.
Page views in 2020: 7,432
Page views in 2021 to date: 8,857
Cite this page: McCarthy M, Zhang L. Hemangioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/livertumorhemangioma.html. Accessed July 24th, 2021.
Definition / general
  • Benign vascular tumors further classified based on characteristic morphologic findings
  • Predominately asymptomatic with no reported malignant transformation
Essential features
  • Hepatic hemangiomas are the most common benign tumor of the liver
  • Classification is based on characteristic histologic features; there should be no significant cytologic atypia or mitotic activity
  • Vast majority of hemangiomas are asymptomatic and require no intervention; larger lesions (> 5 cm) are more likely to be symptomatic and susceptible to complications such as hemorrhage, rupture and Kasabach-Merritt syndrome
  • No cases of malignant transformation have been documented
Terminology
  • Cavernous hemangioma
    • Most common subtype of hepatic hemangioma
  • Capillary hemangioma
    • Extremely rare
  • Anastomosing hemangioma
    • Rare, newly described variant
    • More frequently occurs in genitourinary tract
  • Sclerosing / sclerosed hemangioma
  • Hepatic small vessel neoplasm
    • Rare, with mild atypical features
    • Benign or low grade entity in adults
    • No cases of recurrences or metastasis reported
ICD coding
  • ICD-O: 9120/0 - hemangioma, NOS
  • ICD-11: 2E81.0Y - neoplastic hemangioma of other specified site
  • ICD-11: XH5AW4 - hemangioma, NOS
Epidemiology
Sites
Etiology
Clinical features
Diagnosis
  • Predominantly based on imaging studies
  • Core needle biopsy contraindicated due to risk of hemorrhage and low diagnostic yield (J Med Life 2015;8 Spec Issue:4)
Laboratory
  • Alkaline phosphatase (ALP) and gamma glutamyl transferase (GGT) usually within normal limits (Visc Med 2020;36:292)
  • Alpha fetoprotein (AFP), carcinogenic antigen 19-9 (CA 19-9) and carcinogenic antigen (CEA) within normal limits (J Med Life 2015;8 Spec Issue:4)
Radiology description
  • Multiple modalities available (J Med Life 2015;8 Spec Issue:4)
    • Overall stable findings on serial exam are a reliable indicator of benignity
    • Contrast enhancing ultrasonographic and technetium-99m scintigraphy are most specific
  • Ultrasound (US)
    • Conventional: well circumscribed homogenous nodule; hyperechoic in smaller lesions, mixed echogenicity in larger lesions
    • Contrast enhancing: more specific; peripheral nodular enhancement in arterial phase, centripetal filling in late phase
  • Computer tomography (CT)
    • Well circumscribed hypodense lesion
    • Contrast demonstrates peripheral nodular enhancement with progressive centripetal filling
  • Magnetic resonance imaging (MRI)
    • Well circumscribed homogenous lesion
    • Hypointense on T1, hyperintense with "cotton wool" appearance on T2
  • Technetium-99m scintigraphy
    • Perfusion / blood pool mismatch
    • Cold lesion in early phase, intensive activity in late phase
Radiology images

Contributed by Lizhi Zhang, M.D.
CT scan: arterial phase

CT scan: arterial phase

CT scan: venous phase

Prognostic factors
Case reports
Treatment
Gross description
  • Most often solitary lesions
  • Usually < 4 cm but range from a few millimeters to > 30 cm in size
    • If > 10 cm, referred to as giant hemangioma
  • Well circumscribed with red-brown, spongy / honeycombed cut surface
Gross images

Contributed by Lizhi Zhang, M.D.
Cavernous hemangioma

Cavernous hemangioma

Microscopic (histologic) description
  • Cavernous hemangioma
    • Circumscribed proliferation of variably sized, dilated and thin walled vessels lined by a single layer of flat endothelial cells
    • No cytologic atypia or mitosis
    • Vascular spaces separated by fibrous septa containing small vessels
    • Focal thrombi, calcification, hyalinization, extramedullary hematopoiesis and stromal edema may be present
    • May have irregular interface with surrounding hepatic parenchyma
  • Sclerosing / sclerosed hemangioma
    • Abundant fibrotic stroma that compresses and replaces lesional vessels
    • Only vague outline of residual vessels may be present; elastin or trichrome may aid in highlighting such vessels
  • Capillary hemangioma
    • Lobular proliferation of small, thin walled vessels lined by a single layer of bland endothelial cells
  • Anastomosing hemangioma
    • Overall well circumscribed; anastomosing, small, capillary-like vessels lined by endothelium with mild cytologic atypia and focal hobnailing
    • Thrombi are frequent
    • Hyaline globules and extramedullary hematopoiesis may be present
    • May resemble angiosarcoma but lacks infiltrative growth, severe cytologic atypia or mitotic activity
  • Hepatic small vessel neoplasm
    • Similar to anastomosing hemangioma but with infiltrative proliferation of small, thin walled vessels around portal tracts and into adjacent liver parenchyma
    • Lined by flattened to plumped or hobnailed endothelial cells
    • Extramedullary hematopoiesis may be present
    • Lack of multilayering, papillary architecture, significant cytologic atypia, mitotic activity and necrosis
    • Ki67 proliferation index most helpful tool in distinguishing from angiosarcoma (Hum Pathol 2016;54:143)
Microscopic (histologic) images

Contributed by Lizhi Zhang, M.D.
Cavernous hemangioma Cavernous hemangioma Cavernous hemangioma

Cavernous hemangioma

CD31 immunostain

CD31 immunostain

ERG immunostain

ERG immunostain

Sclerosing hemangioma

Sclerosing hemangioma


Sclerosing hemangioma

Sclerosing hemangioma

Capillary hemangioma

Capillary hemangioma

Anastomosing hemangioma Anastomosing hemangioma

Anastomosing hemangioma

Hepatic small vessel neoplasm Hepatic small vessel neoplasm

Hepatic small vessel neoplasm

Positive stains
  • Endothelial markers: CD31, CD34, ERG, FLI1, Factor VIII are strongly and uniformly positive
  • Elastin and trichrome useful in highlighting lesional vessels in sclerosing hemangioma
  • Ki67 proliferation index is most helpful tool in distinguishing hepatic small vessel neoplasm from angiosarcoma
    • Mean nuclear labeling 3.7% and 42.8% respectively
Negative stains
Molecular / cytogenetics description
Sample pathology report
  • Liver, segment IV, segmentectomy:
    • Cavernous hemangioma (3.5 cm in greatest dimension)
Differential diagnosis
Board review style question #1
What subtype of hepatic hemangioma is most common?

  1. Anastomosing
  2. Capillary
  3. Cavernous
  4. Hepatic small cell neoplasm
  5. Sclerosing / sclerosed
Board review style answer #1
C. Cavernous

Comment Here

Reference: Hemangioma
Board review style question #2

A 35 year old woman presents with an incidental 2.5 cm hepatic mass. A representative field from the resection specimen is shown above. The lesional cells are positive for FLI1 and CD34. Which stain would be most useful in distinguishing hepatic small vessel neoplasm from angiosarcoma?

  1. Cytokeratin
  2. ER / PR
  3. Glypican 3
  4. Ki67
  5. Podoplanin
Board review style answer #2
D. Ki67

Comment Here

Reference: Hemangioma
Back to top
Image 01 Image 02