Soft tissue
Vascular: benign
Epithelioid hemangioma

Editor-in-Chief: Debra Zynger, M.D.
Borislav Alexiev, M.D.

Topic Completed: 10 September 2019

Revised: 10 September 2019

Copyright: 2019, PathologyOutlines.com, Inc.

PubMed Search: Epithelioid hemangioma [title] vascular

Borislav Alexiev, M.D.
Page views in 2019 to date: 157
Cite this page: Borislav A. Epithelioid hemangioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissueepithelioidhemangioma.html. Accessed September 20th, 2019.
Definition / general
Essential features
  • Numerous stromal eosinophils and lymphoid reaction with follicles in most cases
  • Most frequent sites are head and distal extremities
  • All cases show diffuse strong expression of vascular markers
  • FOS rearrangement in a third of epithelioid hemangiomas across different locations and histologic variants
Terminology
  • Angiolymphoid hyperplasia with eosinophilia
  • Nodular angioblastic hyperplasia with eosinophilia and lymphofolliculosis
  • Histiocytoid hemangioma
  • Inflammatory angiomatous nodule
  • Atypical or pseudopyogenic granuloma
ICD coding
  • ICD-0: 9125 / 0 - epithelioid hemangioma
Epidemiology
Sites
Etiology
  • The etiology of most epithelioid hemangiomas is unknown (Am J Surg Pathol 2004;28:523)
  • There has been considerable debate as to whether this is a reactive vascular proliferation or a benign vascular neoplasm, although most available data favor the latter (Am J Surg Pathol 2015;39:1313)
Clinical features
  • Most patients present with a slow growing, solitary subcutaneous or dermal nodule (J Cutan Pathol 2018;45:395)
  • Multifocal disease in the same anatomical region is encountered with some frequency
Diagnosis
  • Tissue sampling is the gold standard for a definitive diagnosis
Radiology images

Images hosted on other servers:

Right scapula mass

Prognostic factors
  • Local recurrence occurs in up to one third of patients (J Craniofac Surg 2018;29:e736)
  • The vast majority of recurrences are indolent
  • There are no reports of distant metastases
Case reports
Treatment
Clinical images

Images hosted on other servers:
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Erythematous nodule

Multiple papules

Gross description
  • Most have a nonspecific nodular appearance with pale pink to red-brown color (Am J Surg Pathol 2004;28:523)
  • Subcutaneous lesions may be confused with lymph nodes because of circumscription and a prominent lymphoid reaction
Frozen section description
  • Diagnosis may be difficult due to extensive inflammation
Microscopic (histologic) description
  • Lobular proliferation of small, capillary sized vessels lined by plump, epithelioid endothelial cells, often surrounding central vessel (Br J Dermatol 1969;81:1)
  • Endothelial cells with abundant eosinophilic or amphophilic cytoplasm that is sometimes vacuolated (Am J Surg Pathol 2004;28:523)
  • Cytoplasm may appear to project into lumen (hobnail or “tombstone” appearance)
  • Single, large nucleus with fine chromatin and central nucleolus
  • Rare spindle cell morphology
  • Mitotic figures are uncommon
  • No necrosis
  • No nuclear pleomorphism
  • Abundant stromal chronic inflammation (numerous eosinophils, lymphocytes, mast cells and plasma cells)
  • Lymphoid tissue may form reactive germinal centers, particularly at periphery of lesion
  • Dermal examples lack association with an artery, vessels have a more mature appearance and endothelial cells are less plump and more cobblestone-like in appearance
  • Epithelioid hemangioma with atypical features (Genes Chromosomes Cancer 2014;53:951)
    • Solid growth
    • Increased cellularity
    • Mild to moderate nuclear pleomorphism
    • Necrosis may be seen
    • No overt sarcomatous areas
Microscopic (histologic) images

Contributed by Borislav A. Alexiev, M.D.
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Vascular neoplasm with inflammation

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Capillary sized vessels with inflammation

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Epithelioid endothelial cells

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ERG

Cytology description
Positive stains
Negative stains
Molecular / cytogenetics description
  • FOS rearrangement in a third of epithelioid hemangioma across different locations and histologic variants (Am J Surg Pathol 2015;39:1313)
  • A novel FOS-LMNA fusion transcript in a typical variant of epithelioid hemangioma (Am J Surg Pathol 2015;39:1313)
  • Epithelioid hemangiomas with atypical features harbor ZFP36-FOSB fusions (recommended diagnostic terminology for a pathology report for these cases is "epithelioid hemangiomas with atypical features") (Genes Chromosomes Cancer 2014;53:951)
Sample pathology report
  • Left upper lip mass, excision:
    • Epithelioid hemangioma (see comment)
    • Comment: There is a dermal and subcutaneous proliferation of small, capillary sized vessels lined by epithelioid endothelial cells. The process is well demarcated and intimately associated with a small artery. A brisk stromal eosinophilic and lymphocytic inflammatory infiltrate is present. Immunohistochemically the epithelioid endothelial cells have strong expression of ERG and CD31 while are negative for HHV8. This constellation of morphological and immunohistochemical features strongly supports the diagnosis of epithelioid hemangioma. It is a benign vascular neoplasm prone to local recurrence unless completely excised. No distant metastatic spread has been reported.
Differential diagnosis
Board review question #1
    Which of the following is true about epithelioid hemangioma?

  1. ~30% of cases metastasize
  2. Diagnosis always requires clinicopathological and radiological correlation
  3. Epithelioid hemangiomas harbor SERPINE1-FOSB fusions
  4. Epithelioid hemangiomas with atypical features harbor ZFP36-FOSB fusions
  5. Tumor is characterized by cords of epithelioid endothelial cells distributed in a myxohyaline stroma
Board review answer #1
D. Epithelioid hemangiomas with atypical features harbor ZFP36-FOSB fusions

Comment Here

Reference: Epithelioid hemangioma
Board review question #2
    A 65 year old man presented with a left upper lip mass. Hematoxylin-eosin stains demonstrated proliferation of small, capillary sized vessels lined by plump, epithelioid endothelial cells. Numerous stromal eosinophils, plasma cells and lymphocytes were also seen. Immunohistochemical stains for CD31 and ERG were positive in epithelioid endothelial cells. SMA stain highlighted the preserved myopericytic layer around vessels. Which of the following is most likely the correct diagnosis?

    Missing Image

  1. Angiomatosis
  2. Epithelioid angiosarcoma
  3. Epithelioid hemangioendothelioma
  4. Epithelioid hemangioma
  5. Pseudomyogenic hemangioendothelioma
Board review answer #2
D. Epithelioid hemangioma

Comment Here

Reference: Epithelioid hemangioma
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