Soft tissue
Vascular
Benign
Epithelioid hemangioma
Author: Borislav A. Alexiev, M.D.
Editorial Board Member: Farres Obeidin, M.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Last author update: 18 January 2022
Last staff update: 28 September 2023
Copyright: 2019-2024, PathologyOutlines.com, Inc.
PubMed Search: Epithelioid hemangioma vascular
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Etiology | Clinical features | Diagnosis | Radiology images | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Frozen section description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Molecular / cytogenetics description | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Borislav A. Epithelioid hemangioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissueepithelioidhemangioma.html. Accessed December 22nd, 2024.
Definition / general
- Benign vascular neoplasm with well formed, capillary sized vessels lined by plump, epithelioid (histiocytoid) endothelial cells with abundant eosinophilic or amphophilic cytoplasm (Br J Dermatol 1969;81:1, Am J Surg Pathol 2004;28:523, Am J Surg Pathol 2015;39:1313)
- Many lesions are rich in eosinophils, plasma cells, lymphocytes and mast cells
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-O: 9125/0 - epithelioid hemangioma
- ICD-11: 2E81.0Y & XH10T4 - neoplastic haemangioma of other specified site & epithelioid haemangioma
Epidemiology
- Wide age range (most common: 20 - 50 years) (J Cutan Pathol 2018;45:395)
- No gender predilection, although earlier studies report a female predominance (J Cutan Pathol 2018;45:395)
Sites
- Head and neck, especially the forehead, preauricular area and scalp (J Cutan Pathol 2018;45:395)
- Distal extremities, especially the digits
- Other sites include bone, oral cavity, penis, lymph node (Am J Surg Pathol 2004;28:523)
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
- 12 year old boy with osteolytic lesion of distal femoral epiphysis (Malays J Pathol 2014;36:63)
- 31 year old man presented with a mass on the right nasal root; 56 year old man presented with a slow growing lesion in the upper right eyelid (J Craniofac Surg 2018;29:e736)
- 35 year old woman patient presented with multiple consecutive dermal nodules in her left forearm (Korean J Pathol 2014;48:394)
- 65 year old woman with lytic lesion involving T7-8 vertebral bodies (Acta Neurol Belg 2015;115:843)
- 83 year old man with orbital mass (Acta Clin Croat 2015;54:92)
Treatment
- Complete surgical excision (Spine J 2013;13:e7)
- Reexcision for local recurrences
- Chemoembolization and microwave ablation is a feasible, safe and effective technique in the treatment of bone epithelioid hemangioma (Acta Orthop Traumatol Turc 2018;52:157)
Clinical images
Images hosted on other servers:
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. and @JMGardnerMD on Twitter
Vascular neoplasm with inflammation
Capillary sized vessels with inflammation
Epithelioid endothelial cells
ERG
Epithelioid hemangioma
Cytology description
- Cohesive epithelioid cells with moderate to abundant eosinophilic cytoplasm, ovoid nuclei and small nucleoli
- Occasional abortive vascular channels, eosinophils and lymphocytes (Acta Cytol 2003;47:275, Diagn Cytopathol 1998;18:227)
Positive stains
- FOSB (J Cutan Pathol 2018;45:395)
- CD31
- ERG
- CD34
- FLI1
- Factor VIII
- AE1 / AE3
- SMA in myopericytic layer around the vessels
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
- Kimura disease (Am J Surg Pathol 1987;11:758, Arch Pathol Lab Med 2007;131:650):
- Most common in middle aged Asian men
- Elevated serum immunoglobulin E (IgE) levels and peripheral blood eosinophilia are common
- Proliferation of postcapillary venules
- Necrosis of germinal centers
- Polykaryocytes (Warthin-Finkeldey type giant cells)
- No FOS rearrangements
- Epithelioid hemangioendothelioma (Histopathology 2015;67:699, Diagn Pathol 2014;9:131):
- Chains and cords of epithelioid cells distributed in a myxohyaline stroma
- Cells contain intracytoplasmic lumina
- No well formed vessels
- No abundant stromal eosinophils, plasma cells and lymphocytes
- WWTR1-CAMTA1 fusion
- Epithelioid angiosarcoma (Arch Pathol Lab Med 2011;135:268, Am J Surg Pathol 2011;35:60):
- Dissecting growth pattern
- Predominantly solid areas
- Coagulative necrosis
- Brisk mitotic activity
- Significant nuclear atypia
Board review style question #1
- Which of the following is true about epithelioid hemangioma?
- ~30% of cases metastasize
- Diagnosis always requires clinicopathological and radiological correlation
- Epithelioid hemangiomas harbor SERPINE1-FOSB fusions
- Epithelioid hemangiomas with atypical features harbor ZFP36-FOSB fusions
- Tumor is characterized by cords of epithelioid endothelial cells distributed in a myxohyaline stroma
Board review style answer #1
D. Epithelioid hemangiomas with atypical features harbor ZFP36-FOSB fusions
Comment Here
Reference: Epithelioid hemangioma
Comment Here
Reference: Epithelioid hemangioma
Board review style 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?
- Angiomatosis
- Epithelioid angiosarcoma
- Epithelioid hemangioendothelioma
- Epithelioid hemangioma
- Pseudomyogenic hemangioendothelioma
Board review style answer #2
