Epithelioid Hemangioma of Bone and Soft Tissue: A Reappraisal of a Controversial Entity

Errani, Costantino; Zhang, Lei; Panicek, David M.; Healey, John H.; Antonescu, Cristina R.

doi:10.1007/s11999-011-2070-0

Cited by 92 publications

(91 citation statements)

References 2 publications

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“…44 In fact, the radiological appearance of epithelioid hemangioma is not specific, having significant overlap with malignant vascular lesions, such as EHE or angiosarcoma.…”

Section: Epithelioid Hemangiomamentioning

confidence: 99%

Malignant vascular tumors—an update

2014

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“…44 In fact, the radiological appearance of epithelioid hemangioma is not specific, having significant overlap with malignant vascular lesions, such as EHE or angiosarcoma.…”

Section: Epithelioid Hemangiomamentioning

confidence: 99%

Malignant vascular tumors—an update

2014

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“…Epithelioid hemangioma (EH) is an uncommon but distinctive vascular neoplasm displaying well-formed vascular channels lined by prominent epithelioid endothelial cells 1-3 . As suggested from the variety of its previous designations, such as intravenous atypical vascular proliferation 4 , angiolymphoid hyperplasia with eosinophilia (ALHE) 5, 6 , inflammatory angiomatous nodule 7 , and histiocytoid hemangioma 8 , the morphologic spectrum of EH exhibits a wide range of appearances, including intravascular growth, a heavy inflammatory infiltrate, and a cellular/solid proliferation.…”

Section: Introductionmentioning

confidence: 99%

Frequent FOS Gene Rearrangements in Epithelioid Hemangioma

Huang¹,

Zhang

Sung

et al. 2015

American Journal of Surgical Pathology

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153

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Epithelioid hemangioma (EH) is a unique benign vasoformative tumor composed of epithelioid endothelial cells. Although a small subset of EHs with atypical features harbor ZFP36-FOSB fusions, no additional genetic abnormalities have been found to date in the remaining cases. Based on a novel FOS-LMNA gene fusion identified by RNA sequencing in an index case of a skeletal EH with typical morphology, we sought to investigate the prevalence of FOS rearrangement in a large cohort of EHs. Thus 57 additional EH cases lacking FOSB rearrangements were studied for FOS gene abnormalities by FISH and results were correlated with morphologic appearance and clinical presentation. The EHs were subclassified as typical (n=25), cellular (n=21) and ALHE (angiolymphoid hyperplasia with eosinophilia)(n=12) variants. The ALHE was defined as an EH with a vascular ‘blow-out’ pattern associated with a variable degree of inflammation. There were 17 (29%) cases bearing FOS gene rearrangements among 58 cases tested, including 12 males and 5 females, with a mean age of 42 years. Most FOS-rearranged EHs occurred in the bone (10) and soft tissue (6), while only one case was cutaneous. The predominant anatomic site was the extremity (12), followed by trunk (3), head and neck (1), and penis (1). The incidence of FOS rearrangement was significantly higher in bone (59%, p = 0.006) and lower in head and neck (5%, p = 0.009). Twelve of the FOS rearranged cases were cellular EH (p = 0.001) associated with moderate mitotic activity (2-5/10 HPF) and milder inflammatory background. All 12 ALHE cases lacked FOS gene abnormalities, suggesting different pathogenesis. In conclusion, FOS rearrangement was present in a third of EHs across different locations and histologic variants; however, it was more prevalent in cellular EH and intra-osseous lesions, compared to those in skin, soft tissue and head and neck. This genetic abnormality can be useful in challenging cases, to distinguish cellular EHs from malignant epithelioid vascular tumors. These results also suggest that dysregulation of the FOS family of transcription factors through chromosomal translocation is as a key event in the tumorigenesis of EH except for the ALHE variant.

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“…The term epithelioid hemangioma was suggested as a replacement for ALHE by Enzinger and Weiss in 1988 [4] ''to reflect the belief that most were benign neoplasms and to clearly delimit these lesions from the more aggressive tumor, epithelioid hemangioendothelioma, a neoplastic process with metastatic potential'' [6]. Errani et al [5] recently reported that a chromosomal translocation, t(1;3)(p36;q25) identified in epithelioid hemangioendothelioma is not present in EH and thus can be used to differentiate these two entities. However, EH and epithelioid hemangioendothelioma are histologically distinct, so cytogenetic analysis is rarely, if ever, indicated for accurate diagnosis.…”

Section: Discussion and Treatmentmentioning

confidence: 99%