Epithelioid Fibrous Histiocytoma: A Concise Review

Felty, Cameron C; Linos, Konstantinos

doi:10.1097/dad.0000000000001272

Cited by 25 publications

(42 citation statements)

References 26 publications

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“…4 Epithelioid fibrous histiocytoma, although previously considered a morphologic variant of benign fibrous histiocytoma, was recently identified as a distinct entity. 5 although focal positivity for S-100 protein may be present. 5 Epithelioid leiomyoma has been well-documented in the gastrointestinal and female genital tract but is rarely reported in the skin.…”

Section: Discussionmentioning

confidence: 99%

“…5 although focal positivity for S-100 protein may be present. 5 Epithelioid leiomyoma has been well-documented in the gastrointestinal and female genital tract but is rarely reported in the skin. 6 Histologically, the tumor exhibits multinodular growth pat-…”

Section: Discussionmentioning

confidence: 99%

“…Epithelioid fibrous histiocytoma, although previously considered a morphologic variant of benign fibrous histiocytoma, was recently identified as a distinct entity . Histopathologically, it shows a well‐defined superficial dermal proliferation of round to angulated epithelioid cells with vesicular nucleus.…”

Section: Discussionmentioning

confidence: 99%

“…On immunohistochemistry, the tumor cells exhibit reactivity for factor XIIIa, epithelial membranous antigen, and vimentin. It can be differentiated from melanocytic schwannoma by absence of staining with HMB‐45 and Melan‐A, although focal positivity for S‐100 protein may be present …”

Section: Discussionmentioning

confidence: 99%

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Asymptomatic skin‐colored papule on the upper eyelid in a young man

2019

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Asymptomatic skin‐colored papule on the upper eyelid in a young man

2019

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“…From a histopathological point of view, the differential diagnosis of RH includes other histiocytoses as well as disorders with infiltrates made of epithelioid oncocytic cells or epithelioid macrophages including sarcoidosis, Spitz naevus, histiocytic sarcoma, epithelioid fibrous histiocytoma and giant cell tumours of bone . Immunophenotype together with architectural features, background, atypia and clinical data is generally enough to differentiate all of these disorders from RH (Table ).…”

Section: Histopathology and Molecular Biologymentioning

confidence: 99%

Reticulohistiocytoses: a revision of the full spectrum

Bonometti

Berti

Onlus³

2020

Acad Dermatol Venereol

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Reticulohistiocytoses (RH) are rare and clinically heterogeneous histiocytic disorders of dermatological interest. Three clinical entities with superimposable histopathological features are currently considered, namely solitary reticulohistiocytoma, diffuse/generalized reticulohistiocytosis and multicentric reticulohistiocytosis. Although in the last decade, RH studies have only minimally progressed, histiocytosis research has advanced considerably: the prognostic and therapeutic importance of the clinical subclassification of histiocytosis patients as well as of the detection of genetic alterations in the genes of the ERK pathway has been highlighted. According to these insights, we previously reported the presence of molecular alteration RH and described a subset of patients with disseminated multisystem involvement lacking arthritis. In the present review, we aim to update and revise the knowledge regarding RH. We first reviewed their histopathological, immunophenotypical and ultrastructural features, discussed their histopathological differential diagnosis with other conditions characterized by infiltrates made of oncocytic or epithelioid cells (with special regard to Destombes-Rosai-Dorfman disease) and finally summarized the molecular landscape of RH. We therefore tried to adjust the clinical subclassification of Langerhans cell histiocytosis to the clinical phenotypes of RH, outlining five clinically different groups of patients. Finally, we reconsidered the clinical workflow to the evaluation of RH patients, in light of the 5 different clinical groups and discussed the different therapeutic approaches and the possible role of target inhibitors.

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Molecular investigation of ALK‐rearranged epithelioid fibrous histiocytomas identifies CLTC as a novel fusion partner and evidence of fusion‐independent transcription activation

Georgantzoglou

Green

Winnick

et al. 2022

Genes Chromosomes & Cancer

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Epithelioid fibrous histiocytoma (EFH) is a rare cutaneous neoplasm, which is characterized by the presence of rearrangements involving the ALK gene. Although EFH was long considered a variant of fibrous histiocytoma, the identification of its unique genetic signature confirmed that it represents a distinct entity. The aim of the present study was to examine a cohort of ALK‐immunoreactive EFH cases to further characterize gene fusion partners. Next generation sequencing detected ALK fusions in 11 EFH cases identified in the pathology archives of two different institutions. The most common fusion partner was DCTN1 (N = 4) followed by CLTC (N = 2) and VCL (N = 2), while the remaining cases harbored fusions involving SPECC1L, PPFIBP1, and PRKAR1A. In one case no fusion was detected by NGS and FISH despite suitable sample quality. Notably, IHC demonstrated positive ALK expression and the level of aligned ALK reads was comparable to the fusion‐positive cases. To the best of our knowledge, this is the first report of CLTC as a fusion partner in EFH. The two CLTC rearranged cases in our cohort also represent the first two EFH cases in the literature that involve exon 19 of ALK, instead of exon 20. These findings underscore the remarkable plasticity of ALK as an oncogenic driver and further expand the list of its potential fusion partners in EFH. Lastly this is also the first report of ALK‐immunoreactive EFH with no underlying fusion suggesting a fusion independent transcription mechanism as seen in other tumors.

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Epithelioid Fibrous Histiocytoma: A Concise Review

Cited by 25 publications

References 26 publications

Asymptomatic skin‐colored papule on the upper eyelid in a young man

Asymptomatic skin‐colored papule on the upper eyelid in a young man

Reticulohistiocytoses: a revision of the full spectrum

Molecular investigation of ALK‐rearranged epithelioid fibrous histiocytomas identifies CLTC as a novel fusion partner and evidence of fusion‐independent transcription activation

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