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Skin Fibrous Histiocytoma, Epithelioid Variant

MedGen UID:
353575
•Concept ID:
C1707544
•
Neoplastic Process
Synonyms: Cutaneous Epithelioid Fibrous Histiocytoma; Cutaneous Fibrous Histiocytoma, Epithelioid Variant; Epithelioid Fibrous Histiocytoma; Epithelioid fibrous histiocytoma
SNOMED CT: Epithelioid fibrous histiocytoma (816970007)

Definition

A morphologic variant of fibrous histiocytoma of the skin. It is characterized by the presence of epithelioid fibrohistiocytic cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSkin Fibrous Histiocytoma, Epithelioid Variant

Professional guidelines

PubMed

Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):178-86. Epub 2015 Mar 26 doi: 10.1177/0394632015575977. PMID: 25816393

Recent clinical studies

Etiology

Cutaneous soft tissue tumors: how do we make sense of fibrous and "fibrohistiocytic" tumors with confusing names and similar appearances?
Hornick JL
Mod Pathol 2020 Jan;33(Suppl 1):56-65. Epub 2019 Oct 25 doi: 10.1038/s41379-019-0388-4. PMID: 31653978
Epithelioid fibrous histiocytoma: molecular characterization of ALK fusion partners in 23 cases.
Dickson BC, Swanson D, Charames GS, Fletcher CD, Hornick JL
Mod Pathol 2018 May;31(5):753-762. Epub 2018 Jan 12 doi: 10.1038/modpathol.2017.191. PMID: 29327718
Variants of dermatofibroma--a histopathological study.
Alves JV, Matos DM, Barreiros HF, Bártolo EA
An Bras Dermatol 2014 May-Jun;89(3):472-7. doi: 10.1590/abd1806-4841.20142629. PMID: 24937822Free PMC Article
Pleomorphic dermal neoplasms: a review.
Brenn T
Adv Anat Pathol 2014 Mar;21(2):108-30. doi: 10.1097/PAP.0000000000000009. PMID: 24508694
Cutaneous fibrohistiocytic tumours - an update.
Luzar B, Calonje E
Histopathology 2010 Jan;56(1):148-65. doi: 10.1111/j.1365-2559.2009.03447.x. PMID: 20055912

Diagnosis

Spindle cell variant of epithelioid cell histiocytoma (spindle cell histiocytoma) with ALK gene fusions: Cases series and review of the literature.
Kazlouskaya V, Ho J, Jedrych J, Karunamurthy A
J Cutan Pathol 2021 Jul;48(7):837-841. Epub 2020 Dec 11 doi: 10.1111/cup.13923. PMID: 33217002
Fibrous histiocytoma/dermatofibroma in children: the same as adults?
Berklite L, Ranganathan S, John I, Picarsic J, Santoro L, Alaggio R
Hum Pathol 2020 May;99:107-115. Epub 2020 Apr 1 doi: 10.1016/j.humpath.2020.03.012. PMID: 32246988
Epithelioid Fibrous Histiocytoma: A Concise Review.
Felty CC, Linos K
Am J Dermatopathol 2019 Dec;41(12):879-883. doi: 10.1097/DAD.0000000000001272. PMID: 30289773
ALK rearrangement and overexpression in epithelioid fibrous histiocytoma.
Doyle LA, Mariño-Enriquez A, Fletcher CD, Hornick JL
Mod Pathol 2015 Jul;28(7):904-12. Epub 2015 Apr 10 doi: 10.1038/modpathol.2015.49. PMID: 25857825
Pleomorphic dermal neoplasms: a review.
Brenn T
Adv Anat Pathol 2014 Mar;21(2):108-30. doi: 10.1097/PAP.0000000000000009. PMID: 24508694

Prognosis

Fibrous histiocytoma/dermatofibroma in children: the same as adults?
Berklite L, Ranganathan S, John I, Picarsic J, Santoro L, Alaggio R
Hum Pathol 2020 May;99:107-115. Epub 2020 Apr 1 doi: 10.1016/j.humpath.2020.03.012. PMID: 32246988
Cutaneous soft tissue tumors: how do we make sense of fibrous and "fibrohistiocytic" tumors with confusing names and similar appearances?
Hornick JL
Mod Pathol 2020 Jan;33(Suppl 1):56-65. Epub 2019 Oct 25 doi: 10.1038/s41379-019-0388-4. PMID: 31653978
Epithelioid Fibrous Histiocytoma: A Concise Review.
Felty CC, Linos K
Am J Dermatopathol 2019 Dec;41(12):879-883. doi: 10.1097/DAD.0000000000001272. PMID: 30289773
Variants of dermatofibroma--a histopathological study.
Alves JV, Matos DM, Barreiros HF, Bártolo EA
An Bras Dermatol 2014 May-Jun;89(3):472-7. doi: 10.1590/abd1806-4841.20142629. PMID: 24937822Free PMC Article
Pleomorphic dermal neoplasms: a review.
Brenn T
Adv Anat Pathol 2014 Mar;21(2):108-30. doi: 10.1097/PAP.0000000000000009. PMID: 24508694

Clinical prediction guides

Epithelioid Fibrous Histiocytoma With Chondroblastoma-Like Features: A Report of a Rare Entity and Discussion of Related Diagnostic Challenges.
Wright GR, Archibald CW, Fontaine D, Dakin-Hache K, Walsh NM
Am J Dermatopathol 2022 Jan 1;44(1):e11-e15. doi: 10.1097/DAD.0000000000001986. PMID: 34086645
Cutaneous soft tissue tumors: how do we make sense of fibrous and "fibrohistiocytic" tumors with confusing names and similar appearances?
Hornick JL
Mod Pathol 2020 Jan;33(Suppl 1):56-65. Epub 2019 Oct 25 doi: 10.1038/s41379-019-0388-4. PMID: 31653978
Epithelioid Fibrous Histiocytoma: A Concise Review.
Felty CC, Linos K
Am J Dermatopathol 2019 Dec;41(12):879-883. doi: 10.1097/DAD.0000000000001272. PMID: 30289773
Pleomorphic dermal neoplasms: a review.
Brenn T
Adv Anat Pathol 2014 Mar;21(2):108-30. doi: 10.1097/PAP.0000000000000009. PMID: 24508694
Atypical fibrous histiocytoma arising in the perianal area: a case report and review of the literature.
Wang YT, Smart CN
Am J Dermatopathol 2014 Feb;36(2):171-3. doi: 10.1097/DAD.0b013e31829bd682. PMID: 24061404

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