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Skin: Cutaneous benign fibrous histiocytomas

Written2001-06Roberta Vanni
Dip. Scienze e Tecnologie Biomediche, Sezione di Biologia e Genetica, Università di Cagliari, Cittadella Universitaria, 09142 Monserrato (CA), Italy

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ICD-Topo C440-C449 SKIN
ICD-Morpho 8832/0 Dermatofibroma (fibrous histiocytoma)
Atlas_Id 5092
Phylum Lung, Heart, Skin, Other::Cutaneous benign fibrous histiocytoma
WHO/OMS Classification Lung, Heart, Skin, Other


Note Cutaneous benign fibrohistiocytic (BFH) tumours are among the most common soft tissue lesions. Their biological nature, in particular whether they are neoplastic or reactive, has long been disputed.

Clinics and Pathology

Etiology unknown. Sometimes, it is associated with insect bites and vaccines.
Epidemiology It is among the most common soft tissue tumors of the skin. Its polymorphic histologic appearence is responsible for the array of different names by which it has been known in the literature in the past.
Clinics The cutaneous benign fibrous histiocytoma is a solitary, slowly growing, asymptomatic pigmented papule that usually makes its appearence during early or mid-adult life, with a slight predilection for females. It is mainly located on the extremities. The lesions are elevated or peduncolated with a diameter ranging from a few millimeters to a few centimeters. The overlying skin may assume a red or red-brown color. An excessive overdeposit of haemosiderin can be responsible of a black color. Clinical and histological diagnosis is straighfoward in most cases. However, difficulties in diagnosis may arise in cases when the clinical and histological features deviate from normal appearence.
Pathology It has a predominant dermal location. It is composed of a mixture of fibroblastic, myofibroblastic-like, and histiocytic cells, often arranged in a cartwheel or storiform pattern and accompained by varying numbers of inflammatory cells, foam cells and siderophages. When a single unusual histological feature predominates it may mask the typical features and uncertain diagnosis may arise. In addition to the common pattern, a number of variants are currently recognized some of which may mimic other benign or malignant lesions.They are: cellular benign fibrous histiocytoma (it mimics dermatofibrosarcoma protuberans and leiomyosarcoma), aneurysmal (angiomatoid) benign fibrous histiocytoma (it may resemble a melanocytic or vascular tumor or angiomatoid malignant fibrous histiocytoma), epithelioid benign fibrous histiocytoma (it can be confuse with Spitz nevus), atypical (pseudosarcomatous) benign fibrous histiocytoma (it can be misdiagnosed as a malignant tumor), benign fibrous histiocytoma 'ankle type' (it may mimic a xanthoma), palisading, and atrophic fibrous histiocytoma.
Evolution These lesions may regress spontaneously, sometime they recur: the cellular and aneurysmal variants may recur more often then the other subtypes.


Note Very limited information are available concerning the genetics or clonality of these lesions, most likely reflecting the fact that suitably prepared tissue is rarely collected from these common cutaneous lesions, which are generally excised in an outpatient setting.


Clonal chromosome changes are present in the 38% of the cases reported in the literature. No recurrent chromosome abnormalities have been observed. The clonal changes are more frequent in the cellular variant, and they are not characterised by chromosome abnormalities generally associated with other more aggressive fibrohistiocytic tumours with which they may be confused. Numerical changes as well as structural abnormalities, including ring chromosomes have been described.Although the aberrations did not correlate with the histological subtypes, the presence of clonal chromosome changes in a number of cases supports a neoplastic pathogenesis of BFH.


Is cutaneous benign fibrous histiocytoma (dermatofibroma) a reactive inflammatory process or a neoplasm?
Calonje E
Histopathology. 2000 ; 37 (3) : 278-280.
PMID 10971705
Aneurysmal benign fibrous histiocytoma: clinicopathological analysis of 40 cases of a tumour frequently misdiagnosed as a vascular neoplasm.
Calonje E, Fletcher CD
Histopathology. 1995 ; 26 (4) : 323-331.
PMID 7607620
Cellular benign fibrous histiocytoma. Clinicopathologic analysis of 74 cases of a distinctive variant of cutaneous fibrous histiocytoma with frequent recurrence.
Calonje E, Mentzel T, Fletcher CD
The American journal of surgical pathology. 1994 ; 18 (7) : 668-676.
PMID 8017561
Dermatofibroma is a clonal proliferative disease.
Chen TC, Kuo T, Chan HL
Journal of cutaneous pathology. 2000 ; 27 (1) : 36-39.
PMID 10660130
Benign fibrous tissue tumors.
Enzinger FM, Weiss SW
In Soft Tissue Tumors..
The benign fibrohistiocytic tumours of the skin.
Niemi KM
Acta dermato-venereologica. Supplementum. 1970 ; 50 (63) : 1-66.
PMID 4100564
Cytogenetic evidence of clonality in cutaneous benign fibrous histiocytomas: a report of the CHAMP study group.
Vanni R, Fletcher CD, Sciot R, Dal Cin P, De Wever I, Mandahl N, Mertens F, Mitelman F, Rosai J, Rydholm A, Tallini G, Van Den Berghe H, Willén H
Histopathology. 2000 ; 37 (3) : 212-217.
PMID 10971696
Cellular fibrous histiocytoma of the skin: evidence of a clonal process with different karyotype from dermatofibrosarcoma.
Vanni R, Marras S, Faa G, Licheri S, Daniele GM, Fletcher CD
Genes, chromosomes & cancer. 1997 ; 18 (4) : 314-317.
PMID 9087573


This paper should be referenced as such :
Vanni, R
Skin: Cutaneous benign fibrous histiocytomas
Atlas Genet Cytogenet Oncol Haematol. 2001;5(3):213-214.
Free journal version : [ pdf ]   [ DOI ]
On line version :

External links

arrayMap Topo ( C44) arrayMap ((UZH-SIB Zurich)   [auto + random 100 samples .. if exist ]   [tabulated segments]
Disease databaseSkin: Cutaneous benign fibrous histiocytomas
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