Background

This rare tumor of the skin usually occurs on the extremities of adults. These tumors are benign.

OUTLINE

Epidemiology
Histopathological Features and Variants
Special Stains/Immunohistochemistry/Electron Microscopy
Differential Diagnosis
Prognosis and Treatment
Commonly Used Terms
Internet Links

EPIDEMIOLOGY	CHARACTERIZATION
INCIDENCE	Rare
AGE RANGE-MEDIAN	Adults
SEX (M:F)	May have female preference

HISTOLOGICAL TYPES	CHARACTERIZATION
GENERAL
Angiofibroblastoma of the skin: a report of seven cases in support of a distinctive entity. Diaz-Cascajo C, Schaefer D, Borghi S. Center for Dermatopathology, Freiburg, and Dermatology Praxis, Oehringen, Germany.	J Cutan Pathol 2002 Oct;29(9):534-9 Abstract quote BACKGROUND: Angiofibroblastoma of the skin is a recently described benign tumor with fibroblastic differentiation. The tumor is composed of stellate and spindle fibroblasts embedded in a fibromyxoid and fibrous matrix that contains numerous capillary-sized blood vessels. METHODS: All cases of fibromyxoid tumors of the skin from the files of two dermatopathologic centers and from consultation cases, together with clinical data and histopathologic specimens, were reviewed. Additionally, immunohistochemical studies were performed in all cases. RESULTS: Seven cases of angiofibroblastoma of the skin were found, including two cases previously reported. All cases showed similar histopathologic findings. The morphologic versatility of fibroblasts, however, explains some of the microscopic variations that were observed in three cases of this series. Immunohistochemical studies support the fibroblastic nature of the neoplasms. CONCLUSIONS: Our series provides data suggesting that angiofibroblastoma of the skin represents a distinctive entity, which should be considered in the differential diagnosis of other fibromyxoid tumors of the skin.

 

SPECIAL STAINS/IMMUNOPEROXIDASE/ OTHER	CHARACTERIZATION
SPECIAL STAINS
IMMUNOPEROXIDASE
Angiofibroblastoma of the skin: a histological, immunohistochemical and ultrastructural report of two cases of an undescribed fibrous tumour. Diaz-Cascajo C, Metze D. Center for Dermatopathology, Freiburg, Germany.	Histopathology 1999 Aug;35(2):109-13 Abstract quote AIMS: The aim of this report is to present two cases of a distinct mesenchymal tumour of the skin that does not fit into one of the established entities. METHODS AND RESULTS: All cases of fibromyxoid tumours from the files of two dermatopathology centres, together with clinical data and histopathological records, were reviewed. Two cases of a lesion composed of stellate and spindle-shaped cells with the phenotype of fibroblasts embedded in a fibromyxoid to dense fibrous stroma were identified. Because of the large number of capillary-sized blood vessels and their peculiar distribution within the stroma, the name angiofibroblastoma of the skin is proposed for this peculiar neoplasm. In both cases, the tumour appeared as an indolent and slowly growing nodule on the extremities of adults. Immunohistochemical and, in one case, ultrastructural studies were performed. CONCLUSIONS: The lack of recurrences during a follow-up period of 9 and 3 years, respectively, after complete excision together with the bland histopathological appearance supports the benign character of the tumour.

 

DIFFERENTIAL DIAGNOSIS	KEY DIFFERENTIATING FEATURES
FIBROMYXOID LESIONS	Giant cell fibroblastoma Myxoid neurofibroma Angiomyofibroblastoma Perineurioma

 

PROGNOSIS AND TREATMENT	CHARACTERIZATION
PROGNOSTIC FACTORS	All cases benign to date
TREATMENT	Simple excision

Weedon D. Weedon's Skin Pathology. Second Edition. Churchill Livingstone. 2002.
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Robbins Pathologic Basis of Disease. Sixth Edition. WB Saunders 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

Soft Tissue Tumors

Last Updated 10/28/2002

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