Cellular angiofibroma is a recently described histologically distinctive benign mesenchymal neoplasm composed of two main components, the cellular spindle cell component and prominent stromal blood vessels.

Local excision with negative margins appears to be adequate treatment for these lesions.

Age:  The tumour occurs in adult life (40- 60yrs).

Site:  Most common sites are the vulvovaginal region and the inguinoscrotal region. Cases in vulva are often mistaken for a Bartholin gland cyst.

Similar lesions in males have sometimes been called angiomyofibroblastoma-like tumour.

This tumour rarely occurs in retroperitoneum, perineum  and subcutaneous tissues of the chest .

Macroscopic appearances:  Soft or rubbery tumour with a greyish- white cut surface. Lesions in the vulva are characterized by its generally small size (less than 30mm) and usually well circumscribed margin. Examples in men tend to be larger and less clearly marginated.

Histopathology:

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A well circumscribed lesion but is usually not encapsulated.  Focal extension into soft tissue can be seen.

This is a cellular tumour and is composed of small spindle cells arranged in short fascicles together with wispy collagen bundles and relatively abundant small- or medium-sized rounded vessels. Many blood vessels with thick hyalinized walls may be present.

The spindle cells have bland oval to fusiform nuclei, pale eosinophilic cytoplasm and indistinct margins.

There may be prominent mitotic activity (up to 11 per 10 HPF). Abnormal mitoses are absent.  Usually there is no cellular atypia or necrosis. Focal areas of marked cellular atypia reminiscent of symplastic change within a uterine leiomyoma has been reported in one case.

Lobules of adipocytes may be identified at the periphery.

Other features include presence of stromal mast cells and occasional small collections of mature lymphocytes.

Immunohistochemistry:  Tumour cells express  vimentin, CD99, and both estrogen and progesterone receptors. The cells sometimes show positivty for CD34. The cells are negative for S100 protein, smooth muscle actin, desmin, epithelial membrane antigen and cytokeratin.

Differential diagnosis: 

Aggressive angiomyxoma (deeply located, less cellular, abundant myxoid matrix, more infiltrative) ;  

Angiomyofibroblastoma (less cellular, medium sized thickened hyalinized vessels are not present, composed of rather rounded myoid tumour cells which stain positively for desmin ) ;

Spindle cell lipoma (more common in male patients , usually located the neck and upper back , contains ropey refractile collagen bundles , lacks numerous blood vessels and hyalinized blood vessels, tumour cels are consistently positive for CD34)

Perineurioma (arranged in a whorled pattern, lacks thick-walled vessels and is positive for EMA) ;

Leiomyoma ( Piloleiomyoma ; Angioleiomyoma ; Lipoleiomyoma ; Genital Leiomyoma ) ; (features of smooth muscle differentiation- intersecting fascicles of spindle cells with blunt ended nuclei and eosinophilic cytoplasm, stain positively for myogenic markers)

Leiomyosarcoma (Cellular angiofibroma may be mistaken for sarcoma because of brisk mitotic activity) ;  

Solitary fibrous tumour (Hemangiopericytoma-like vascular pattern,  stromal keloidal- type hyalinization);

Fibroepithelial stromal polyp ;  Glomangiopericytoma ;  Mammary-type myofibroblastoma.

Abstracts: Mesenchymal tumors and tumor-like lesions of the female genital tract: a selective review with emphasis on recently described entities.Int J Gynecol Pathol. 2001;20(2):105-27. Vulvovaginal soft tissue tumours: update and review.Histopathology. 2000;36(2):97-108. A review and update of morphologically bland vulvovaginal mesenchymal lesions. Int J Gynecol Pathol. 2005;24(1):26-38. Cellular Angiofibroma: Clinicopathologic and Immunohistochemical Analysis of 51 Cases.Am J Surg Pathol. 2004 Nov;28(11):1426-1435. Cellular angiofibroma of the vulva: report of a case.Eur J Gynaecol Oncol. 2004;25(2):242-4. Cellular angiofibroma and related fibromatous lesions of the vulva: report of a series of cases with a morphological spectrum wider than previously described.Histopathology. 2004;45(4):360-8. Cellular angiofibroma of the vulva: a clinicopathological study of two cases with documentation of some unusual features and review of the literature.J Cutan Pathol. 2003;30(6):405-11. Angiomyofibroblastoma-like tumor (lipomatous variant) of the inguinal region of a male patient.Pathol Int. 2002 Sep;52(9):619-22. Recurrent cellular angiofibroma of the vulva.J Clin Pathol. 2002 ;55(6):477-9. Cellular angiofibroma. A rare vulvar tumor. Report of a case. Ann Pathol. 2001 ;21(2):145-8. Cellular angiofibroma of the vulva.Gynecol Oncol. 2001;81(2):326-9. Cellular angiofibroma of the vulva with DNA ploidy analysis.Int J Gynecol Pathol. 2001 Apr;20(2):200-3. Extravulvar subcutaneous cellular angiofibroma.J Cutan Pathol.1998;25(6):327-32 Cellular angiofibroma: a benign neoplasm distinct from angiomyofibroblastoma and spindle cell lipoma.Am J Surg Pathol. 1997;21(6):636-44.                           Your Ad Here Pathopedia-India.com: Contents ; Introduction of Pathology ; An outline of Diagnostic Techniques available in Pathology ; Cellular Injury ; Diagram showing Structural Changes in Reversible and Irreversible Cell Injury ; Autolysis; Heterolysis ; Necrosis; Coagulation (Coagulative) necrosis ; Caseative (Caseous) necrosis ; Liquefaction necrosis ; Fat necrosis ; Fibrinoid necrosis ; Apoptosis ; Gangrene ; Hyaline Change ; Atrophy ; Hypertrophy ; Hyperplasia ; Metaplasia ; Aplasia ; Hypoplasia ;Cellular Accumulations ; Accumulation of Glycogen, complex lipids and carbohydrates ; Pigments ; Melanin ; Pigments derived from Hemoproteins; Hemosiderin and Hemosiderosis ; Primary Hemochromatosis ; Hematin; Bilirubin; Lipofuscin; Mineral Dusts ; Silica ; Urate ; Amyloid ; Inflammation ; Inflammatory cells in acute and chronic inflammation ; Acute Inflammation; Types of Acute Inflammation; Chemical Mediators ; Chronic Inflammation; Wound Healing ; Circulatory Anatomy, Physiology and Regulation; Normal Fluid Balance; Edema; Morphology of Edema; Diagram showing Capillary System and Mechanisms of Edema Formation; Hyperemia and Congestion; Hemostasis and Thrombosis; Embolism; Fat Embolism; Air Embolism ; Decompression Sickness ; Amniotic Fluid Embolism ; Diagram showing Sources of Arterial Emboli ; Diagram showing Sources of Venous Emboli ; Infarction ; Diagram showing common sites of Systemic Infarction  from Arterial Emboli; Shock; Pathology of Shock; Diagram showing Complications of Shock; Hemorrhage; Custom Search PULMONARY PATHOLOGY: Congenital Cystic Adenomatoid  Malformation ; Acute Respiratory Distress Syndrome  ;Sarcoidosis ; Bronchiolitis ; Emphysema ; Bronchial Asthma ;Chronic Bronchitis Pulmonary Alveolar Proteinosis ; Lipid Pneumonia ; Pulmonary Hypertension ;Pulmonary edema ;Pulmonary Infection ; Pneumococcal Pneumonia ; Haemophilus influenza Infection;Klebsiella Pneumoniae ; Mycoplasma Pneumonia ; Pneumocystis Pneumonia ; Legionellosis ; Localized Fibrous Tumour of the Pleura ; Biphasic Epithelial/Mesenchymal Lung Tumours; Pulmonary Carcinosarcoma ; Pulmonary Blastoma ; Large Cell Neuroendocrine tumour;