Soft tissue Tumors - Giant cell angiofibroma

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Soft tissue Tumors

Fibroblastic / myofibroblastic tumors

Giant cell angiofibroma

Reviewer: Komal Arora, M.D. (see Reviewers page)
Revised: 20 July 2012, last major update July 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Uncommon benign neoplasm of multinucleated giant cells and ectatic vascular spaces
● May be related to solitary fibrous tumor (Am J Surg Pathol 2000;24:971, Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;107:685, Ophthal Plast Reconstr Surg 2008;24:325)

Epidemiology
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● Median age 45 years
● More common in men in eyelid/orbital sites, more common in women elsewhere
● Often slow growing painful mass

Case reports
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● 25 year old woman with giant cell angiofibroma of parapharyngeal space (Int J Oral Maxillofac Surg 2010;39:1024)
● 44 year old man with buccal mucosa tumor (Med Oral Patol Oral Cir Bucal 2008;13:E540)
● 50 year old woman with inguinal tumor (Ann Diagn Pathol 2000;4:240)
● 57 year old woman with eyelid tumor extending into orbit (Can J Ophthalmol 2006;41:216)

Treatment
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● Excision, does not recur

Sites
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● Eyelid, nasolacrimal duct (Ophthal Plast Reconstr Surg 2001;17:202)
● Lacrimal sac or orbit (Am J Surg Pathol 1995;19:1286, Hum Pathol 2011;42:120)

Gross description
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● Well-circumscribed with variable capsule, median 3 cm (larger in extra-orbital regions), may have hemorrhagic or cystic cut surface

Micro description
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● Cellular areas with bland round to spindle cells in myxocollagenous stroma with small-medium thick walled vessels and multinucleated giant stromal cells that line ectatic vascular spaces

Micro images
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Various images

Oral cavity tumor

Positive stains
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● CD34, CD99, vimentin, variable bcl2

Negative stains
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● CD31, CD68, c-kit/CD117, muscle specific actin, S100, desmin

Cytogenetics
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● Rarely t(12;17)(q15;q23) (Cancer Genet Cytogenet 2006;165:157)
● Rarely 6q13 abnormalities (Cancer Genet Cytogenet 2000;116:47)

End of Soft Tissue Tumors > Fibroblastic / myofibroblastic tumors > Giant cell angiofibroma

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