Soft tissue tumors - Giant cell angioblastoma

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Soft tissue tumors

Low / intermediate grade vascular tumors

Giant cell angioblastoma

Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 24 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Rare, congenital/neonatal soft-tissue tumor, infiltrative but slow growing; appears to be benign
● Hand, palate, scalp, rarely bone (Zhonghua Bing Li Xue Za Zhi 2010;39:752, Diagn Pathol 2012 Aug 29;7:113)

Treatment
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● Surgery, interferon-alpha

Clinical images
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Palate

Hand

Gross description
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● Ulcerated tumors infiltrating soft tissue and bone

Micro description
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● Solid, nodular, and plexiform proliferation of oval-spindle cells with striking concentric aggregation around small vascular channels
● Cells resemble undifferentiated mesenchymal cells, fibroblasts, myofibroblasts, pericytes
● Also large mononuclear and multinucleate giant cells with histiocytic features (Am J Surg Pathol 2001;25:185)

Micro images
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Femur

Stains

Positive stains
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● CD68 (large mononuclear and multinucleate giant cells)

Differential diagnosis
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● Bacillary angiomatosis: positive special stains for organisms, patients usually immunosuppressed
● Epithelioid hemangioendothelioma with osteoclast-like giant cells: Factor 8+ cells that don't resemble oval-spindle cells of giant cell angioblastoma, no concentric aggregation around vessels
● Giant cell fibroblastoma: CD34+, molecular rearrangements of #17 and #22
● Myopericytoma: older age group, no giant cells
● Plexiform fibrohistiocytic tumor: children/young adults, no onion-skin layering of tumor cells around vessels

End of Soft tissue tumors > Low / intermediate grade vascular tumors > Giant cell angioblastoma

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