Giant cell angiofibroma, GCA

Definition

A benign tumour containing multinucleate giant cells and angiectatic spaces. This entity is possibly closely related to solitary fibrous tumour, with one paper describing cases of intermediate appearance4.

Clinical features

Usually occurs in the middle-aged, most commonly in the orbit or eyelid of males and in extra-orbital sites in females. It may occur elsewhere in the head5,7 and neck4, mediastinum3, retroperitoneum4, back4, axilla7, vulva4 and inguinal region6,7.

Macroscopic appearances

The lesion is circumscribed and variably encapsulated4. The cut surface is yellowish-white and there may be haemorrhage and cystic change4.

Histopathology

Cellular areas consist of bland round to spindle cells, along with multinucleate giant cells; the nuclei are central or in a floret arrangement1,4. The giant cells often line pseudovascular spaces. There are well-formed thick-walled small to medium size vessels. There may be staghorn pericytomatous vascular spaces, similar to those seen in solitary fibrous tumour4. The stroma varies from collagenous to myxoid. One cases contained islands of adipocytes, creating an overlap with lipomatous hemangiopericytoma4.

Immunohistochemistry

Ultrastructure

Cells show fibroblastic features. In one case, they showed Schwannian features7.

Differential diagnosis

• Solitary fibrous tumour

If extra-orbital:

• Giant cell fibroblastoma

• Synovial sarcoma

• Fibrosarcoma

• MFH

• Deep fibrous histiocytoma

• Haemangiopericytoma

• Schwannoma

• Various neoplasms containing osteoclast-like histiocytes: the giant cells in GCA resemble the mononuclear component and are positive for CD34, CD99, sometimes for bcl-2, but are negative for CD68.

Prognosis

This tumour is benign, with rare local recurrences.

References

0World Health Organization Classification of Tumours, Tumours of Soft Tissues and Bone, IARC Press 2002.

1Dei Tos, A. P., S. Seregard, et al. (1995). "Giant cell angiofibroma. A distinctive orbital tumor in adults." Am J Surg Pathol 19(11): 1286-93.

2Hayashi, N., G. Borodic, et al. (1999). "Giant cell angiofibroma of the orbit and eyelid." Ophthalmology 106(6): 1223-9.

3Fukunaga, M. and S. Ushigome (1998). "Giant cell angiofibroma of the mediastinum." Histopathology 32(2): 187-9.

4Guillou, L., S. Gebhard, et al. (2000). "Orbital and extraorbital giant cell angiofibroma: a giant cell-rich variant of solitary fibrous tumor? Clinicopathologic and immunohistochemical analysis of a series in favor of a unifying concept." Am J Surg Pathol 24(7): 971-9.

5Mikami, Y., M. Shimizu, et al. (1997). "Extraorbital giant cell angiofibromas." Mod Pathol 10(11): 1082-7.

6Sigel, J. E., C. Fisher, et al. (2000). "Giant cell angiofibroma of the inguinal region." Ann Diagn Pathol 4(4): 240-4.

7Thomas, R., S. S. Banerjee, et al. (2001). "A study of four cases of extra-orbital giant cell angiofibroma with documentation of some unusual features." Histopathology 39(4): 390-6.

This page last revised 1.7.2004.

©SMUHT/PW Bishop