J Korean Ophthalmol Soc.  2008 Nov;49(11):1850-1856.

Two Cases of Giant Cell Angiofibroma in the Orbit

• Yun JH
• Jung BY
• Jeon C
• Suh YL
• Kim HJ
• Kim YD

Abstract

PURPOSE
To report two cases of giant cell angiofibroma in the orbit.
CASE SUMMARY
(Case 1) A 17-year-old girl was referred for evaluation of the left upper eyelid swelling which had developed 6 months ago. On initial examination, a 1.5 cm sized ovoid and nontender mass was palpated in the medial aspect of the left orbit. CT scan and MR imaging of the orbit showed a non-calcified, well-circumscribed homogenous soft tissue mass, which was uniformly enhanced and did not invade the adjacent tissue. Excisional biopsy of the orbital mass was performed. (Case 2) A 30-year-old man presented with left proptosis which had developed 2 months ago and hemorrhage into the upper and lower eyelid which had developed 1 week ago. CT scan and MR imaging showed an heterogeneously enhancing mass, not involving the adjacent tissue in the superior retrobulbar space. Excisional biopsy through a lateral orbitotomy was performed. Histologic evaluation revealed proliferation of spindle cells with pseudovascular spaces and multinucleated giant cells.Immunohistochemical staining for CD34 and vimentin was positive and staining for CD31, smooth muscle actin was negative. A diagnosis of giant cell angiofibroma was made.
CONCLUSIONS
The possibility of giant cell angiofibroma should be considered in the differential diagnosis for an orbital mass without a hemorrhage or with a hemorrhage in the eyelid in adult patients.

Keyword

Giant cell angiofibroma; Orbit

MeSH Terms

Actins
Adolescent
Adult
Angiofibroma
Biopsy
Diagnosis, Differential
Exophthalmos
Eyelids
Giant Cells
Hemorrhage
Humans
Muscle, Smooth
Orbit
Vimentin
Actins
Vimentin

Figure

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  Figure 1. Case 1. Photograph showing swelling of the left upper eyelid.

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  Figure 2. Case 1. Axial (A) and coronal (B) non-contrast orbit CT images of the orbit demonstrating a 1.2 cm×1.7 cm sized well-circumscribed homogenous soft tissue mass in the left orbit. Neither adjacent tissue invasion nor internal calcification is observed.

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  Figure 3. Case 1. Pre-contrast T1-weighted axial MR image demonstrating a left orbital mass with isosignal intensity compared with brain parenchyme (A). The mass shows homogeneous contrast enhancement in post-contrast fat suppressed T1-weighted axial MR image (B).

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  Figure 4. Case 1. (A) Histopathologic examination reveals patternless proliferation of spindle cells (H & E stain, ×100). (B) Note multinucleated giant cells throughout the stroma (arrows) and pseudovascular structures (arrow head) (H & E stain, ×400).

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  Figure 5. Case 1. Immunohistochemical studies with CD34 (A) and with vimentin (B) showing positive staining of spindle cells and giant cells (×400).

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  Figure 6. Case 2. Photograph showing proptosis, upper and lower lid hemorrhage and subconjunctival hemorrhage of the left eye.

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  Figure 7. Case 2. A: Axial precontrast CT image demonstrating a well circumscribed homogenous intraconal mass without internal calcification in the left orbit. B: Axial postcontrast CT image demonstrating a heterogeneous enhancement and no adjacent tissue invasion.

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  Figure 8. Case 2. T2-weighted axial (A) and coronal (B) MR images demonstrating an intraconal superior left orbital mass with mixed signal intensity.

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  Figure 9. Case 2. Dynamic MR images obtained preoperatively (A), immediately after contrast enhancement (B), at 1 minute (C) and 3 minutes (D). The images show intense enhancement immediately after contrast enhancement and no significant change for 3 minutes.

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