J Korean Ophthalmol Soc.  2013 Oct;54(10):1599-1604.

Malignant Solitary Fibrous Tumor of the Orbit

• Kim J
• Kim YD
• Woo KI

Abstract

PURPOSE
Solitary fibrous tumor (SFT) is a rare tumor of the pleura, mediastinum, pericardium and other organs affecting predominantly middle-aged patients. SFT arising in the orbit is extremely rare, and its malignant form is even rarer. The authors herein describe a case of malignant solitary fibrous tumor of the orbit.
CASE SUMMARY
A 67-year-old male presented with a 4-month history of right proptosis. On ophthalmologic examination, 9-mm proptosis was observed in the right eye, and extraocular movements were limited in all directions of gaze. CT scan and MR imaging showed a lobulated, well-enhancing mass adjacent to the lateral rectus muscle in the superolateral retrobulbar space. Excisional biopsy through a lateral orbitotomy was performed. Histopathological examination showed proliferation of spindle cells with a fascicular pattern interspersed with bands of collagen, increased cellularity, cellular pleomorphism, hemorrhage, necrosis and high mitotic activity. Immunohistochemical staining revealed diffuse positivity for CD34, CD99, Ki67 and p53, and malignant SFT was diagnosed.
CONCLUSIONS
Malignant SFT should be considered in the differential diagnosis of acutely progressing unilateral proptosis.

Keyword

Malignant solitary fibrous tumor; Orbit; Proptosis

MeSH Terms

Aged
Biopsy
Collagen
Diagnosis, Differential
Exophthalmos
Eye
Hemorrhage
Humans
Male
Mediastinum
Muscles
Necrosis
Orbit*
Pericardium
Pleura
Solitary Fibrous Tumors*
Collagen

Figure

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  Figure 1. Preoperative facial photographs showing proptosis and limitation of extraocular movements of the right eye.

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  Figure 2. Axial precontrast (A), axial (B) and coronal (C) postcontrast CT images of the orbit show a 47 mm-sized, lobulated soft-tissue mass with well enhancement in the superolateral aspect of the right posterior orbit.

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  Figure 3. Orbital MR images demonstrate a lobulated mass with homogenous enhancement (C, F), which shows iso-signal intensity to gray matter on both T1 (A, D) and T2 (B, E) -weighted axial and coronal images in the superolateral aspect of the right posterior orbit.

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  Figure 4. An intraoperative photograph shows 4.5 ×3.5 cm- sized lobulated mass.

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  Figure 5. (A) Hematoxylin and eosin stain shows spindle shaped cells with fascicular pattern interspersed with bands of collagen (×100). (B) Increased cellularity and mild cellular pleomorphism are identified (×400). (C) Remarkable necrosis is present (× 100).

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  Figure 6. (A, B, C, D) Immunohistochemical stains show diffuse positivity for CD34, CD99, Ki67 and p53, respectively (×200).

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