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Taiwan Journal of Ophthalmology 3 (2013) 120e122

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Taiwan Journal of Ophthalmology

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Case report

Orbital solitary fibrous tumor: A report of two cases

Wai-Man Cheang a, Li-Chen Wei a,*, John Wang b, Hin-Yeung Tsai a

aDepartment of Ophthalmology, Taichung Veterans General Hospital, Taichung, Taiwan, ROCbDepartment of Pathology, Taichung Veterans General Hospital, Taichung, Taiwan, ROC

a r t i c l e i n f o

Article history:Received 8 January 2013Received in revised form23 April 2013Accepted 13 May 2013Available online 25 June 2013

Keywords:immunohistochemical stainingorbital solitary fibrous tumorsurgery

* Corresponding author. Department of OphthaGeneral Hospital, Number 160, Section 3, Chung-KanTaiwan, ROC.

E-mail address: lichen5883@yahoo.com.tw (L.-C. W

2211-5056/$ e see front matter Copyright � 2013, Thhttp://dx.doi.org/10.1016/j.tjo.2013.05.004

a b s t r a c t

Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm that can be found in the orbit. Here, we reporttwo cases affected by orbital SFT. Both patients were female, aged 52 years and 59 years, respectively, andhad experienced a painless unilateral orbital lesion. Computed tomography (CT) imaging revealed a well-circumscribed and contrast-enhanced soft tissue mass simultaneously. The tumors were located in thelaterotemporal extraconal space of the right orbit and the inferior portion of the left orbit, respectively.Both patients underwent complete resection of their tumors. The histological findings showed alter-nating hypercellular and hypocellular areas composed of bland spindle cells with a fibrous stroma. Thestrong immunoreactivity for CD34 supported the diagnosis of orbital SFT. There was no recurrence at the2-year and 3-year follow-up visits, respectively. SFTs should be considered in the differential diagnosis ofan orbital tumor. The combination of a CT scan, histologic findings, and immunohistochemical stainingwill provide an accurate diagnosis. En bloc excision of the tumor is the mainstay of treatment in order toavoid recurrence.Copyright � 2013, The Ophthalmologic Society of Taiwan. Published by Elsevier Taiwan LLC. All rights

reserved.

1. Introduction

Solitary fibrous tumors (SFTs) arise from mesenchymal cells orfibroblasts. They usually present in the pleura, but recently a fewcases involving the orbit have been described.1,2 Patients with anorbital SFT may present with unilateral, painless, slowly progres-sive proptosis or a palpable mass in the periocular area. Computedtomography (CT) scanning and magnetic resonance imaging (MRI)reveal a well-circumscribed, contrast-enhanced soft tissue mass.3

Histological examination and positive CD34 immunoreactivity areuseful in discriminating SFTs from other tumors.4,5 Here, we reporttwo cases of orbital SFT.

2. Case reports

2.1. Case 1

A 52-year-old woman developed gradual proptosis of the righteye over several months. On ophthalmic examination, her visualacuity was 6/6.7 in the right eye. There was upward and lateral

lmology, Taichung Veteransg Road, Taichung City 40705,

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limitation of right ocular movement. The lesion caused an inferiordisplacement of the eyeball and exophthalmos (Fig. 1A). Hertelexophthalmometry gave readings of 16.5 mm for the right eye and13 mm for the left eye. CT scans revealed a contrast-enhancedsolitary mass located in the lateral extraconal space of the rightorbital cavity without bony erosion (Fig. 1B).

A lateral orbitotomy was performed. The mass was dissectedand excised completely (Fig. 1C). The bone fragment was thenreplaced and secured with 3-0 nylon through pre-drilled holes.In its gross dimensions, the lesion was a 3.8 cm � 3 cm � 1.5 cm,thin, encapsulated, and well-circumscribed tumor. Microscopi-cally, it revealed coexistent hypocellular and hypercellular areasseparated by fibrous stroma (Fig. 1D and E). The hypercellularareas were composed of bland spindle cells with a storiformpattern. No mitotic activity or atypia cells were found. Thesubsequent immunohistochemical staining revealed CD34 posi-tivity (Fig. 1F), Ki-67 positivity of less than 5%, and S100 nega-tivity. The patient was free of recurrence at a 2-year post-surgicalfollow-up.

2.2. Case 2

A 59-year-old woman had developed a slowly progressing,palpable, painlessmass over the infraorbital area of her left eye overthe previous 3 years. Her visual acuity was 6/6.7 for the left eye.

an. Published by Elsevier Taiwan LLC. All rights reserved.

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Fig. 1. (A) Upper eyelid swelling, proptosis, and medial inferior deviation of the globe is noted in the patient’s right eye. (B) Coronal CT scan reveals a contrast-enhanced mass (*)next to the lacrimal gland (arrow) in the lateral extraconal space of the right orbit, causing downward displacement of the globe. (C) The 3.5 cm mass is dissected and removedcompletely. (D) Photomicrograph showing diffuse spindle cells and collagen deposition (magnification 200�). (E) Histology reveals the deposition of abundant collagen fibers andhypercellular (arrowhead) and hypocellular (asterisk) areas (magnification 100�). (F) Immunohistochemical staining shows positivity for CD34 (magnification 200�).

W.-M. Cheang et al. / Taiwan Journal of Ophthalmology 3 (2013) 120e122 121

Findings relating to extraocular movement, pupillary examination,and Hertel exophthalmometry were unremarkable. CT scansrevealed a well-enhanced orbital mass located at the left inferioraspect of the orbit (Fig. 2). The patient underwent anterior orbitot-omy via the lower eyelid. A 2 cm� 1.5 cm� 1 cm, tan-colored, well-circumscribed tumorwas dissected and completely removed. Therewas no infiltrative margin or tumor necrosis.

Microscopically, alternating hypercellular and hypocellularareas with thickened keloid-like collagen fibers were seen. Therewere no mitoses or cellular atypia. Immunohistochemical stainingrevealed a strong positive reaction for CD34, but negative stainingfor actin M851, S100, and AE1/AE3. Ki67 showed only 1% positivity.There was no recurrence of the tumor at a 3-year post-surgicalfollow-up.

3. Discussion

SFTs are uncommon, benign, and slowly progressing spindle-cell tumors. They were originally described on the mesothelialsurfaces of the pleura. In line with the hypothesized origin of thetumor frommesenchymal cells with fibroblastic proliferation, it hasbeen called a “localized mesothelioma.”1 However, the histogenesisof SFT is still controversial. Recently, this tumor has been describedin extrapleural sites such as the upper airway tract, nasal andparanasal sinuses, parotid and salivary glands, thyroid, lung,mediastinum, pericardium, peritoneum, liver, spine, soft tissue, andorbit.1 Orbital SFTs can affect any orbital space. Lesions arising fromlacrimal gland fossa, lacrimal sacs, conjunctivae, and sclerae havealso been reported.6e9

Fig. 2. Computed tomography shows a well-enhanced lesion over the inferior aspectof the left orbital cavity (arrow).

W.-M. Cheang et al. / Taiwan Journal of Ophthalmology 3 (2013) 120e122122

Only 48 cases of orbital SFT have been published in the litera-ture.1,2 Orbital SFTs affect a wide age range (9e76 years) of patients,predominating in middle-aged adults. There is no obvious predi-lection by sex. The most common clinical sign is unilateral painlessproptosis with an insidious onset over several months to years(range: 6e84 months). In our study, both cases were middle-agedwomen who presented with a progressive, painless, unilateralorbital mass.

In our cases, the orbital SFTs were shown as well-circumscribed,contrast-enhanced masses on CT imaging. The radiological findingsof orbital SFT have been described by Kim et al.3 Orbital SFTs appearas well-circumscribed masses with moderate to intense enhance-ment and mostly without characteristics of bony erosion on CTscanning. On MRI scans, they demonstrate T1-weighted signalisointensity and T2-weighted hypointensity. However, the radio-logic features of orbital SFTs are rather nonspecific. It is difficult todifferentiate them from highly vascular lesions such as hemangi-omas, fibrous histiocytomas, neurofibromas, meningiomas, andschwannomas.

The first orbital SFTwas independently reported byWestra et al4

and Dorfman et al5 in 1994. They described the classic histopath-ologic features and immunohistochemical characteristics of SFTs,with alternating hypercellular and hypocellular areas of spindlecells against a background of thick collagen bundles, and ahemangiopericytoma-like pattern of vascularity.4,5,10 Moreover,CD34 is believed to be the most important diagnostic marker forSFT. SFT shows a strong and diffuse immunopositivity for CD34 (in79e100% of cases), vimentin, and bcl-2, and immunonegativity forkeratin, cytokeratin, epithelial membrane antigen, S100, smoothmuscle actin, and desmin on immunohistochemical staining.4,11e13

Both of our cases showed a low Ki67 proliferative indexdless than5% and only 1%, respectively. The Ki-67 index usually reacts in 0e2%of spindle-cell nuclei in benign SFT. In malignant cases, the per-centage of positively staining nuclei can be up to 40%.14

The mainstay of treatment of orbital SFTs is surgical resection.Generally, orbital SFTs represent a benign disease, and most cases

have been treated by local excision. Both of our patients were freeof recurrence for several years after complete resection of theirtumor. However, local recurrence has been reported in nine cases inthe literature.1,2 These were mainly related to incomplete initialexcision and the increased mitotic activity of the tumor. Only onecase of malignant transformation with 8 years of follow-up hasbeen reported to date.15 The aggressive histologic pattern of thetumor can include abnormal mitotic figures, cellular pleomor-phism, and giant tumor cells.16e18

In conclusion, SFTs are rare but should be included in the dif-ferential diagnosis of orbital tumors in a patient who presents witha well-circumscribed and contrast-enhanced mass. The histologiccharacteristics and immunohistochemical markers, including CD34positivity, are important diagnostic tools in diagnosing an SFT.Complete surgical excision with careful postoperative follow-up isthe mainstay of treatment.

References

1. Bernardini FP, de Conciliis C, Schneider S, Kersten RC, Kulwin DR. Solitaryfibrous tumor of the orbit. Is it Rare? Report of a case series and review of theliterature. Ophthalmology 2003;110:1442e8.

2. Subramanian N, Mohan ER, Mahesh L, Biswas J, Rao NA. Solitary fibrous tumorof the orbit: a clinicopathologic study of six cases with review of the literature.Surv Ophthalmol 2003;48:544e54.

3. Kim HJ, Kim HJ, Kim YD, Yim YJ, Kim ST, Jeon P, et al. Solitary fibrous tumor ofthe orbit: CT and MR imaging findings. Am J Neuroradiol 2008;29:857e62.

4. Westra WH, Gerald W, Rosai J. Solitary fibrous tumor. Consistent CD34immunoreactivity and occurrence in the orbit. Am J Surg Pathol 1994;18:992e8.

5. Dorfman DM, To K, Dickersin GR, Rosenberg AE, Pilch BZ. Solitary fibrous of theorbit. Am J Surg Pathol 1994;18:281e7.

6. Scott IU, Tanenbaum M, Rubin D, Lores E. Solitary fibrous tumor of the lacrimalgland fossa. Ophthalmology 1996;103:1613e8.

7. Woo KI, Suh YL, Kim YD. Solitary fibrous tumor of the lacrimal sac. Ophthal PlastReconstr Surg 1999;15:450e3.

8. Pe’er J, Maly A, Deckel Y, Frenkel S. Solitary fibrous tumor of the conjunctiva.Arch Ophthalmol 2007;125:423e6.

9. Su GW, Perez N, Simons KB, Harris GJ. Solitary fibrous tumor of the sclera. ArchOphthalmol 2007;125:1572e4.

10. Arturi L, Bollero D, Pucci A, Ferrero R, Hamedani M. Solitary fibrous tumor ofthe orbit: clinical and histological evidence. Eur J Plast Surg 2004;26:419e21.

11. Suster S, Fisher C, Moran CA. Expression of bcl-2 oncoprotein in benign andmalignant spindle cell tumors of soft tissue, skin, serosal surfaces, andgastrointestinal tract. Am J Surg Pathol 1998;22:863e72.

12. Paal E, Miettinen M. Retroperitoneal leiomyomas: a clinicopathologic andimmunohistochemical study of 56 cases with a comparison to retroperitonealleiomyosarcomas. Am J Surg Pathol 2001;25:1355e63.

13. Miettinen M, Shekitka KM, Sobin LH. Schwannomas in the colon and rectum: aclinicopathologic and immunohistochemical study of 20 cases. Am J Surg Pathol2001;25:846e55.

14. Hanau CA, Miettinen M. Solitary fibrous tumor: histological and immunohis-tochemical spectrum of benign and malignant variants presenting at differentsites. Hum Pathol 1995;26:440e9.

15. Carrera M, Prat J, Quintana M. Malignant solitary fibrous tumor of the orbit:report of a case with 8 years follow up. Eye 2001;15:102e4.

16. Heathcote JG. Pathology update: solitary fibrous tumor of the orbit. Can JOphthalmol 1997;32:432e5.

17. Alexandrakis G, Johnson TE. Recurrent orbital solitary fibrous tumor in a 14-year-old girl. Am J Ophthalmol 2000;130:373e6.

18. McElvanney AM, Noble JL, O’Donovan DG, Bonshek RE, Banerjee SS. Solitaryfibrous tumour: an atypical presentation within the orbit. Eye 1996;10(Pt 3):396e9.

http://refhub.elsevier.com/S2211-5056(13)00036-7/sref1http://refhub.elsevier.com/S2211-5056(13)00036-7/sref1http://refhub.elsevier.com/S2211-5056(13)00036-7/sref1http://refhub.elsevier.com/S2211-5056(13)00036-7/sref1http://refhub.elsevier.com/S2211-5056(13)00036-7/sref2http://refhub.elsevier.com/S2211-5056(13)00036-7/sref2http://refhub.elsevier.com/S2211-5056(13)00036-7/sref2http://refhub.elsevier.com/S2211-5056(13)00036-7/sref2http://refhub.elsevier.com/S2211-5056(13)00036-7/sref3http://refhub.elsevier.com/S2211-5056(13)00036-7/sref3http://refhub.elsevier.com/S2211-5056(13)00036-7/sref3http://refhub.elsevier.com/S2211-5056(13)00036-7/sref4http://refhub.elsevier.com/S2211-5056(13)00036-7/sref4http://refhub.elsevier.com/S2211-5056(13)00036-7/sref4http://refhub.elsevier.com/S2211-5056(13)00036-7/sref5http://refhub.elsevier.com/S2211-5056(13)00036-7/sref5http://refhub.elsevier.com/S2211-5056(13)00036-7/sref5http://refhub.elsevier.com/S2211-5056(13)00036-7/sref6http://refhub.elsevier.com/S2211-5056(13)00036-7/sref6http://refhub.elsevier.com/S2211-5056(13)00036-7/sref6http://refhub.elsevier.com/S2211-5056(13)00036-7/sref7http://refhub.elsevier.com/S2211-5056(13)00036-7/sref7http://refhub.elsevier.com/S2211-5056(13)00036-7/sref7http://refhub.elsevier.com/S2211-5056(13)00036-7/sref8http://refhub.elsevier.com/S2211-5056(13)00036-7/sref8http://refhub.elsevier.com/S2211-5056(13)00036-7/sref8http://refhub.elsevier.com/S2211-5056(13)00036-7/sref9http://refhub.elsevier.com/S2211-5056(13)00036-7/sref9http://refhub.elsevier.com/S2211-5056(13)00036-7/sref9http://refhub.elsevier.com/S2211-5056(13)00036-7/sref10http://refhub.elsevier.com/S2211-5056(13)00036-7/sref10http://refhub.elsevier.com/S2211-5056(13)00036-7/sref10http://refhub.elsevier.com/S2211-5056(13)00036-7/sref11http://refhub.elsevier.com/S2211-5056(13)00036-7/sref11http://refhub.elsevier.com/S2211-5056(13)00036-7/sref11http://refhub.elsevier.com/S2211-5056(13)00036-7/sref11http://refhub.elsevier.com/S2211-5056(13)00036-7/sref12http://refhub.elsevier.com/S2211-5056(13)00036-7/sref12http://refhub.elsevier.com/S2211-5056(13)00036-7/sref12http://refhub.elsevier.com/S2211-5056(13)00036-7/sref12http://refhub.elsevier.com/S2211-5056(13)00036-7/sref13http://refhub.elsevier.com/S2211-5056(13)00036-7/sref13http://refhub.elsevier.com/S2211-5056(13)00036-7/sref13http://refhub.elsevier.com/S2211-5056(13)00036-7/sref13http://refhub.elsevier.com/S2211-5056(13)00036-7/sref14http://refhub.elsevier.com/S2211-5056(13)00036-7/sref14http://refhub.elsevier.com/S2211-5056(13)00036-7/sref14http://refhub.elsevier.com/S2211-5056(13)00036-7/sref14http://refhub.elsevier.com/S2211-5056(13)00036-7/sref15http://refhub.elsevier.com/S2211-5056(13)00036-7/sref15http://refhub.elsevier.com/S2211-5056(13)00036-7/sref15http://refhub.elsevier.com/S2211-5056(13)00036-7/sref16http://refhub.elsevier.com/S2211-5056(13)00036-7/sref16http://refhub.elsevier.com/S2211-5056(13)00036-7/sref16http://refhub.elsevier.com/S2211-5056(13)00036-7/sref17http://refhub.elsevier.com/S2211-5056(13)00036-7/sref17http://refhub.elsevier.com/S2211-5056(13)00036-7/sref17http://refhub.elsevier.com/S2211-5056(13)00036-7/sref18http://refhub.elsevier.com/S2211-5056(13)00036-7/sref18http://refhub.elsevier.com/S2211-5056(13)00036-7/sref18http://refhub.elsevier.com/S2211-5056(13)00036-7/sref18

Orbital solitary fibrous tumor: A report of two cases1 Introduction2 Case reports2.1 Case 12.2 Case 2

3 DiscussionReferences