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Orbital solitary fibrous tumor: a clinicopathologic study of ten cases with long-term follow-up

  • Clinical Article
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Summary

Background

Solitary fibrous tumor (SFT) is a rare spindle-cell benign neoplasm and located in orbit. The present research represents case reports of ten patients with orbital SFT.

Method

Prognosis was assessed by clinic service and telephone interview. Clinical data were retrieved from the medical records. Immunohistochemistry for CD34, CD99, EMA, HMB-45, Bcl-2, GFAP, S-100, MBP, CK, and MIB-1 was performed. Distributions of time to progression and recurrence between different operative methods were estimated using the Kaplan-Meier method and compared using the log-rank test.

Findings

The ten patients included six men and four women (range: 19–75 years). The most common initial symptom was painless proptosis (n = 6). After the ten initial surgeries, with four requiring gross total resection (GRT) and six requiring subtotal resection (STR), an additional nine were required to remove recurrent tumors. Eighteen samples were obtained for pathological examination (the third sample for case 4 was not achieved). Cellular areas with partial hemangiopericytoma pattern were noted in four samples (4/18); scarce mitosis was noted in six samples (6/18). All SFTs (18/18) were positive for CD34, CD99 and vimentin, while all SFTs (18/18) were negative for EMA, CK, MBP, HBM-45 and GFAP. Bcl-2 was positive in 13/18 cases, while S-100 was negative in 14/18 cases. The MIB-1 labeling index varied from 1% to 3%. Follow-up was available for nine patients with a median of 88 months. All four patients who underwent GTR on initial operation did not recur; while the five patients who underwent STR recurred. The log-rank test showed that the incomplete surgical resection was significantly associated with recurrence (p = 0.015).

Conclusions

Orbital SFT is a rare mesenchymal tumor and painless proptosis is the most common initial symptom. Immunohistochemistry should be used to differentiate SFT from other tumors. Orbital SFT usually has a non-aggressive histological characteristic, and has a good prognosis if GTR is performed. Postoperative regular and long-term follow-up remains mandatory to monitor recurrence.

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Acknowledgements

We thank professor Jian-Kang Shen for expert opinions during the development of this manuscript. We also thank Ya-Xuan Wang for collecting data. This work was supported by grants from Shanghai Leading Academic Discipline Project (S30205) and Shanghai Science and Technology Committee Major Program Project (10411964000).

Conflicts of interest

None.

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Authors and Affiliations

  1. Department of Neuropathology, Huashan Hospital, Fudan University, Shanghai, 200040, China

    Hong Chen

  2. Department of Ophthalmology, Ninth People’s Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, 200011, China

    Cai-Wen Xiao

  3. Department of Neurological Surgery, Wayne State University, Wayne, MI, 48201, USA

    Tony Wang

  4. Department of Neurosurgery, Huashan Hospital, Fudan University, Shanghai, 200040, China

    Jin-Song Wu, Cheng-Chuan Jiang & Xiao-Qiang Wang

  5. Department of Ophthalmology, Eye and ENT Hospital, Fudan University, Shanghai, 200031, China

    Jiang Qian & Cheng-Hua Wei

Authors
  1. Hong Chen
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  2. Cai-Wen Xiao
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  3. Tony Wang
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  4. Jin-Song Wu
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  5. Cheng-Chuan Jiang
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  6. Jiang Qian
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  7. Cheng-Hua Wei
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  8. Xiao-Qiang Wang
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Corresponding authors

Correspondence to Jiang Qian or Xiao-Qiang Wang.

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Comment

Neurosurgery at Huashan Hospital, Shanghai, is at its own level of magnitude—for example, 6,700 meningiomas, 183 hemangiopericytomas and 28 solitary fibrous tumors (SRTs) of the central nervous system (CNS) operated on in a 9-year period (2002–10). The authors report ten cases of solitary fibrous tumour (SFT) of the orbital cavity. Orbital SFT is so rare that this reviewer encountered none in 30 years—but the authors rightly point out that SFTs have been underdiagnosed. China and its megacities allow the creation of huge neurosurgical units compared with our European ones, and such units may produce neurosurgical series much larger than published from Europe.

Lessons for researchers of clinical neuro-oncology in Europe?

1. National and international CNS tumor series rather than single-center ones.

2. Comparison of long-term outcome with that of matched catchment populations—double almost exclusively in Europe.

Juha E Jääskeläinen

Kuopio Finland

1. Vassal F, Manet R, Forest F, Camdessanche JP, Péoc’h M, Nuti C (2011) Solitary fibrous tumors of the central nervous system: report of five cases with unusual clinicopathological and outcome patterns. Acta Neurochir (Wein) 153:377–384

Hong Chen and Cai-Wen Xiao contributed equally to this work

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Chen, H., Xiao, CW., Wang, T. et al. Orbital solitary fibrous tumor: a clinicopathologic study of ten cases with long-term follow-up. Acta Neurochir 154, 249–255 (2012). https://doi.org/10.1007/s00701-011-1254-4

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  • DOI: https://doi.org/10.1007/s00701-011-1254-4

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