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A review of solitary fibrous tumours of the orbit and ocular adnexa

Abstract

Solitary fibrous tumour (SFT) is an uncommon spindle cell tumour of mesenchymal origin characterised by NAB2-STAT6 gene fusion. Although it was first described in the pleura, it can occur in connective tissue in any part of the body, but rarely presents in the orbit and ocular adnexa. SFT, which is part of the same disease spectrum as other fibroblastic tumours such as giant cell angiofibroma, haemangiopericytoma and fibrous histiocytoma, usually presents as a painless, slow-growing mass in any age group and generally follows a benign course, with a good prognosis after complete excision. However, malignant forms rarely occur. Even for benign tumours a more aggressive clinical behaviour is possible, with relentless infiltrative local growth, frequent recurrence following surgery, and malignant transformation with the potential for metastatic spread. Careful long-term follow-up is essential. The published literature on SFTs of the orbit and ocular adnexa is reviewed, and the aetiology, clinical presentation, epidemiology, radiological features, histopathology, immunohistochemistry, risk stratification, clinical management, and prognosis are discussed, reflecting on our own experience.

摘要

孤立性纤维性瘤(SFT)是一种以NAB2-STAT6基因融合为特征的罕见间充质梭形细胞肿瘤。虽然SFT最早报道出现于胸膜, 但它可发生于身体任何部位的结缔组织中, 但在眼眶和眼附属器中少见。SFT与其他成纤维细胞性肿瘤 (如巨细胞血管纤维瘤、血管外皮细胞瘤和纤维组织细胞瘤) 属于同一疾病谱, 在所有年龄组中通常表现为无痛的、生长缓慢的肿块, 并且通常作为良性疾病治疗, 完全切除后预后良好。然而, 也会发现恶性SFT。即使是良性肿瘤, 也可能出现更具侵袭性的临床表现, 包括持续性局部浸润性生长、术后频繁复发以及具有转移扩散风险的恶变。因此, 对SFT患者进行细致的长期随访很有必要。我们对已发表的有关眼眶和眼附属器SFT的文献进行了回顾, 并对SFT的病因、临床表现、流行病学、放射学特征、组织病理学、免疫组化、危险分层、临床治疗和预后进行了讨论, 提出了自己的见解。

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Fig. 1: Clinical presentation and progression of SFT in left superior rectus.
Fig. 2: CT scan features of SFT.
Fig. 3: MRI features of SFT.
Fig. 4: Histopathology and immunochemistry of SFT.

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CR proposed and devised the scope of the paper, conducted literature search, wrote the paper and made edits. PS contributed to the literature search and writing of the paper, and prepared the illustrations. DGO’D contributed to the literature search, critically reviewed the paper, suggested edits and contributed histopathology slides.

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Correspondence to Paolo Scollo.

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René, C., Scollo, P. & O’Donovan, D. A review of solitary fibrous tumours of the orbit and ocular adnexa. Eye (2022). https://doi.org/10.1038/s41433-022-02160-w

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