Orbital solitary fibrous tumor: a clinicopathologic study from a Chinese tertiary hospital with a literature review

Abstract

PurposeTo report the clinical features, imaging manifestations, histopathology, and immunohistochemical features of several cases of orbital solitary fibrous tumors (SFTs) in a Chinese tertiary hospital, and to undertake a literature review of this rare disease.MethodsA non-comparative retrospective review of clinical presentations, imaging manifestations, histopathology, and immunohistochemical features as well as the management and disease outcomes of patients with orbital SFT was conducted along with a review of orbital SFT cases in the literature.ResultsThis study includes two male and two female patients, with an average age of 53 years. Common presentations among these patients included a palpable subcutaneous mass, swelling of the eyelid, proptosis, diplopia, and vision disturbance. Three patients (cases 2–4) underwent imaging scans. All patients had complete surgical excisions and the tissue was subjected to pathological analysis. One patient (Case 4) experienced a recurrence with malignant transformation and received a re-excision surgery and postoperative radiotherapy. All patients remain alive and well after a minimum follow-up of 12 months (range 12–34 months).ConclusionDespite its rare occurrence, we suggest that the possibility of orbital SFTs needs to be considered when a painless, slowly growing orbital mass is identified. Typical characteristic magnetic resonance imaging features of orbital SFTs are iso- or hypointense signals on T1 and T2-weighted images, with marked enhancement. A positive cluster of differentiation 34 (CD34) staining is an important diagnostic clue favoring SFT. Some orbital SFTs are infiltrating, aggressive, or recur with malignant transformation. Therefore, regular long-term follow-up after complete excision is mandatory.

A Clinicopathologic Study of Seven Cases of Orbital Solitary Fibrous Tumours

“…Another feature published in the literature, demonstrated by our patients, was the numerous branching of thin-walled vessels of different sizes -the staghorn pattern [7][8]. Other features include thick bands of collagen interspersed between tumour cells and alternating hypo-and hypercellular areas [3,7,9].…”

“…Immunohistochemical studies specific to SFT include strong and diffuse positivity to CD34, vimentin and BCL-2 and non-specific reactivity to CD99 [7,[9][10]. SFTs are negative to desmin, cytokeratin, factor VIII-related antigen, S-100, smooth muscle actin (SMA), and muscle-specific actin [3,[9][10].…”

“…Though five patients had positive staining for S-100, the strong positive staining of CD34 and the presence of other positive staining were supportive of the diagnosis of SFT. Interestingly, a reported case series with patients that showed negative staining for S-100 had patients from an older age group [3,[11][12]. However, a case report of a 22-year-old patient showed equivocal results for S-100 staining [6].…”

“…Mainstay treatment is complete excision of the tumour. Local invasion and recurrence are associated with incomplete excision of the tumour [3,7,14].…”

Factors for Postoperative Recurrence of Orbital Solitary Fibrous Tumor: An Analysis of Long-Term Clinical Follow-Up Results From a Chinese Tertiary Hospital

“…Complete removal of the tumor determines the prognosis of the disease. 13 Most of the current surgical operations are performed by ophthalmologists, [14][15][16][17] but the surgical approaches of ophthalmology are relatively limited. When the location of the tumor is not suitable for the operation of the ophthalmologist, an improper surgical approach may bring the possibility of tumor recurrence.…”