Orbital solitary fibrous tumors: a multi-centered histopathological and immunohistochemical analysis with radiological description

Alkatan, Hind M.; Alsalamah, Abrar K.; Almizel, Abdulrahman; Alshomar, Khalid M.; Maktabi, Azza; Elkhamary, Sahar; Eberhart, Charles G.; Iuliano, Adriana; Lanni, Vittoria; Strianese, Diego

doi:10.5144/0256-4947.2020.227

Cited by 15 publications

(17 citation statements)

References 46 publications

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“…Compared with the cerebral cortex or muscle, MRI basically depicts isointensity and isointensity to hypointensity on T1WI and T2WI, respectively, although a few lesions may show heterogeneous hyperintensity or cystic appearance in SFT (5,16,20). It is known that signal intensity-void, tubular structures are shown on MRI, which represent fast-flow vessels within the tumor (5,16,20).…”

Section: Discussionmentioning

confidence: 99%

Solitary Fibrous Tumor of the Orbit: A Clinicopathologic Study of Two Cases With Review of the Literature

Mitamura

Kase

Suzuki

et al. 2020

In Vivo

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Background/Aim: Orbital solitary fibrous tumor (SFT) is a rare lesion among orbital tumors, which can be misdiagnosed as another mesenchymal tumor. In this study we report two cases of orbital SFT, focusing on the imaging and pathological findings of the vascular structure inside the tumor. Case Report: A 26-year-old woman and 43-year-old man presented with orbital SFT. The pathological findings revealed a patternless growth pattern of the tumor cells and hemangiopericytoma-like vascularity as well as thickened, dilated blood vessels within the tumor tissue. Tumor cells revealed a diffuse strong positivity for cluster of differentiation 34 (CD34) and signal transducer and activator of transcription 6 (STAT6) in both cases, while Bcell lymphoma 2 (bcl-2) and CD99 were positive in one case. Characteristic findings within the tumor were the arterial components, where a variety of STAT6, CD99 and bcl-2positive smooth muscle cells were intermingled. Conclusion: Histologically, the tumor tissues might be characterized by not only conventional hemangiopericytoma-like vasculature but also dilated arterial vessels, which were shown to be part of the tumor components.Solitary fibrous tumor (SFT), a rare spindle-cell tumor, commonly arises from the pleura (1) but may also occur in extrapleural sites (2-5). Orbital SFT is a rare lesion and about 90 cases have been reported in the literature to date (6, 7). This tumor is likely to simulate other mesenchymal tumors such as fibrous histiocytoma, liposarcoma, synovial sarcoma, and neurofibroma. Since SFT is rich in feeding arteries, preoperative clinical diagnosis is important in assessing the likelihood of intraoperative bleeding (8), and total tumor resection is recommended (9). However, histopathological findings of the feeding vessels in SFT remain unknown. In this study, we report two cases of orbital SFT, focusing on the imaging and pathological findings of the vascular structure inside the tumor. The institutional review board in Hokkaido University and Teine Keijinkai Hospital waived the ethical assessment of the clinical study because of case reports. This study adhered to the principles of the Declaration of Helsinki. Case ReportCase 1. A 26-year-old woman presented with progressive and painless lower eyelid swelling for 2 months. An elastic hard, non-tender and non-pulsatile mass was palpable in the lower eyelid. Computed tomography revealed a well-defined heterogeneous mass along the lower wall of the right orbit (Figure 1A). Magnetic resonance imaging (MRI) demonstrated an orbital lesion with an isointense signal on T1-weighted images (T1WI) (Figure 1B) and a hyperintense signal on T2weighted images (T2WI) (Figure 1C). Postcontrast T1WI revealed a strong enhancement of the lesion that contained a linear flow void-like hypointensity (Figure 1D, arrow). Surgical excision was performed. There was bleeding from the 3649 This article is freely accessible online.

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Section: Discussionmentioning

confidence: 99%

Solitary Fibrous Tumor of the Orbit: A Clinicopathologic Study of Two Cases With Review of the Literature

Mitamura

Kase

Suzuki

et al. 2020

In Vivo

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“…Das durchschnittliche Erkrankungsalter der Patienten mit einem SFT in der Orbita liegt bei 45 Jahren 3 , 18 . Mit einem medianen Erkrankungsalter von 46 Jahren sind die Ergebnisse der vorliegenden Studie mit den berichteten Daten in der Literatur in Einklang zu bringen.…”

Section: Diskussionunclassified

“…Dies ist auf die beengten Platzverhältnisse in der Orbita einerseits und die leichte Irritation des Bulbus als Sinnesorgan andererseits zurückzuführen. Die durchschnittliche Größe von SFT in der Augenhöhle bei Diagnosestellung beträgt 1 – 3 cm 3 , 18 , 19 . In dieser Patientenkohorte lag die durchschnittliche Tumorgröße gemessen am maximalen Durchmesser bei 28 mm und deckt sich somit mit den Daten aus der Literatur.…”

Section: Diskussionunclassified

Solitär fibröse Tumoren der Orbita – klinisch-pathologische Charakteristik, Therapie und Prognose

Meier,

Eckstein,

Baba

et al. 2023

Klin Monbl Augenheilkd

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Zusammenfassung Hintergrund Aufgrund der Seltenheit der solitär fibrösen Tumoren ist das Ziel dieser Studie, die klinischen Charakteristika, die Therapie und das Outcome betroffener Patienten zu beschreiben und Faktoren zu identifizieren, die mit einer Rezidiventwicklung einhergehen. Methodik Retrospektive Studie einer Kohorte von 20 Patienten, die zwischen 2002 und 2023 in der Universitätsklinik für Mund-Kiefer-Gesichtschirurgie an einem orbitalen solitär fibrösen Tumor operiert wurden. Demografische, klinische und therapeutische Daten sowie Ergebnisse aus der Tumornachsorge wurden gesammelt. Das Tumorvolumen und molekulargenetische Mutationen wurden nachbestimmt. Ergebnisse Das mediane Alter bei Erstoperation lag bei 49,5 Jahren. In 65% der Fälle war die linke Orbita betroffen. Das häufigste klinische Symptom war der Exophthalmus (80%). Dieser wurde mit einer mittleren Seitendifferenz von 3,9 mm (Range 1 – 10 mm) angegeben. Die Tumoren waren vermehrt im Intra- und Extrakonalraum, im kraniolateralen Quadranten und mittleren Drittel lokalisiert. Die Tumorvolumina lagen im Median bei 7,66 cm³ (Range 2,15 – 12,57 cm³). Bei allen Patienten wurde die Diagnose durch pathologische Untersuchungsmethoden gestellt. Alle molekulargenetisch untersuchten Tumoren wiesen eine NAB2-STAT6-Mutation auf. Am häufigsten detektiert wurde die Fusion NAB2 Exon 4 – STAT6 Exon 2. Alle Patienten wurden initial über eine frontolaterale Orbitotomie behandelt. Bei 35% (n = 7) kam es zu einer unvollständigen Resektion (R1-Status). Die Rezidivrate lag bei 25% (n = 5) mit einem medianen krankheitsfreien Intervall von 45,5 Monaten (Range 23 – 130). 80% (n = 4) der Rezidive wurden initial R1-reseziert. Schlussfolgerung Orbitale solitär fibröse Tumoren sind seltene Raumforderungen, die sich klinisch durch Zeichen der Bulbusverdrängung äußern. Die Diagnose wird durch histologische und immunhistochemische Untersuchungen gestellt und kann mit einem molekulargenetischen Nachweis der NAB2-STAT6-Mutation bewiesen werden. Der therapeutische Goldstandard ist die vollständige operative Entfernung. Eine R1-Resektion wird bei intrakonaler Lage sowie bei Lage im hinteren Orbitadrittel aufgrund der schwierigeren chirurgischen Erreichbarkeit wahrscheinlicher. Der größte Risikofaktor für die Entwicklung eines Rezidivs im weiteren Krankheitsverlauf ist die unvollständige operative Entfernung. Späte Rezidive sind möglich, weswegen eine langfristige Anbindung an eine spezialisierte Klinik notwendig ist.

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“…The hemosiderotic brolipomatous tumor is histologically characterized by the presence of mature adipose tissue and spindle cell component accompanied by hemosiderin pigment deposition within macrophages, in the cytoplasm of some of the spindle cells and also within the extracellular stroma. The spindle cell component of these lesions is generally positive for CD34 and negative for CD68, S100, SMA and desmin [4,5]. The biological course of SFT tends to be benign in nature, while some cases with recurrent SFT and its malignant transformation have been reported in recent years.…”

Section: Introductionmentioning

confidence: 99%

Clinicopathological Characteristics of Three Cases with Recurrent Orbital Solitary Fibrous Tumors: A Retrospective Study and Literature Review

Cui¹,

Wang²,

Cui³

et al. 2021

Preprint

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Purpose Solitary fibrous tumor (SFT) is a spindle cell neoplasm that rarely occurs in orbit. This study aimed to report clinical, imaging, and pathological features of three patients with recurrent orbital SFTs.Methods Clinical, imaging, and pathological data of the three patients were retrospectively reviewed, and the results were compared with those of previously reported cases with recurrent orbital SFT. Results One female and two male patients (mean age, 54 years old) were included in this study. The present cases and literature review showed that the average time to recurrence in patients who aged under 50 years old was shorter than that in those who aged over 50 years old. The most common site for recurrent orbital SFT was the retrobulbar area of the orbit (23.8%). Imaging examinations showed consistent intensity of MRI signals before and after recurrence. Immunohistochemical results of all cases revealed the expressions of CD34. The mitotic rate increased in 4/8 cases, and the percentage of Ki-67-positive cells was elevated in 5/16 cases. Conclusion These results suggested that young patients were more likely subjected to recurrent orbital SFT. The postoperative pathological diagnosis revealed that patients with recurrent orbital SFT had more nuclear abnormalities and mitotic activity, as well as a higher percentage of Ki-67-positive cells, indicating that orbital recurrent SFT tended to be malignant according to both morphological features and immunohistochemistry results.

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Orbital solitary fibrous tumors: a multi-centered histopathological and immunohistochemical analysis with radiological description

Cited by 15 publications

References 46 publications

Solitary Fibrous Tumor of the Orbit: A Clinicopathologic Study of Two Cases With Review of the Literature

Solitary Fibrous Tumor of the Orbit: A Clinicopathologic Study of Two Cases With Review of the Literature

Solitär fibröse Tumoren der Orbita – klinisch-pathologische Charakteristik, Therapie und Prognose

Clinicopathological Characteristics of Three Cases with Recurrent Orbital Solitary Fibrous Tumors: A Retrospective Study and Literature Review

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