Solitary Fibrous Tumours of the Orbit – Clinical Pathology, Therapy and Prognosis

Abstract

Background Solitary fibrous tumours are rare. The aim of this study is to describe the clinical features, therapy and outcome of affected patients and to identify factors associated with recurrence.

Methods Retrospective study of a cohort of 20 patients who underwent surgery for orbital solitary fibrous tumour at the University Department of Oral and Maxillofacial Surgery between 2002 and 2023. Demographic, clinical, and therapeutic data as well as tumour follow-up results were collected. Tumour volume and molecular genetic mutations were retrospectively determined.

Results The median patient age was 49.5 years at initial surgery. The left orbit was affected in 65% of cases. The most common clinical symptom was proptosis (80%). This was reported with a mean lateral difference of 3.9 mm (range: 1 – 10 mm). The tumours were localised predominantly in the intra- and extraconal space, craniolateral quadrant and middle third. The median tumour volume was 7.66 cm³ (range 2.15 – 12.57 cm³). In all patients, the diagnosis was made by pathological examination. All tumours investigated showed a NAB2-STAT6 mutation. The most frequently detected mutation was the fusion NAB2 exon 4 – STAT6 exon 2. All patients were initially managed with frontolateral orbitotomy. Incomplete resection (R1-status) occurred in 35% (n = 7). The recurrence rate was 25% (n = 5), with a median disease-free interval of 45.5 months (range 23 – 130). 80% (n = 4) of recurrences were initially R1-resected.

Conclusion Orbital solitary fibrous tumours are rare tumours and are clinically manifested by signs of displacement of orbital structures. Diagnosis is made by histology and immunohistochemistry and can be proven with the molecular genetic detection of the NAB2-STAT6 mutation. The therapy of choice is complete surgical resection. R1-resection is more likely in the intraconal location as well as in location in the posterior third of the orbit – due to difficult surgical accessibility. The greatest risk factor for the development of recurrence is incomplete surgical excision. Late recurrences are possible, which is why a long-term connection to a specialised clinic is necessary.

Fazit

• Der solitär fibröse Tumor ist eine seltene Tumorentität, der auch in der Orbita vorkommen kann. In den meisten Fällen zeigt er einen benignen Verlauf.

• Der therapeutische Goldstandard ist die vollständige operative Entfernung des Tumors. R1-Resektionen sind in der Orbita aufgrund der erschwerten operativen Zugänglichkeit häufiger als in anderen Lokalisationen.

• Nach einer R1-Resektion ist das Rezidivrisiko erhöht. Risikofaktoren für eine R1-Resektion sind die Lokalisation des Tumors in dem Intrakonalraum und/oder im hinteren Orbitadrittel.

Conclusion

• Solitary fibrous tumors are a rare tumor entity that can also occur in the orbit. In most cases, the course is benign.

• The therapeutic gold standard is complete surgical removal of the tumor. R1 resections are more common in the orbit than in other localizations due to impaired surgical accessibility.

• After R1 resection, the risk of recurrence is increased. Risk factors for R1 resection are localization of the tumor in the intraconal space and/or in the posterior third of the orbit.

Key words

solitary fibrous tumours - orbital neoplasia - frontolateral orbitotomy - retrospective study - nab2-stat6 mutation - recurrence risk factors

Publication History

Received: 05 July 2023

Accepted: 30 August 2023

Article published online:
15 November 2023

• References/Literatur

• 1 Klemperer P, Coleman BR. Primary neoplasms of the pleura. A report of five cases. Am J Ind Med 1930; 22: 1-31 DOI: 10.1002/ajim.4700220103.
• 2 Cox DP, Daniels T, Jordan RC. Solitary fibrous tumor of the head and neck. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010; 110: 79-84 DOI: 10.1016/j.tripleo.2010.01.023.
• 3 Thompson LDR, Liou SS, Feldman KA. Orbit Solitary Fibrous Tumor: A Proposed Risk Prediction Model Based on a Case Series and Comprehensive Literature Review. Head Neck Pathol 2021; 15: 138-152 DOI: 10.1007/s12105-020-01184-6.
• 4 Vijitha VS, Kapoor AG, Mittal R. et al. Preoperative embolisation of orbital solitary fibrous tumour. BMJ Case Rep 2020; 13: e235576 DOI: 10.1136/bcr-2020-235576.
• 5 Hanau CA, Miettinen M. Solitary fibrous tumor: histological and immunohistochemical spectrum of benign and malignant variants presenting at different sites. Hum Pathol 1995; 26: 440-449 DOI: 10.1016/0046-8177(95)90147-7.
• 6 Westra WH, Gerald WL, Rosai J. Solitary fibrous tumor. Consistent CD34 immunoreactivity and occurrence in the orbit. Am J Surg Pathol 1994; 18: 992-998 DOI: 10.1097/00000478-199410000-00003.
• 7 Robinson DR, Wu YM, Kalyana-Sundaram S. et al. Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing. Nat Genet 2013; 45: 180-185 DOI: 10.1038/ng.2509.
• 8 Chmielecki J, Crago AM, Rosenberg M. et al. Whole-exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors. Nat Genet 2013; 45: 131-132 DOI: 10.1038/ng.2522.
• 9 Gold JS, Antonescu CR, Hajdu C. et al. Clinicopathologic correlates of solitary fibrous tumors. Cancer 2002; 94: 1057-1068
• 10 Davanzo B, Emerson RE, Lisy M. et al. Solitary fibrous tumor. Transl Gastroenterol Hepatol 2018; 3: 94 DOI: 10.21037/tgh.2018.11.02.
• 11 Kayani B, Sharma A, Sewell MD. et al. A Review of the Surgical Management of Extrathoracic Solitary Fibrous Tumors. Am J Clin Oncol 2018; 41: 687-694 DOI: 10.1097/COC.0000000000000348.
• 12 Badawy M, Nada A, Crim J. et al. Solitary fibrous tumors: Clinical and imaging features from head to toe. Eur J Radiol 2022; 146: 110053 DOI: 10.1016/j.ejrad.2021.110053.
• 13 Loius DN, Ohgaki H, Wiestler OD, Cavenee WK. eds. WHO Classification of Tumours of the Central Nervous System (Revides 4th edition). Lyon: IARC; 2016
• 14 Fletcher CDM, Unni KK, Mertens F. eds. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press; 2002
• 15 Dorfman DM, To K, Dickersin GR. et al. Solitary fibrous tumor of the orbit. Am J Surg Pathol 1994; 18: 281-287 DOI: 10.1097/00000478-199403000-00008.
• 16 Demicco EG, Park MS, Araujo DM. et al. Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model. Mod Pathol 2012; 25: 1298-1306 DOI: 10.1038/modpathol.2012.83.
• 17 Bernardini FP, de Conciliis C, Schneider S. et al. Solitary fibrous tumor of the orbit: is it rare? Report of a case series and review of the literature. Ophthalmology 2003; 110: 1442-1448 DOI: 10.1016/S0161-6420(03)00459-7.
• 18 Alkatan HM, Alsalamah AK, Almizel A. et al. Orbital solitary fibrous tumors: a multi-centered histopathological and immunohistochemical analysis with radiological description. Ann Saudi Med 2020; 40: 227-233 DOI: 10.5144/0256-4947.2020.227.
• 19 Gao XJ, Peng XL, Wang YJ. et al. Orbital solitary fibrous tumours: clinicopathological characteristics and recurrence prediction. Graefes Arch Clin Exp Ophthalmol 2023; 261: 223-231 DOI: 10.1007/s00417-022-05795-3.
• 20 Gheorghisan-Galateanu AA, Terzea DC, Burcea I. et al. Cystic appearance – a new feature of solid fibrous tumours in the lacrimal gland: a case report with literature review. Diagn Pathol 2019; 14: 63 DOI: 10.1186/s13000-019-0845-x.
• 21 de Juan Ferre A, Alvarez Alvarez R, Casado Herraez A. et al. SEOM Clinical Guideline of management of soft-tissue sarcoma (2020). Clin Transl Oncol 2021; 23: 922-930 DOI: 10.1007/s12094-020-02534-0.
• 22 Maier M, Hofmann R, Rodepeter F. et al. [Retroperitoneal hemangiopericytoma-a rare case with multiple relapses-even 20 years after primary diagnosis]. Urologe A 2019; 58: 424-427 DOI: 10.1007/s00120-019-0879-4.
• 23 Yokoi T, Tsuzuki T, Yatabe Y. et al. Solitary fibrous tumour: significance of p 53 and CD34 immunoreactivity in its malignant transformation. Histopathology 1998; 32: 423-432 DOI: 10.1046/j.1365-2559.1998.00412.x.
• 24 Yoshida A, Tsuta K, Ohno M. et al. STAT6 immunohistochemistry is helpful in the diagnosis of solitary fibrous tumors. Am J Surg Pathol 2014; 38: 552-559 DOI: 10.1097/PAS.0000000000000137.
• 25 Furusato E, Valenzuela IA, Fanburg-Smith JC. et al. Orbital solitary fibrous tumor: encompassing terminology for hemangiopericytoma, giant cell angiofibroma, and fibrous histiocytoma of the orbit: reappraisal of 41 cases. Hum Pathol 2011; 42: 120-128 DOI: 10.1016/j.humpath.2010.05.021.
• 26 Nakada S, Minato H, Nojima T. Clinicopathological differences between variants of the NAB2-STAT6 fusion gene in solitary fibrous tumors of the meninges and extra-central nervous system. Brain Tumor Pathol 2016; 33: 169-174 DOI: 10.1007/s10014-016-0264-6.
• 27 Park HK, Yu DB, Sung M. et al. Molecular changes in solitary fibrous tumor progression. J Mol Med (Berl) 2019; 97: 1413-1425 DOI: 10.1007/s00109-019-01815-8.
• 28 Goldsmith JD, van de Rijn M, Syed N. Orbital hemangiopericytoma and solitary fibrous tumor: a morphologic continuum. Int J Surg Pathol 2001; 9: 295-302 DOI: 10.1177/106689690100900406.
• 29 Fritchie KJ, Jin L, Rubin BP. et al. NAB2-STAT6 Gene Fusion in Meningeal Hemangiopericytoma and Solitary Fibrous Tumor. J Neuropathol Exp Neurol 2016; 75: 263-271 DOI: 10.1093/jnen/nlv026.
• 30 Zhao L, Wang F, Wang JM. et al. [Diagnosis and treatment of orbit solitary fibrous tumor: a report of 4 cases]. Zhonghua Yan Ke Za Zhi 2016; 52: 268-272 DOI: 10.3760/cma.j.issn.0412-4081.2016.04.007.
• 31 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 (Wien) 2012; 154: 249-255 DOI: 10.1007/s00701-011-1254-4.
• 32 Yang P, Liu HC, Qiu E. et al. Factors for postoperative recurrence of orbital solitary fibrous tumor: an analysis of long-term clinical follow-up results from a Chinese tertiary hospital. BMC Ophthalmol 2021; 21: 61 DOI: 10.1186/s12886-021-01825-6.
• 33 Tanaka K, Yano H, Hayashi H. et al. Total resection combined with osteotomy is more effective for orbital solitary fibrous tumor excision: a report of three cases. Int Ophthalmol 2018; 38: 345-351 DOI: 10.1007/s10792-016-0421-2.
• 34 Catanzaro S, Copelli C, Manfuso A. et al. Intraoperative navigation in complex head and neck resections: indications and limits. Int J Comput Assist Radiol Surg 2017; 12: 881-887 DOI: 10.1007/s11548-016-1486-0.
• 35 Yu H, Shen SG, Wang X. et al. The indication and application of computer-assisted navigation in oral and maxillofacial surgery-Shanghaiʼs experience based on 104 cases. J Craniomaxillofac Surg 2013; 41: 770-774 DOI: 10.1016/j.jcms.2013.01.016.
• 36 Bumm K, Bohr C, Bozzato A. et al. [Soft tissue navigation and image-guided removal of foreign bodies in head and neck surgery]. HNO 2009; 57: 1016-1022 DOI: 10.1007/s00106-008-1779-x.
• 37 Saynak M, Veeramachaneni NK, Hubbs JL. et al. Solitary Fibrous Tumors of Chest: Another Look with the Oncologic Perspective. Balkan Med J 2017; 34: 188-199 DOI: 10.4274/balkanmedj.2017.0350.
• 38 Gholami S, Cassidy MR, Kirane A. et al. Size and Location are the Most Important Risk Factors for Malignant Behavior in Resected Solitary Fibrous Tumors. Ann Surg Oncol 2017; 24: 3865-3871 DOI: 10.1245/s10434-017-6092-z.
• 39 Demicco EG, Wagner MJ, Maki RG. et al. Risk assessment in solitary fibrous tumors: validation and refinement of a risk stratification model. Mod Pathol 2017; 30: 1433-1442 DOI: 10.1038/modpathol.2017.54.
• 40 Pasquali S, Gronchi A, Strauss D. et al. Resectable extra-pleural and extra-meningeal solitary fibrous tumours: A multi-centre prognostic study. Eur J Surg Oncol 2016; 42: 1064-1070 DOI: 10.1016/j.ejso.2016.01.023.
• 41 Sugita S, Segawa K, Kikuchi N. et al. Prognostic usefulness of a modified risk model for solitary fibrous tumor that includes the Ki-67 labeling index. World J Surg Oncol 2022; 20: 29 DOI: 10.1186/s12957-022-02497-2.
• 42 Girnita L, Sahlin S, Orrego A. et al. Malignant solitary fibrous tumour of the orbit. Acta Ophthalmol 2009; 87: 464-467 DOI: 10.1111/j.1755-3768.2008.01290.x.
• 43 Kakkar A, Sakthivel P, Rajeshwari M. et al. Recurrent Sinonasal CD34-Negative Malignant Solitary Fibrous Tumor Diagnosed on STAT6 Immunohistochemistry and NAB2-STAT6 Fusion. Head Neck Pathol 2020; 14: 250-256 DOI: 10.1007/s12105-018-00999-8.
• 44 Kim JM, Choi YL, Kim YJ. et al. Comparison and evaluation of risk factors for meningeal, pleural, and extrapleural solitary fibrous tumors: A clinicopathological study of 92 cases confirmed by STAT6 immunohistochemical staining. Pathol Res Pract 2017; 213: 619-625 DOI: 10.1016/j.prp.2017.04.026.
• 45 Baranov E, Hornick JL. Soft Tissue Special Issue: Fibroblastic and Myofibroblastic Neoplasms of the Head and Neck. Head Neck Pathol 2020; 14: 43-58 DOI: 10.1007/s12105-019-01104-3.
• 46 Jaquiéry C, Leiggener C, Cornelius CP. et al. Aktuelle Behandlungsstrategien von knöchernen Verletzungen der Orbitae. OP-Journal 2013; 29: 136-146 DOI: 10.1055/s-0033-1350790.