BACKGROUND Synovial sarcoma is a malignant mesenchymal neoplasm with variable epithelial differentiation. Most synovial sarcoma cases are reported in young adults and can arise in any body site. Notably, primary orbital synovial sarcoma is rare. CASE SUMMARY An 8-year-old east Asian girl with 1-month history of gradual painless proptosis and lacrimation of the right eye was admitted. The patient presented with painless proptosis, downward eyeball displacement, and upward movement disorders. According to clinical manifestations, imaging examinations and postoperative immunohistochemical examinations, the diagnosis was monophasic synovial sarcoma with calcification. The patient underwent anterior orbitotomy procedure for removal of the right orbital mass under general anesthesia. The diagnosis of monophasic synovial sarcoma with calcification was confirmed finally through histological and immunohistochemical exam. The follow-up period was 6 mo, and no recurrence was observed during this period. CONCLUSION Primary orbital monophasic synovial sarcoma with calcification is a rare sarcoma, and clinical manifestations and imaging results are not specific. The tumor may present similar features as a benign tumor. Comprehensive analysis of clinical, radiological, and pathological findings is critically important for making the right diagnosis. Conventional treatment approach for synovial sarcoma is surgical resection with adjuvant or neoadjuvant radiotherapy, which is highly effective for localized tumors.

Rationale: Orbital schwannoma is a relatively rare orbital tumor, and calcification of the lesion is rarely found in the orbit. We report a case of orbital schwannoma which was characterized by calcification in the orbital muscle cone, and was cured by intracapsular excision. Patient concerns: A 54-year-old female with a complaint of a mass in the left orbit during a magnetic resonance imaging examination and symptom of dizziness 6 months before, presented with painless exophthalmos and vision decline in the left eye. Diagnoses: According to clinical manifestations, imaging examinations and postoperative immunohistochemical examinations, the diagnosis was orbital schwannoma, with calcification in the muscle cone. Interventions: The patient was treated by intracapsular excision of the left orbit. We removed the intracapsular mass and most part of the cyst wall in order to prevent orbital apex syndrome. Outcomes: The diagnosis of schwannoma with calcification was confirmed finally through histological and immunohistochemical exam. The patient was followed up for 28 months and the orbital CT scan showed that there were no significant lesions found in the orbital muscle cone. Lessons: Understanding clinical, imaging diagnostic, and histopathological features of rare orbital schwannoma with calcification will facilitate timely diagnosis and treatment of this condition. The intracapsular excision can help in avoiding complications.

BackgroundSolitary fibrous tumor (SFT) is an uncommon spindle-cell tumor of mesenchymal origin, it commonly occurs within pleura, the occurrence of SFT in the orbit is very rare. The diagnosis of orbital SFT is challenging in clinical, and requires an integrated approach that includes specific clinical, histological, immunohistochemical, and even molecular findings.MethodsIn our retrospective study, we collected 13 patients with orbital SFT. All patients were diagnosed with orbital SFT by postoperative histopathological and immunohistochemical examination. Patient charts and medical records were reviewed for demographic information, relevant medical and family history, clinical presentation, radiological examination, histopathological and immunohistochemical examination, the treatment and prognosis.ResultsThis study included 7 males and 6 females. The age ranged from 11 to 78 years. 7 patients presented with predominant features of exophthalmos. 7 patients showed eyeball dislocation. 6 patients with motility disturbances. 9 patients had the palpable masses. 6 patients had the secondary lesions. On CT scan, there were 6 patients were located in the superomedial quadrant of the orbit, 3 patients were located in the inferomedial quadrant of the orbit. The morphology of lesions was solitary ovoid mass in 10 patients, and irregular mass in 3 patients. The CT value of the tumor was 22.8-64.4Hu, with the median of 45.9Hu. On MRI, 3 patients showed hypointense mixed signals on T1WI, 10 patients showed isointense mixed signal on T1WI. 3 patients showed hypointense mixed signals, 4 patients showed isointense mixed signals, 6 patients showed hyperintense signals on T2WI. There were 12 patients showed obviously enhancement, and we found patchy lesions which were no enhancement in the tumor. All patients were treated by surgery. Immunohistochemical analysis showed that the tumor cells exhibited diffuse immunoreactivity for CD34 and CD99 in all the patients. The lesions were positive for Bcl-2 in 11 patients. The lesions were positivity for Ki-67 <5% in one patient, 5%-10% in 10 patients, >10% in 2 patients. ConclusionsThe lesions are often outside the muscular cone, most commonly located at superomedial quadrant and inferomeddial quadrant of the orbit. The density of the lesions is uneven in most cases, the mean CT values of the tumors on CT scans are variable. The signal of lesions on orbital MRI is uncertainty, it is inheterogeneous enhancement, and the lamellar unenhanced regions can also be found in the lesion. The complete gross resection, and even more aggressive wide excision is preferred. Although the Ki-67 labelling index is very low, malignant forms with an increased propensity for local recurrence have been found. A longer follow-up is needed to determine the recurrence rate of the disease, and to identify the causes of recurrence.

BACKGROUND Solitary fibrous tumor (SFT) is predominant within the pleura but very rare in the orbit, which is why the diagnosis of orbital SFT poses challenges in clinical practice. Accordingly, an integrated approach that incorporates specific clinical features, histological, histopathological, and immunohistochemical (IHC) examinations, and molecular analyses is warranted. AIM To retrospectively explore the clinical and imaging characteristics, treatment, outcomes of a series of patients with orbital SFT. METHODS We conducted a retrospective review of a series of patients diagnosed with a histopathologic orbital SFT treated at a single institution. All data on demogra/phics, clinical characteristics, imaging, treatment, postoperative histopathological and IHC examinations, and prognosis were collected. RESULTS In total, 13 patients were enrolled, 7 (53.8%) of whom had the tumor located in the superomedial quadrant of the orbit. Computed tomography revealed a solitary ovoid lesion in 10 (76.9%) patients and irregular lesion in 3 (23.1%) patients. Magnetic resonance imaging results were as follows: On T1 weighted images, 3 (23.1%) patients had hypointense mixed signals, whereas 10 (76.9%) patients showed isointense mixed signals; on T2 weighted images (T2WI), 3 (23.1%), 4 (30.8%), and 6 (46.2%) patients exhibited hypointense mixed, isointense mixed, and hyperintense signals, respectively. Notably, 12 (92.3%) patients showed significant enhancement, whereas there were patchy slightly enhanced areas in the tumor. All patients were treated by surgery. IHC analysis demonstrated that the tumor cells were immunoreactive for CD34, CD99, STAT-6, and vimentin in all patients. The lesions showed Ki-67 positivity < 5% in 1 (7.7) patient, 5%-10% in 10 (76.9%), and > 10% in 2 (15.4%). Two (15.4%) patients exhibited tumor recurrence. CONCLUSION The clinical manifestations and radiologic characteristics of orbital SFT are diverse and not specific. Accurate diagnosis and treatment require detailed radiological and histopathological/IHC evaluation.