Background: Alveolar soft part sarcoma (ASPS) is a clinically and morphologically distinct malignant soft tissue tumour. It occurs mostly in the lower extremities in adults. The purpose of our study was to describe the related clinicopathologic factors, treatment and prognosis of recurrent orbital ASPS in children. Case presentation: Three children aged from 1 to 12 years presented with unilateral proptosis, restricted ocular motility and impaired visual acuity of the affected eye. Periodic acid Schiff (PAS) -positive diastase-resistant crystalline granules were found in all cases. Immunostaining of TFE-3, INL1 and Ki67 was positive in the tumour cells of two patients. The time until local recurrence of primary tumor in patient 2 and patient 3, who only underwent tumour resection, was shorter than that of patient 1, who underwent tumour excision and postoperative radiotherapy. The recurrent masses were successfully treated with complete tumour excision followed by adjuvant radiotherapy. Patient 1 presented metastasis at 11 years after radiotherapy. Conclusions: Orbital ASPS in children is easily misdiagnosed due to its rare occurrence and atypical clinical findings. Early diagnosis with multidisciplinary, complete surgical resection combined with adjuvant radiotherapy is essential for achieving long-term disease-free survival in orbital ASPS patients.

Objectives To determine the clinical features and course of thyroid-associated ophthalmopathy (TAO) in a large sample of Chinese patients. Design and methods We retrospectively identified a cohort of consecutive patients diagnosed with TAO at the West China Hospital from October 1, 2009 to October 1, 2019. We analysed clinical data from 3620 patients, including demographic data, clinical manifestations, ophthalmology examinations, and prognosis. Results TAO most frequently occurred with hyperthyroidism, with most patients developing TAO after thyroid disease (TD). The TAO phenotype was asymmetric in 375 (50.7%) euthyroid patients, 25 (27.8%) hypothyroid patients, and 314 (12.1%) hyperthyroid patients (p < 0.0001). The most frequent symptom was lid lag and the most commonly involved extraocular muscle was the inferior rectus. Severity assessment (NOSPECS score) and clinical activity assessment (Clinical Activity Scores, CAS) differed significantly between male and female patients (P < 0.000). The majority (88.8%) of patients had clinically inactive TAO, and only 3.2% of cases were sight-threatening. Regarding the clinical process, 75.5% of patients had an active phase time less than 12 months and 2.1% showed complete remission. Conclusions TAO most commonly develops in females and is closely related to hyperthyroidism. Euthyroid TAO often has an asymmetric clinical phenotype. CAS combined with magnetic resonance imaging can improve the detection of TAO. NOSPECS scores should be slightly refined regarding the criteria for corneal involvement. Clinical management of TAO should be individualized according to CAS or NOSPECS assessments and a multidisciplinary approach is paramount. A minority of patients showed complete remission.

Thyroid-associated ophthalmopathy (TAO) is common in Graves' disease. However, to date, no standard treatment has been established for TAO. The present study aimed to assess peribulbar injection of corticosteroids for TAO treatment as well as factors affecting therapeutic effectiveness. A retrospective cohort study was performed at West China Hospital, Sichuan University (Chengdu, China). Patients with TAO were administered peribulbar injection of triamcinolone acetonide and dexamethasone monthly. Ocular signs after each injection were assessed until the end of treatment. All patients were followed up for at least six months. Best corrected visual acuity, proptosis values, eye motility assessed by the Hess chart, as well as eyelid width and downward movement were determined. In addition, clinical data were retrospectively reviewed to explore factors affecting therapeutic effectiveness by logistic regression analysis. In the present study, 386 patients with TAO (515 eyes) were evaluated; 71.37% of cases of eyelid swelling were relieved and upper eyelid retraction was improved in 47.58% of affected patients. Eye movement disorders, diplopia and strabismus were all alleviated to varying degrees, with few adverse reactions. Logistic regression analysis demonstrated that therapeutic effectiveness was relatively lower in males [odds ratio (OR)=0.32, P=0.001] and patients with thyroid dysfunction (OR=0.41, P=0.002), and that non-smokers had a higher odds of substantial improvement (OR=4.62, P=0.008). The duration of TAO was not significantly associated with the clinical outcome. Patients with reduced disease severity and elevated clinical activity score exhibited higher effectiveness (all P<0.05). In conclusion, peribulbar injection of corticosteroids is effective in treating mild to moderate TAO, with the therapeutic response affected by gender, smoking and disease severity.

Background Although myxofibrosarcoma (MFS) is the most common mesenchymal tumor, occurrence in the orbit is extremely rare. A characteristic clinical feature of MFS is its propensity for local recurrence (LR). Still, none of published literature has described the entire clinical course of multiple recurrences of MFS in the orbit. Here we present an unusual case in which a patient with multiple recurrences of MFS in the orbit followed-up for 5 years. We describe its clinical-pathological correlation, treatment, and prognosis. Case presentation A 70-year-old woman sought treatment for a year history of right upper eyelid swelling in May 2014. Since then, she underwent three surgical procedures in the right orbit in the same region (in 2014, 2016, and 2017). The pathology analysis of the three surgical tissue samples was consistent with myxofibrosarcoma, characterized by spindle-shaped cells and variably myxoid stroma. In the 2 years follow-up after the third surgery, there was no evidence of tumor recurrence. Conclusion Myxofibrosarcoma of the orbit is extremely rare. Since few reports are available in the literature, the diagnosis and management of the orbit myxofibrosarcoma is still a challenge to ophthalmologists. Histopathology is highly valuable in the diagnosis. As a clinical characteristic, the lesions have a high risk of local recurrence; therefore, aggressive resection and careful postoperative follow-up are paramount.

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