Safety and efficacy of combined immunosuppression and orbital radiotherapy in thyroid-related restrictive myopathy: two-center experience.

PURPOSE: To evaluate the safety and efficacy of oral steroids when combined with long-term oral azathioprine (AZA) and orbital radiotherapy in patients with active thyroid-related restrictive myopathy. METHODS: A total of 88 patients from adnexal outpatient clinics of Bristol Eye Hospital, UK, and 2nd Department of Ophthalmology at Aristotle University of Thessaloniki, Greece, were enrolled in a retrospective, twin-center study. All patients were diagnosed with active thyroid eye disease and concomitant restrictive myopathy. Treatment included oral AZA, low-dose steroids, and orbital radiotherapy (20 Gy). Clinical activity scores as well as orthoptic assessments were consistently evaluated. Clinical activity scores, improved levels of diplopia, and single muscle excursions were considered major criteria for treatment success. RESULTS: Clinical success was achieved in 54 (61.4%), 57 (64.8%), and 61 (69.3%) patients at 3-, 6-, and 12-month time points, respectively, after the initiation of the combined treatment. At 18 months following initiation of treatment, the percentage of treatment success reached 73.9% (n = 65). Nine patients developed AZA-related side effects. In 4 patients the drug had to be discontinued. CONCLUSIONS: Combined immunosuppression with orbital radiotherapy appears to reduce morbidity in patients with marked restrictive myopathy by improving major motility parameters such as diplopia and duction amplitude.

Parosteal osteosarcoma of the orbit.

A 60-year-old woman was referred to Bristol Eye Hospital because of a progressive, painless, right proptosis. CT revealed a well-defined, hyperdense lesion adjacent to the lateral orbital wall. A marginal excision was performed. The mass was stony-hard, lobulated, and encapsulated. Histopathologic findings were consistent with a low-grade parosteal osteosarcoma. Parosteal osteosarcoma is a rare osteogenic tumor that usually affects the long bones. It represents a malignant, though well differentiated, tumor that has a relatively good prognosis after a wide excision. However, local recurrences are not rare and have been associated with dedifferentiation. The latter affects the prognosis adversely and, thus, regular follow-ups are strongly suggested after the initial tumor excision. In the present case, no further treatment was administered and the patient was reported disease free 26 months after surgery.