A high index of suspicion and appropriate intervention with histological diagnosis can help in the management and quality of life in patients with metastatic orbital disease. Overall survival is limited and we encountered statistical limitations proving differences in the survival based on the sub-type of primary tumour involved. Metastatic orbital melanoma presented a higher incidence when compared with previous studies, probably due to the increase frequency of skin found in the Australian population.
Objective: To perform a multicenter review of the clinical features and treatment of 31 patients with idiopathic sclerosing orbital inflammation.
Methods:We included all patients with histologically confirmed idiopathic sclerosing orbital inflammation from 5 regional orbital centers. We reviewed the case notes to determine the clinical presentation, diagnostic features, and response to treatment. The main outcome measures were duration and nature of symptoms, anatomical location of disease, histopathological findings, treatment modalities, treatment efficacy and adverse effects, and final clinical status.Results: We included 13 male and 18 female patients ranging in age from 7 to 83 years. The average duration of symptoms at presentation was 13.4 months. There was a predilection for the lateral and superior quadrants. Thir-teen patients had apical disease, and 4 had extraorbital involvement. Histopathological findings invariably showed sclerosis associated with a sparse mixed cellular infiltrate. Twenty-seven patients were treated with oral prednisolone, response to which was good in 9 patients, partial in 11, and poor in 7. Six patients were treated with a second-line immunosuppressive agent, and 6 received radiotherapy. The response to radiotherapy was generally poor.Conclusions: Idiopathic sclerosing orbital inflammation is a rare condition that can be difficult to diagnose and manage. Early intervention with immunosuppression in the form of corticosteroids combined with secondline agents can result in control and even regression of the disease.
Review of the literature shows that microcystic adnexal carcinoma affecting the orbital and periorbital tissues is difficult to differentiate clinically and microscopically from other conditions including squamous cell carcinoma. Eighty-one percent of cases that have histopathology checked in the initial assessment are still misdiagnosed. A high degree of suspicion is necessary if the lesion extends beyond an apparently adequate surgical margin, multiple recurrences occur, or superficial perineural spread is present. Mohs technique is the treatment of choice and may be improved if paraffin sections or immunohistocytochemistry are performed. Wide excision should be carried out once clear margins are obtained. Radiotherapy may be necessary in cases with multiple recurrences.
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