Clinical and pathologic profile of angiomyxomas of the orbit.

Purpose: To describe clinical, imaging, and histopathologic features of angiomyxomas of the orbit, which are extremely rare tumors in the orbit.
Methods: A retrospective review of clinical case records and imaging findings of histopathologically diagnosed cases of angiomyxoma over a period of 8 years (2001-2008). The histopathologic features were studied by routine hematoxylin-eosin staining, special stain (Alcian blue), and immunohistochemistry.
Results: Four cases (2 male and 2 female) with a mean age of 35 years (range, 28-40 years) were diagnosed as angiomyxoma. Two of these were angiomyxomas, and 2 were aggressive angiomyxomas. All patients presented with gradual progressive proptosis. The mean duration of symptoms was 10.5 months (range, 5-24 months). There was associated reduction in the visual acuity in 2 cases. The superior orbit was involved in all 4 patients. CT scan showed a heterogeneously enhancing irregular mass confined to the superior orbit. Surgical removal of the mass was performed in all 4 cases. On follow-up, there was recurrence at 6 months in 1 case, which was histopathologically diagnosed as an angiomyxoma.
Conclusions: Angiomyxoma is an extremely rare, locally aggressive orbital tumor, occurring in the third to fourth decade of life. Complete excision is the treatment of choice. These tumors are likely to have a recurrence due to their infiltrative growth and require long-term follow-up.