Key result
Multimodal treatment including surgery and adjuvant radiotherapy resulted in no recurrences or metastases among 9 patients with orbital alveolar soft-part sarcoma.
Observational (n=9)
Early diagnosis and a multidisciplinary approach including adjuvant radiotherapy may prevent recurrence and metastases in orbital alveolar soft-part sarcoma.
May support multimodal therapy in orbital ASPS; leaves open need for prospective validation in larger cohorts.
AIM: To study the clinico-radiological and clinic-pathological features of orbital alveolar soft-part sarcomas (ASPS). MATERIALS AND METHODS: We retrieved 9 cases of orbital ASPS. Histopathological and immunohistochemical features and clinico-radiological findings were evaluated. RESULTS: Age at presentation varied from 1 to 31 years. Both, children and adults were affected. Proptosis was the most common clinical presentation. PAS-positive diastase-resistant crystals and granules were found in all cases. All patients received adjuvant radiotherapy. Two cases had positive surgical margins and were treated with additional adjuvant chemotherapy. None of the patients had recurrence or metastases till the last follow up. CONCLUSION: Early diagnosis with a multidisciplinary, multimodal approach of treatment is quintessential in achieving a long disease-free survival in patients with ASPS.
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Honavar et al. (2014) conducted an observational in Orbital alveolar soft-part sarcoma (n=9). Multimodal treatment (surgery, adjuvant radiotherapy, and chemotherapy) was evaluated on Recurrence or metastases. Multimodal treatment including surgery and adjuvant radiotherapy resulted in no recurrences or metastases among 9 patients with orbital alveolar soft-part sarcoma.
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