RESUMEN
PURPOSE: To determine the effect of a staged procedure in the treatment of primary lacrimal sac epithelial malignancy. METHODS: This is a retrospective case series of 18 consecutive patients with primary lacrimal sac epithelial malignancy treated at an orbital tumor referral center between 2002 and 2017. Study was conducted in compliance with the Declaration of Helsinki. All patients underwent biopsy of the mass to confirm the diagnosis pathologically. Chemotherapy concurrent with radiotherapy was delivered to the patients to reduce and concrete the tumor prior to surgery. En bloc resection of the lacrimal sac malignancy and nasolacrimal duct was followed. RESULTS: Eleven patients were male and 7 patients were female. The median follow-up time was 72.2 months. Nine patients had squamous cell carcinoma, 7 poorly differentiated carcinoma, 1 transitional cell carcinoma, and 1 adenoid cystic carcinoma. After chemotherapy and radiotherapy, the tumor volume was reduced significantly (p < 0.0001). En bloc resection of the lacrimal sac malignancy was performed in all patients with concurrent partial ethmoidectomy in 8 patients and medial maxillectomy in 5 patients. One patient (5.6%) suffered from adenoid cystic carcinoma died of metastatic disease. Two patients (11.1%) with local recurrence received reoperation, and 1 patient (5.6%) with pulmonary metastasis received gamma knife radiosurgery. These patients are alive with no evidence of tumor. Other patients are alive without evidence of disease at last follow up. No patient had new onset of lymph node enlargement during and after the treatment. CONCLUSIONS: The staged procedure is a promising method for the treatment of primary lacrimal sac epithelial malignancy with no postoperative lymph node metastasis.