Purpose: To describe the clinic-radiological, pathological profile, and management outcomes of primary alveolar soft-part sarcoma (ASPS) of the orbit. Methods: A retrospective analysis of all histopathologically proven cases of orbital ASPS that presented between May 2016 and September 2019 was done. Data collected included demographics, clinical features, imaging characteristics, metastatic workup, management, and follow-up. Results: Five patients, of which four were males, presented to us during the study period. The mean age of presentation was 12.6 years (range 3–22 years). The most common presenting features were abaxial proptosis ( n = 4) and diminished vision ( n = 4). Imaging showed a well-defined orbital mass in all patients with internal flow voids in three. Preoperative percutaneous embolization with cyanoacrylate glue was done in these three patients owing to high vascularity. Four patients underwent complete tumor excision. One patient underwent exenteration. Histopathology showed polygonal tumor cells arranged in a pseudo-alveolar pattern and Periodic Acid-Schiff (PAS) positive crystals in the cytoplasm in all patients. One patient had systemic metastasis at presentation and developed a local recurrence after 3 months. No recurrence or metastasis was noted in the remaining four patients at a mean final follow-up of 11.2 months (range 5–15 months). Conclusion: ASPS is a rare orbital neoplasm that poses a diagnostic and therapeutic challenge. Imaging might show a soft-tissue tumor with high vascularity. Multidisciplinary management with interventional radiologists for preoperative embolization of vascular lesions helps minimize intraoperative bleeding and aids in complete tumor resection. A localized orbital disease carries a better prognosis.
PURPOSE: To analyze the correlation between patient-reported regurgitation on pressure over lacrimal sac (ROPLAS) with the physician's examination in diagnosing primary-acquired nasolacrimal duct obstruction (PANDO). METHODS: A cross-sectional study was done over 5 months (March–July 2018). All cases diagnosed as PANDO were included in the study. The maneuver of pressing over the lacrimal sac area and noticing the egress of mucoid or clear fluid from the surrounding area by the patient was termed as self-ROPLAS. A specific patient history of performance of this maneuver was compared with a clinician-performed ROPLAS and subsequent objective lacrimal drainage evaluation. The various reasons for performing self-ROPLAS by the patients were documented. RESULTS: A total of 134 patients were included in the study, out of which 59 (44.02%) were males and 75 (55.9%) were females. History of self-ROPLAS was present in 64 (47.8%) of the patients, whereas the physician examination revealed ROPLAS to be positive in 92 (68.6%) of the patients. All patients (100%) with a positive history of self-ROPLAS had nasolacrimal duct obstruction on subsequent examination. The most common reason for performing self-ROPLAS was for emptying the discharge from the medial canthal region to reduce the painless swelling. CONCLUSION: Self-ROPLAS is highly suggestive of an obstructed nasolacrimal duct and can be used as a screening tool by the primary physician to triage the patients toward ophthalmic plastic clinics or consult.
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