Doyle Madeline, Odashiro Alexandre N, Pereira Patrícia R, Odashiro Macanori, Kobayashi Fernando, Shinzato Ilton, Burnier Miguel N
Henry C. Witelson Ocular Pathology Laboratory, McGill University, Montreal, Quebec, Canada.
Orbit. 2012 Jun;31(3):168-70. doi: 10.3109/01676830.2011.648806.
To our knowledge, pleomorphic liposarcoma (PL) of the orbit has only been reported in the literature four times. This rarity makes it more difficult to diagnose and to treat in this clinical setting. A 62-year-old female presented with pruritus, edema, proptosis and diplopia 5 months OS. Imaging revealed an intraorbital mass displacing the globe, with infiltration into the sinus. The tumor was removed and the histological examination revealed a highly cellular tumor with heterogenous histology, with a few vacuolated cells and many malignant features. Immunohistochemistry allowed for the differential diagnosis, resulting in a diagnosis of PL of the orbit. The cells were immuno-positive for S-100 and negative for all other relevant markers. According to the literature, prognosis for this neoplasm is quite poor, and exenteration represents the best treatment option. The patient refused exenteration and radiation therapy, however, at 2 year follow-up, she remained recurrence-free.
据我们所知，眼眶多形性脂肪肉瘤（PL）在文献中仅被报道过四次。这种罕见性使得在这种临床情况下更难以诊断和治疗。一名62岁女性在出现症状5个月后出现瘙痒、水肿、眼球突出和复视。影像学检查显示眶内肿块使眼球移位，并侵入鼻窦。肿瘤被切除，组织学检查显示为高度细胞性肿瘤，组织学异质性，有一些空泡化细胞和许多恶性特征。免疫组织化学有助于鉴别诊断，最终诊断为眼眶PL。细胞S-100免疫阳性，所有其他相关标志物均为阴性。根据文献，这种肿瘤的预后相当差，而眼眶内容剜除术是最佳治疗选择。然而，患者拒绝了眼眶内容剜除术和放射治疗，在2年的随访中，她没有复发。
