Manjandavida Fairooz P, Chahar Shaifali, Dave Brijal
Department of Oculoplasty, Orbit and Ocular Oncology, HORUS Specialty Eye Care , Bangalore , India.
Department of Pathology, Majumdar Shaw Cancer Center, Narayana Health City , Bangalore , India.
Orbit. 2019 Oct;38(5):401-406. doi: 10.1080/01676830.2018.1538381. Epub 2018 Nov 29.
A 19-year-old female student presented with painless, progressive proptosis in the right eye for the past six months causing significant cosmetic blemish and double vision. Clinical evaluation revealed normal visual acuity, normal pupillary reflex, severe proptosis, and dystopia. Orbital imaging revealed a heterogeneous mass in superior orbit displacing the globe inferiorly and causing extensive bony erosion of frontal bone superiorly and invading the frontal sinus. Excision biopsy was performed with en-bloc removal of the tumor. Surgical delivery of a slimy mass with infiltrated bony tissue led to a presumptive diagnosis of myxoid liposarcoma. Careful histopathologic analysis coupled with immunohistochemical studies were performed which established the rare diagnosis of low-grade myxoid spindle cell neoplasm with features suggestive of intraosseous myxoma. On follow-up at 15 months, our patient revealed no signs of recurrence. Isolated intraosseous orbital myxoma is a rare entity; only two such cases have been reported in the literature.
一名19岁女学生在过去6个月中出现右眼无痛性、进行性眼球突出,造成明显的外貌瑕疵和复视。临床评估显示视力正常、瞳孔反射正常、严重眼球突出和眼球移位。眼眶成像显示眶上部有一不均匀肿块,将眼球向下推移,导致额骨上部广泛骨质侵蚀并侵犯额窦。进行了切除活检,整块切除肿瘤。手术取出一个伴有浸润性骨组织的黏液性肿块,初步诊断为黏液样脂肪肉瘤。进行了仔细的组织病理学分析并结合免疫组化研究,确诊为罕见的低度黏液样梭形细胞瘤,具有骨内黏液瘤的特征。在15个月的随访中,患者未出现复发迹象。孤立性骨内眼眶黏液瘤是一种罕见的疾病;文献中仅报道过两例此类病例。