Departments of Ophthalmology.
Pathology, Emory University School of Medicine, Atlanta, Georgia, U.S.A.
Ophthalmic Plast Reconstr Surg. 2021;37(3S):S134-S140. doi: 10.1097/IOP.0000000000001804.
The authors present 3 patients from this retrospective case series to review the clinical findings, imaging, pathology, and treatment of orbital atypical lipomatous tumor/well-differentiated liposarcoma. Pathology of biopsy specimens ranged from spindle cell proliferations mimicking neurofibroma to proliferations of well-differentiated adipocytes. Immunohistochemical stains were positive for murine double minute 2 in 1 case, and fluorescent in situ hybridization showed amplification of murine double minute 2 in 2 cases. Treatments ranged from serial debulking, proton beam irradiation, and exenteration. None of the patients developed metastases. A literature review supported the low-grade nature of this lesion. Orbital atypical lipomatous tumor/well-differentiated liposarcoma is a low-grade, indolent liposarcoma that may be locally invasive. The histologic diagnosis is enhanced with immunohistochemical staining for murine double minute 2 and fluorescent in situ hybridization analysis for amplification of murine double minute 2. Although treatment may vary according to the individual, conservative therapies may be attempted prior to radical surgery.
作者从这一回顾性病例系列中介绍了 3 名患者，以回顾眶部非典型脂肪肉瘤/高分化脂肪肉瘤的临床发现、影像学表现、病理学和治疗。活检标本的病理学表现从类似于神经纤维瘤的梭形细胞增生到分化良好的脂肪细胞增生不等。1 例病例的鼠双微体 2 免疫组织化学染色阳性，2 例病例的荧光原位杂交显示鼠双微体 2 扩增。治疗方法包括连续减瘤、质子束放疗和眶内容剜除术。没有患者发生转移。文献复习支持该病变的低度恶性。眶部非典型脂肪肉瘤/高分化脂肪肉瘤是一种低度恶性、惰性的脂肪肉瘤，可能具有局部侵袭性。鼠双微体 2 的免疫组织化学染色和鼠双微体 2 扩增的荧光原位杂交分析有助于提高组织学诊断。尽管治疗方法可能因个体而异，但在进行根治性手术之前，可能可以尝试保守治疗。
