Schmack Ingo, Patel Rajiv M, Folpe Andrew L, Wojno Ted, Zaldivar Renzo A, Balzer Bonnie, Kang Shin J, Weiss Sharon W, Grossniklaus Hans E
Emory Eye Center, Emory University School of Medicine, Atlanta, GA, USA.
Am J Surg Pathol. 2007 Feb;31(2):193-8. doi: 10.1097/01.pas.0000213374.02171.0a.
Prolapse of subconjunctival intraconal orbital fat is a rare cause of an intraorbital mass lesion. Over the past several years, we have seen a number of cases in which this prolapsed fat was confused pathologically with a neoplasm of adipocytic lineage, specifically pleomorphic lipoma and atypical lipomatous neoplasm (well-differentiated liposarcoma). We report the clinical, histopathologic, and immunohistochemical findings in 21 specimens from 17 patients, all of whom presented with prolapsed intraconal orbital fat. All specimens were routinely examined and processed for light microscopy. Immunohistochemistry for CD34, CD68, S100 protein, vimentin, alpha-smooth muscle actin, and Ki-67, and Giemsa, Masson trichrome, and alcian blue histochemical stains were performed. Clinical and follow-up information was extracted from a chart review. The mean age (+/-SD) of the patients was 65.6+/-11.9 years (range: 41 to 85 y); 2 were women and 15 were men. Subconjunctival prolapsed orbital fat was localized in the superotemporal quadrant or lateral canthus around the rectus muscle below the lacrimal gland. The lesions were unilateral in 10 and bilateral in 7 patients. No recurrence was clinically evident over a mean (+/-SD) follow-up time of 2.5+/-3.2 years (range: 1 mo to 13.5 y). Histopathologically, all specimens showed an admixture of mature fat, fibrous septae lacking hyperchromatic cells, adipocytes with intranuclear vacuoles (Lochkern cells), multinucleated giant cells with a wreathlike configuration of normochromatic nuclei (floret cells), and varying numbers of histiocytes, lymphocytes, plasma cells, and mast cells. "Control" sections of normal orbital fat showed occasional Lochkern cells but lacked floret cells. By immunohistochemistry, the floret cells expressed only CD34 and vimentin, whereas the Lochkern cells expressed CD34, S100 protein, and vimentin. We conclude that subconjunctival herniated orbital fat commonly contains multinucleated floretlike giant cells, fibrous septae, and Lochkern cells, features that may result in diagnostic confusion with pleomorphic lipoma and atypical lipomatous neoplasms. Importantly, specific diagnostic features, such as aggregates of bland spindled cells associated with wiry collagen, as seen in pleomorphic lipoma, and enlarged hyperchromatic cells within fibrous septae, as in atypical lipomatous neoplasms, are entirely absent in herniated orbital fat. Multinucleated floret cells present in prolapsed orbital fat likely represent a reactive phenomenon, as they are not present in normal orbital fat.
眶内脂肪锥内结膜下脱垂是眶内肿块病变的罕见原因。在过去几年中,我们见到了一些病例,其中这种脱垂脂肪在病理上与脂肪细胞谱系的肿瘤相混淆,特别是多形性脂肪瘤和非典型脂肪瘤性肿瘤(高分化脂肪肉瘤)。我们报告了17例患者21份标本的临床、组织病理学和免疫组化结果,所有患者均表现为眶内脂肪锥内脱垂。所有标本均常规检查并处理以进行光学显微镜检查。进行了CD34、CD68、S100蛋白、波形蛋白、α平滑肌肌动蛋白和Ki-67的免疫组化,以及吉姆萨、马松三色和阿尔辛蓝组织化学染色。临床和随访信息从病历回顾中提取。患者的平均年龄(±标准差)为65.6±11.9岁(范围:41至85岁);2例为女性,15例为男性。结膜下脱垂的眶脂肪位于颞上象限或泪腺下方直肌周围的外眦。10例病变为单侧,7例为双侧。在平均(±标准差)2.5±3.2年(范围:1个月至13.5年)的随访时间内,临床上无复发迹象。组织病理学上,所有标本均显示成熟脂肪、缺乏核深染细胞的纤维间隔、有核内空泡的脂肪细胞(Lochkern细胞)、核染色正常呈花环样排列的多核巨细胞(小花细胞)以及数量不等的组织细胞、淋巴细胞、浆细胞和肥大细胞混合存在。正常眶脂肪的“对照”切片可见偶尔的Lochkern细胞,但无小花细胞。免疫组化显示,小花细胞仅表达CD34和波形蛋白,而Lochkern细胞表达CD34、S100蛋白和波形蛋白。我们得出结论,结膜下疝出的眶脂肪通常含有多核小花样巨细胞、纤维间隔和Lochkern细胞,这些特征可能导致与多形性脂肪瘤和非典型脂肪瘤性肿瘤的诊断混淆。重要的是,特定的诊断特征,如多形性脂肪瘤中所见的与细胶原纤维相关的温和梭形细胞聚集,以及非典型脂肪瘤性肿瘤中纤维间隔内增大的核深染细胞,在疝出的眶脂肪中完全不存在。脱垂眶脂肪中存在的多核小花细胞可能代表一种反应性现象,因为它们在正常眶脂肪中不存在。