Papalas John A, Proia Alan D, Hitchcock Mike, Gandhi Parag, Cummings Thomas J
Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA.
Am J Dermatopathol. 2010 Jun;32(4):374-9. doi: 10.1097/dad.0b013e3181beaac7.
Neurothekeoma palpebrae is the diagnostic term used to describe nerve sheath myxoma or neurothekeoma of the eyelid. Although these tumors are not uncommonly found in the head and neck region, eyelid involvement is very uncommon. We present 2 cases of cellular neurothekeoma and 1 case of nerve sheath myxoma occurring in the eyelid. Patient 1 was a 13-year-old girl with a left upper eyelid lesion of 6-months duration. Microscopic examination disclosed a cellular neurothekeoma with mild myxoid change and osteoclast-like multinucleated cells. Tumor cells infiltrated the orbicularis oculi muscle. The tumor cells were immunopositive for NKI.C3, CD34, and focally for S-100 protein. Multinucleated cells were reactive to CD68. Mart-1, smooth muscle actin, CD31, keratin, desmin, myogenin, synaptophysin, and neurofilament protein (NFP) were negative. Patient 2 was a 4-year-old girl with a left upper eyelid lesion diagnosed clinically as a chalazion. The lesion was incised. Five months later, the patient returned with a firm 5.5-mm nodule at the site. Excision revealed cellular neurothekeoma invading the orbicularis oculi. Tumor cells were NKI.C3 immunopositive and S-100 protein negative, and the multinucleated cells were CD68 positive. Patient 3 was a 70-year-old woman with a 10-year history of a subcutaneous right lower eyelid nodule which had recently changed color. The excised specimen demonstrated a hypocellular, well-circumscribed myxoid tumor surrounded by a thin rim of fibrous connective tissue. Tumor cells contained moderate amounts of eosinophilic cytoplasm with irregular, hyperchromatic nuclei. Nucleoli and mitotic figures were not apparent. Tumor cells were NKI.C3 negative, and S100 protein and glial acidic fibrillary protein positive, consistent with a nerve sheath myxoma. Including our 3 cases, 10 cases of nerve sheath myxoma and neurothekeoma occurring in the eyelid have been reported the English language literature. Although uncommon, these lesions should be kept in the differential diagnosis of eyelid tumors that can masquerade as chalazia.
眼睑神经鞘黏液瘤是用于描述眼睑神经鞘黏液瘤或神经鞘瘤的诊断术语。尽管这些肿瘤在头颈部并不罕见，但累及眼睑的情况非常少见。我们报告2例发生于眼睑的细胞性神经鞘瘤和1例神经鞘黏液瘤。病例1为一名13岁女孩，左上眼睑肿物已有6个月。显微镜检查显示为细胞性神经鞘瘤，伴有轻度黏液样改变和破骨细胞样多核细胞。肿瘤细胞浸润眼轮匝肌。肿瘤细胞对NKI.C3、CD34呈免疫阳性，对S-100蛋白呈局灶性阳性。多核细胞对CD68呈反应性。Mart-1、平滑肌肌动蛋白、CD31、角蛋白、结蛋白、肌细胞生成素、突触素和神经丝蛋白（NFP）均为阴性。病例2为一名4岁女孩，左上眼睑肿物临床诊断为睑板腺囊肿。该肿物被切除。5个月后，患者因原部位出现一个坚硬的5.5毫米结节而复诊。切除标本显示为细胞性神经鞘瘤侵犯眼轮匝肌。肿瘤细胞对NKI.C3呈免疫阳性，对S-100蛋白呈阴性，多核细胞对CD68呈阳性。病例3为一名70岁女性，右下眼睑皮下结节已有10年病史，近期颜色发生改变。切除标本显示为一个细胞稀少、边界清楚的黏液样肿瘤，周围有一层薄的纤维结缔组织包绕。肿瘤细胞含有中等量嗜酸性细胞质，细胞核不规则、深染。核仁和有丝分裂象不明显。肿瘤细胞对NKI.C3呈阴性，对S100蛋白和胶质酸性纤维蛋白呈阳性，符合神经鞘黏液瘤。包括我们的3例病例在内，英文文献中共报道了10例发生于眼睑的神经鞘黏液瘤和神经鞘瘤。尽管罕见，但在鉴别诊断可伪装为睑板腺囊肿的眼睑肿瘤时应考虑到这些病变。
