线状皮脂腺痣综合征(linear nevus sebaceous syndrome，LNSS)是一种以皮脂腺痣(nevus sebaceous，NS)为特征性改变，同时合并癫痫、智力迟钝、神经缺陷或骨骼畸形等病变的疾病。本文报道1例经病理组织学检查确诊的LNSS患者，同时伴有双眼脉络膜骨瘤和脑部先天发育异常。由于线状皮脂腺综合征伴双眼多发异常较为少见，本文将总结该例患者的临床和病理表现，旨在为临床诊疗提供一定参考资料。
Linear nevus sebaceous syndrome (LNSS) is a disease characterized by nevus sebaceous (NS) and accompanied by epilepsy, mental retardation, nerve defect or skeletal deformity. We report a case of linear sebaceous nevus syndrome diagnosed by histopathological examination with bilateral choroidal osteoma and congenital developmental abnormalities of the brain. Since linear sebaceous gland syndrome with binocular abnormalities is relatively rare, this paper will summarize the clinical and pathological manifestations of this patient, aiming to provide certain reference for clinical diagnosis and treatment.
目的：研究泪腺腺样囊性癌不同病理组织学分型和分级间骨质破坏、神经侵犯和预后的差异。方法：回顾性分析2010年4月至2019年4月首都医科大学附属北京同仁医院眼肿瘤科收治并经病理组织学检测确诊的30例泪腺腺样囊性癌患者的完整病历资料。分析患者的一般病历资料、医学影像学资料、病理组织学检查结果以及预后情况等，并系统研究泪腺腺样囊性癌病理组织学分型和分级与患者预后的关系。结果：30例患者中，病理组织学分型为筛状型9例(30.0%)，实体型10例(33.3%)，混合型11例(36.7%)，其中实性和腺管混合型4例(13.3%)、实性和筛状混合型4例(13.3%)、筛状和腺管混合型3例(10.0%)。病理组织学分级为I级12例(40.0%)；II级4例(13.3%)；III级14例(46.7%)。不同病理组织学分型肿瘤骨质破坏发生率差异有统计学意义(P=0.046)；不同病理组织学分级肿瘤骨质破坏发生率差异无统计学意义(P=0.513)。不同病理组织学分型与分级肿瘤神经侵犯、远处转移发生均无相关性(均P>0.05)。在行手术联合放射治疗后，不同病理组织学分型肿瘤的无复发率差异有统计学意义(P<0.05)；实体型的无复发率在36个月内最低(P=0.037)；而病理学分级与手术联合放射治疗后的无复发率无关(P=0.059)。结论：泪腺腺样囊性癌病理组织学分型与骨质破坏发生率和复发率显著相关，其中实体型肿瘤较易复发。而病理学分型与神经侵犯和远处转移发生率无关。病理组织学分级与骨质破坏、神经侵犯、远处转移和复发率无显著相关性。
Objective: To investigate the differences in bone destruction, nerve invasion, and prognosis of lacrimal gland adenoid cystic carcinoma (LGACC) among different histologic types and grades. Methods: A retrospective analysis was performed on 30 cases of lacrimal gland adenoid cystic carcinoma confirmed by histopathology who were admitted to the Department of Ophthalmic Oncology, Beijing Tongren Hospital, Capital Medical University from April 2010 to April 2019. The general data, imaging findings, histological examination and prognosis were collected and analyzed, with the focus on the relationship between the histological characteristics and the prognosis of lacrimal gland adenoid cystic carcinoma. Results: Among the 30 patients, 9 cases of cribriform type (30.0%); 10 cases of solid type (33.3%); 11 cases of mixed type (36.7%), including 4 cases of solid and glandular (13.3%), 4 cases of solid and cribriform (13.3%), 3 cases of cribriform and glandular (10.0%). The histopathological grade was I in 12 cases (40.0%), II in 4 cases (13.3%) and III in 14 cases (46.7%). There was statistical difference in the incidence of bone destruction among different histological types (P=0.046). There was no significant difference in the incidence of bone destruction among different histological grades (P=0.513).There was no significant difference between different histological grades and types and the incidence of nerve invasion and distant metastasis (all P>0.05). After surgery combined with radiotherapy, there was a statistical difference in the recurrence-free rate of different histological types, and the recurrence-free rate of solid type was the lowest within 36 months (P=0.037). Histological grade was not associated with recurrence-free rate after surgery combined with radiotherapy (P=0.059). Conclusion: The histological type of adenoid cystic carcinoma of lacrimal gland was significantly correlated with the incidence of bone destruction and recurrence rate, in which solid type was more likely to relapse. And the histological type was not associated with the incidence of nerve invasion or distant metastasis. Histopathological grade was not significantly associated with the rate of bone destruction, nerve invasion, distant metastasis, and recurrence.
线状皮脂腺痣综合征(linear nevus sebaceous syndrome，LNSS)是一种以皮脂腺痣(nevus sebaceous，NS)为特征性改变，同时合并癫痫、智力迟钝、神经缺陷或骨骼畸形等病变的疾病。本文报道1例经病理组织学检查确诊的LNSS患者，同时伴有双眼脉络膜骨瘤和脑部先天发育异常。由于线状皮脂腺综合征伴双眼多发异常较为少见，本文将总结该例患者的临床和病理表现，旨在为临床诊疗提供一定参考资料。
Linear nevus sebaceous syndrome (LNSS) is a disease characterized by nevus sebaceous (NS) and accompanied by epilepsy, mental retardation, nerve defect or skeletal deformity. We report a case of linear sebaceous nevus syndrome diagnosed by histopathological examination with bilateral choroidal osteoma and congenital developmental abnormalities of the brain. Since linear sebaceous gland syndrome with binocular abnormalities is relatively rare, this paper will summarize the clinical and pathological manifestations of this patient, aiming to provide certain reference for clinical diagnosis and treatment.
