泪腺腺样囊性癌临床诊疗中需关注的问题

Clinical Diagnosis and Treatment of Lacrimal Gland Adenoid Cystic Carcinoma

泪腺腺样囊性癌(LGACC)是泪腺最常见的原发性恶性上皮肿瘤,具有复发率高、嗜神经生长和易远处转移的特点。临床表现以泪腺区肿块为主,但特异性欠佳;MRI和CT结合在诊断LGACC中具有重要价值,PET/CT在确定LGACC性质和转移等方面具有重要作用。病理组织学检查结果是确诊的金标准,病理组织学类型对于判断患者的预后较为重要。目前治疗LGACC最常用的方案为手术切除联合术后局部放疗,因其发病机制尚不明确,积极治疗后总体预后仍然较差,故本文将结合相关文献及临床诊疗经验,从LGACC的流行病学、临床表现、影像学检查、病理组织学改变、治疗及预后等方面加以述评,期望能够提高其诊疗质量、改善患者的预后。

Lacrimal gland adenoid cystic carcinoma(LGACC)is the most common primary malignant epithelial tumor of the lacrimal gland and has the characteristics of high recurrence rate,neurotropic growth,and risk of distant metastasis.The clinical manifestation is mainly a mass in the lacrimal gland area with a poor specificity.The combination of MRI and CT is important in the diagnosis of LGACC,and PET/CT is of great value in determining the nature and metastasis of LGACC.Histopathological examination is the gold standard for diagnosis,and the type of histopathology is significant for predicting the prognosis of patients.At present,the most commonly used treatment for LGACC is surgical resection combined with postoperative local radiotherapy.However,the overall prognosis is poor after active treatment because the pathogenesis of LGACC remains unclear.This article will combine relevant literature and the author’s clinical experience to review the epidemiology,clinical manifestations,imaging examination,histopathological changes,treatment,and prognosis of LGACC.Results will contribute to improve the quality of diagnosis and treatment and the prognosis of patients.