Lacrimal gland pleomorphic adenoma: a narrative review

ABSTRACT We present a literature review of 57 publications describing this pathology, published from the year 2012. In all these studies patients were reported to depict a slow-growing, motionless mass, which is painless at most times. All cases were managed by total excision, except for one report where adjuvant radiotherapy was applied. Among the several therapeutic strategies, the total tumor resection, preserving the tumor pseudocapsule intact, appears to be a consensus in treating the disease efficiently. Furthermore, fine-needle aspiration biopsy, including the assessment of genetic alterations, has proved to be a valuable tool in the diagnosis of challenging cases. Our literature survey also suggests that an incisional biopsy before the surgery may lead to the pseudocapsule disruption, thus considerably increasing the chances of adenoma recurrence, enabling its malignization. At present, genetics studies indicate that the molecular aberrations involved in the adenoma are similar to those represented in the salivary gland tumor pathogenesis. Further, in the recurrent cases, the pathology becomes difficult to treat and multiple surgeries may be required, occasionally, leading to radical surgery treatment.

摘要 我们对2012年以来发表的57篇描述该病变的文献进行了综述。所有纳入研究均显示，患者表现为生长缓慢、无活动度的肿块，且多数情况下无疼痛感。除1篇文献采用辅助放疗（adjuvant radiotherapy）治疗外，其余所有病例均行完整切除术。在多种治疗策略中，完整切除肿瘤并保持其假包膜（tumor pseudocapsule）完整的方案，已成为公认的高效治疗该疾病的共识方式。此外，包括基因变异检测在内的细针穿刺活检（fine-needle aspiration biopsy），已被证实为诊断疑难病例的有效手段。本次文献调研还发现，术前实施切开活检（incisional biopsy）可能导致肿瘤假包膜破裂，进而显著增加腺瘤复发（adenoma recurrence）风险，并可能诱发恶变。目前的遗传学研究表明，该腺瘤相关的分子畸变与唾液腺肿瘤（salivary gland tumor）发病机制中涉及的分子畸变具有相似性。进一步而言，复发病例的治疗难度显著提升，往往需要多次手术，有时甚至需采取根治性手术治疗。