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肺部涎腺肿瘤的最新研究进展。

Recent updates in salivary gland tumors of the lung.

机构信息

Department of Laboratory Medicine and Pathology, Mayo Clinic Rochester, MN, United States.

出版信息

Semin Diagn Pathol. 2021 Sep;38(5):98-108. doi: 10.1053/j.semdp.2021.03.001. Epub 2021 Mar 11.

Abstract

Salivary gland tumors are uncommon primary lesions in the lung. Their morphologic, immunophenotypic, and molecular characteristics resemble those of their counterparts in the head and neck or elsewhere. Most common primary pulmonary salivary gland tumors include mucoepidermoid carcinoma, adenoid cystic carcinoma, and epithelial-myoepithelial carcinoma. The study of these neoplasms is hampered by their paucity. Therefore, studies are in general small or restricted to individual cases. Despite this challenge recent advances have been made specifically at the molecular level. Molecular alterations such as MAML2 rearrangements in mucoepidermoid carcinoma, MYB rearrangements in adenoid cystic carcinomas, and EWSR1 rearrangements in hyalinizing clear cell carcinomas and myoepithelial tumors have been identified. These molecular alterations might be helpful in the distinction of these salivary gland tumors from other neoplasms in the lung. However, the distinction from metastatic disease remains challenging. Awareness of these tumors and knowledge of available ancillary studies to confirm the diagnosis is important to avoid misdiagnosis which might lead to differences in treatment, management, and prognosis. Further studies are needed to identify biomarkers to better predict patient's outcome and for individual management and treatment of patients.

摘要

唾液腺肿瘤是肺部少见的原发性病变。它们的形态学、免疫表型和分子特征与头颈部或其他部位的相应肿瘤相似。最常见的原发性肺唾液腺肿瘤包括黏液表皮样癌、腺样囊性癌和上皮-肌上皮癌。由于这些肿瘤的罕见性,对这些肿瘤的研究受到了阻碍。因此,研究通常规模较小或仅限于个别病例。尽管存在这一挑战,但最近在分子水平上取得了进展。已经确定了黏液表皮样癌中的 MAML2 重排、腺样囊性癌中的 MYB 重排以及透明细胞癌和肌上皮肿瘤中的 EWSR1 重排等分子改变。这些分子改变可能有助于将这些唾液腺肿瘤与肺部的其他肿瘤区分开来。然而,与转移性疾病的区分仍然具有挑战性。了解这些肿瘤以及可用于确认诊断的辅助研究对于避免误诊至关重要,误诊可能会导致治疗、管理和预后的差异。需要进一步的研究来确定生物标志物,以更好地预测患者的预后,并为患者的个体化管理和治疗提供依据。

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