de Oliveira Vasconcelos Ricardo Anderson, Araújo Isaac Santos, Ferreira Luiz Miguel, Paiva João Paulo Gonçalves, de Andrade Maíra Medeiros Pacheco, Fernandes Igor, Abrahao-Machado Lucas Faria, Caro-Sánchez Claudia Haydee Sarai, Cano-Valdez Ana Cano, Mosqueda-Taylor Adalberto, Soares Ciro Dantas
Oral Diagnosis Department, Piracicaba Dental School, University of Campinas (UNICAMP), Avenida Limeira 901, Areião, Piracicaba, São Paulo, 13414-900, Brazil.
Private Pathology Service, Getúlio Sales Diagnósticos (GSD), Natal, Rio Grande do Norte, Brazil.
Head Neck Pathol. 2025 May 8;19(1):55. doi: 10.1007/s12105-025-01778-y.
This systematic review summarizes the current literature on Warthin-like mucoepidermoid carcinoma (WL-MEC), highlighting its clinicopathological, immunohistochemical and molecular characteristics.
This study followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines and was registered by PROSPERO (protocol CRD42024523598). Case reports and case series published in English on WL-MEC of major and minor salivary glands were included. The search was conducted in MEDLINE/PubMed, Scopus, Web of Science, and Embase databases in November 2024, along with a search in grey literature. Twenty-one articles met the eligibility criteria. The extracted information included clinicopathological data, immunohistochemical and molecular tests, treatment, recurrence, metastasis, follow-up, and status. The data analysis was performed descriptively, and presented as absolute and relative frequencies, and means or median for numerical variables.
78 WL-MEC cases were analyzed, with a female predominance observed and a wide age range, peaking in the sixth decade of life. Most cases involved the parotid gland and presented as indolent nodular masses, often asymptomatic. Histologically, the majority were low-grade tumors, with multicystic growth patterns and a lymphocytic infiltrate. Immunohistochemical analysis revealed consistent expression of p63, p40, CK5/6, and exhibited low Ki67 proliferation index (< 5%). MAML2 rearrangement was identified in all molecularly analyzed cases (n = 72/ 100%). Surgical treatment was the most common approach, which typically resulted in low recurrence rates, no distant metastasis, and favorable overall outcomes.
WL-MEC cases typically present as indolent, asymptomatic nodules in the parotid gland, often with a female predominance and a wide age range, peaking in the sixth decade of life. Surgery is the primary treatment and is associated with a favorable prognosis, characterized by low rates of recurrence and metastasis. These tumors commonly exhibit low-grade histopathological features, a multicystic pattern, and lymphocytic inflammatory infiltrate. The detection of MAML2 rearrangement, typically via FISH, is an essential diagnostic tool, emphasizing the molecular similarities between WL-MEC and conventional MEC.
本系统评价总结了目前关于沃辛样黏液表皮样癌（WL-MEC）的文献，重点阐述其临床病理、免疫组化及分子特征。
本研究遵循系统评价与Meta分析的首选报告项目指南，并在国际前瞻性系统评价注册库（PROSPERO，注册号CRD42024523598）进行了注册。纳入以英文发表的关于大、小涎腺WL-MEC的病例报告和病例系列。于2024年11月在MEDLINE/PubMed、Scopus、科学引文索引和Embase数据库中进行检索，并检索灰色文献。21篇文章符合纳入标准。提取的信息包括临床病理数据、免疫组化和分子检测、治疗、复发、转移、随访及现状。数据分析采用描述性方法，以绝对和相对频率以及数值变量的均值或中位数表示。
分析了78例WL-MEC病例，观察到女性占优势，年龄范围广，在60岁左右达到高峰。大多数病例累及腮腺，表现为惰性结节状肿块，通常无症状。组织学上，大多数为低级别肿瘤，呈多囊性生长模式并伴有淋巴细胞浸润。免疫组化分析显示p63、p40、CK5/6一致表达，Ki67增殖指数低（<5%）。在所有进行分子分析的病例（n = 72/100%）中均检测到MAML2重排。手术治疗是最常见的方法，通常复发率低，无远处转移，总体预后良好。
WL-MEC病例通常表现为腮腺内的惰性、无症状结节，女性常占优势，年龄范围广，在60岁左右达到高峰。手术是主要治疗方法，预后良好，其特点是复发和转移率低。这些肿瘤通常具有低级别组织病理学特征、多囊性模式和淋巴细胞炎性浸润。通过荧光原位杂交检测MAML2重排是一种重要的诊断工具，强调了WL-MEC与传统黏液表皮样癌之间的分子相似性。
