■Warthin样粘膜表皮样癌(MEC)是MEC的一种新的和罕见的形态学变体,文献中只有少数病例报告。临床病理,Warthin样MEC的分子特征和生物行为尚未得到广泛研究。我们重新评估在我们医院诊断和治疗的所有类似Warthin的MEC患者。
■患者特征,包括临床病理特征,遗传畸变,治疗,和预后信息进行评估和评价。
■确定了29名类似Warthin的MEC患者,19例患者为女性(65.5%),男性10人(34.5%)。患者年龄差异很大,从8岁到68岁(平均42.3岁)。在所有Warthin样MEC患者中检测到MAML2重排的遗传畸变,这表明这种遗传事件是Warthin样MEC的独特特征。25例患者(86.2%)被评估为患有低级疾病(I/II),和四个(13.8%)为高临床阶段疾病(III/IV)。超过一半的患者(16/29)仅接受了部分唾液腺切除术;2例患者接受了扩展的唾液腺切除术,11例患者接受了扩大的唾液腺切除术和颈淋巴结清扫术。中位随访时间为73个月(5-128个月),28例患者在随访期结束时均未复发,1例患者术后1年因肺转移死亡.
■我们的数据表明,大多数类似Warthin的MEC表现出轻度的临床病理过程和较少侵袭性的生物行为,侵略性的生物行为似乎非常罕见。此外,在唾液腺中,MAML2重排似乎是唾液Warthin样MEC的独特分子特征。
UNASSIGNED: Warthin-like Mucoepidermoid carcinoma (MEC) is a new and rare morphological variant of MEC, with only a few case reports in the literature. The clinicopathological, molecular features and bio-behaviors of Warthin-like MEC has not been studied extensively. We reappraisal all Warthin-like MEC patients diagnosed and treated at our hospital.
UNASSIGNED: Patient characteristics including clinicopathological features, genetic aberrations, treatment, and prognostic information were assessed and evaluated.
UNASSIGNED: Twenty-nine Warthin-like MEC patients were identified, 19 patients were female (65.5 %), and 10 were male (34.5 %). The patients\' age varied widely from 8 to 68 years (mean 42.3 years). Genetic aberrations of MAML2 rearrangement were detected in all Warthin-like MEC patients, which suggesting this genetic event is the unique feature of Warthin-like MEC. Twenty-five patients (86.2 %) were assessed as having a low-stage disease (I/II), and four (13.8 %) as having high-clinical stage disease (III/IV). More than half of the patients (16/29) underwent only partial sialoadenectomy; 2 patients underwent extended sialoadenectomy, and 11 patients underwent extended sialoadenectomy with cervical lymph node dissection. After a median follow-up time of 73 months (5-128 months), Twenty-eight patients were alive without recurrence at the end of the follow-up period, one patient died 1 year after surgery due to lung metastasis.
UNASSIGNED: Our data suggested that most Warthin-like MEC exhibited mild clinicopathological course and less aggressive bio-behavior, and an aggressive bio-behavior seemed to be very rare. In addition, in the salivary gland, MAML2 rearrangement seems to be a unique molecular feature of salivary Warthin-like MEC.