腮腺嗜酸细胞型黏液表皮样癌2例临床病理报告及文献回顾OA
Oncocytic mucoepidermoid carcinoma of the parotid gland:a clinicopathological report of two cases and lit-erature review
目的 探讨腮腺嗜酸细胞型黏液表皮样癌(OMEC)的临床病理特征及诊疗方案,以提高临床及病理医师对该罕见变异型黏液表皮样癌的认识.方法 回顾性分析2例发生于腮腺的OMEC患者的临床资料、影像学表现、组织病理学形态、免疫表型与分子特征,并复习相关文献.结果 病例1,男,50岁,因右耳垂后无痛性肿物2年余就诊.行保留面神经的右腮腺扩大切除术.病理示肿瘤以嗜酸细胞为主,含少量黏液细胞;免疫组化示细胞角蛋白5/6、细胞角蛋白7、P63部分阳性;阿利新蓝、过碘酸Schiff及磷钨酸苏木素特殊染色阳性,确诊为右腮腺OMEC,随访1年未见复发转移.病例2,男,61岁,因左耳下肿物3个月就诊.外院行腮腺部分切除术后于吉林大学口腔医院病理会诊,示肿瘤细胞嗜酸细胞占比近100%,呈浸润性生长,且缺乏典型的黏液细胞、表皮样细胞及中间细胞.经荧光原位杂交检测证实 mastermind 样转录共激活因子 2(MAML2)基因断裂阳性,确诊为左腮腺OMEC.后续行保留面神经的腮腺全切除术,短期随访3个月无复发.文献回顾显示,OMEC多发生于腮腺,属低级别肿瘤,预后总体良好.当肿瘤完全由嗜酸细胞组成、缺乏黏液表皮样癌典型细胞特征且异型性低时,极易与嗜酸性腺瘤、结节性嗜酸细胞增生等良性嗜酸性病变混淆.其鉴别诊断依赖于浸润性生长的形态学特征、阳性免疫表型(如P63)以及特征性的MAML2基因重排检测.治疗首选根治性手术切除.结论 以嗜酸细胞为主的OMEC具有较高的临床误诊风险.结合病理形态学浸润特征、免疫组化及MAML2分子检测,是避免误诊、准确判断肿瘤性质并指导后续手术决策的关键.
Objective To investigate the clinicopathological characteristics and diagnostic-therapeutic strategies of oncocytic mucoepidermoid carcinoma(OMEC)of the parotid gland,and to enhance awareness of this rare variant among clinicians and pathologists.Methods The clinical data,imaging findings,histopathological features,immuno-phenotype,and molecular characteristics of two patients with parotid OMEC were retrospectively analyzed,and the rel-evant literature was reviewed.Results Case 1 was a 50-year-old man who presented with a painless mass behind the right earlobe for more than 2 years.The patient underwent extended parotidectomy with preservation of the facial nerve.Histopathological examination revealed that the tumor was predominantly composed of oncocytic cells with a small pro-portion of mucous cells.Immunohistochemically,the tumor cells were partially positive for cytokeratin 5/6,cytokeratin 7,and P63.Special staining with alcian blue,periodic acid-Schiff,and phosphotungstic acid hematoxylin yielded posi-tive results.The diagnosis of right parotid OMEC was established.No recurrence or metastasis was observed during a 1 year follow-up.Case 2 was a 61-year-old man with a 3-month history of a mass beneath the left ear.After partial paroti-dectomy at an outside institution,pathological consultation at the Stomatological Hospital of Jilin University demon-strated that the tumor consisted almost entirely of oncocytic cells,exhibited infiltrative growth,and lacked typical mu-cous,epidermoid,and intermediate cells.Fluorescence in situ hybridization confirmed positive mastermind-like tran-scriptional activator 2(MAML2)gene rearrangement,establishing the diagnosis of left parotid OMEC.The patient sub-sequently underwent total parotidectomy with preservation of the facial nerve,and no recurrence was detected during a short-term 3 months follow-up.A review of the literature indicated that OMEC most commonly arises in the parotid gland and is generally a low-grade malignancy with favorable prognosis.When tumors are composed exclusively of onco-cytic cells,exhibit minimal cytological atypia,and lack the classical cellular components of mucoepidermoid carcinoma,they are highly prone to misdiagnosis as oncocytoma,nodular oncocytic hyperplasia,or other benign oncocytic lesions.Accurate differential diagnosis relies on recognition of infiltrative growth patterns,supportive immunophenotypic mark-ers(e.g.,P63 positivity),and detection of characteristic MAML2 gene rearrangement.Complete surgical excision re-mains the treatment of choice.Conclusion OMEC dominated by oncocytic cells carries a high risk of clinical misdiag-nosis.Integrating the assessment of infiltrative histopathological features with immunohistochemistry and molecular de-tection of MAML2 rearrangement is crucial for accurate diagnosis,appropriate assessment of tumor behavior,and opti-mal surgical decision making.
郑放;聂孟冬;强金彪;金荣豪;王丹丹;史册
吉林大学口腔医院口腔病理科 吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)吉林大学口腔医院口腔病理科 吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)吉林大学口腔医院口腔病理科 吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)吉林大学口腔医院口腔病理科 吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)吉林大学口腔医院口腔病理科 吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)吉林大学口腔医院口腔病理科 吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)
医药卫生
唾液腺肿瘤黏液表皮样癌嗜酸细胞型免疫组织化学分子诊断基因重排mastermind样转录共激活因子2
salivary gland neoplasmsmucoepidermoid carcinomaoncocytic variantimmunohistochemis-trymolecular diagnosisgene rearrangementmastermind-like transcriptional activator 2 gene
《口腔疾病防治》 2026 (6)
576-584,9
吉林省财政厅科技项目(JCSZ2021893-36) This study was supported by the grants from the Jilin Province Department of Finance(No.JCSZ2021893-36).
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