2例中耳神经内分泌腺瘤的临床诊疗及文献回顾

Diagnosis and treatment of two Middle ear adenomatous neuroendocrine tumor and Literature Review

摘要:

目的 中耳神经内分泌腺瘤（MEANTs）是一种罕见的肿瘤，占中耳肿瘤不到2%。该疾病临床表现不典型，手术方案尚无共识，临床医生对该病诊疗存在困难。方法 回顾性分析2020年-2022年厦门大学附属中山医院耳鼻咽喉科收治的2例MEANTs的临床资料，包括术前内镜、听力检查和影像学检查，术后病理诊断明确中耳神经内分泌腺瘤以及术后随访情况。结果 2例均为男性，1例为右耳，1例为左耳。耳内镜下表现1例见外耳道淡红色新生物，鼓膜窥不清；另1例表现为鼓膜完整膨隆；颞骨CT显示鼓室软组织影，可延伸至乳突和外耳道，包绕听小骨，听骨链无明显骨质破坏；MRI表现为鼓室等T1等T2信号影，增强后明显强化。2例均行改良乳突根治术+听骨链重建术+鼓膜修补术。病理形态呈现腺样或巢状实性结构，免疫组化呈现CK-P和神经内分泌标记物不同程度的阳性表达。两例患者在随访期间内均未发现肿瘤复发。结论 MEANTs是一种罕见疾病，具有良性形态特征和缓慢生长的特点，确诊依靠病理检查。手术完整切除是治疗MEANTs的主要手段。由于肿瘤具有潜在局部复发或转移的特性，术后需要定期随访。

Abstract:

Objective Middle ear adenomatous neuroendocrine tumor (MEANTs) is a rare entity and accounts for approximately 2% of all middle ear tumors。The patients usually have nonspecific symptoms，universal standard surgical procedures for different stages of these tumors remain elusive。The doctor has difficulties in diagnosing and treating the disease。Methods Three cases of MEANTs were collected from Xiamen University Zhongshan Hospital between 2020 and 2022。Endoscope examination、audiometry test and temporal bone CT were performed before the surgery and follow-up。Post-operative immunohistochemistry confirmed the diagnosis。Results The patients included two men。One MEANTs occurred on the right side，another occurred on the left side。Endoscope examination of one patients revealed that the right external ear canal was filled with polypoid tissue, tympanic membrane was not possible to visualize。another case presented with complete bulging of the tympanic membrane。In temporal bone CT, the soft-tissue mass located the middle ear was found to extend to the antrum and the external auditory canal。MEANTs tend to surround the ossicles without erosion；MRI images showed equalT1and T2 signals with intensifying in tympanum。Two patients underwent canal wall down mastoidectomy and ossicular reconstruction。Two cases all showed adenoid or solid nest-like structures in the pathological morphology. Immunohistochemistry showed varying degrees of positive expression of CK-P and Neuroendocrine markers. Two patients showed no evidence of recurrence during followed up。Conclusions MEANTs are rare middle ear masses with benign morphological features and slow local growth。The treatment is complete tumoral excision。Patients need to be monitored lifelong for early detection of recurrence。