You Dan, Li Qingzhong, Yu Huiqian
ENT Institute and Otorhinolaryngology Department of Eye & ENT Hospital, State Key Laboratory of Medical Neurobiology and MOE Frontiers Center for Brain Science, Fudan University, Shanghai, PR China.
NHC Key Laboratory of Hearing Medicine, Fudan University, Shanghai, PR China.
Ear Nose Throat J. 2024 Oct 8:1455613241264435. doi: 10.1177/01455613241264435.
Middle ear adenomatous neuroendocrine tumors (MEANTs) are rare middle ear lesions characterized by nonspecific symptoms, signs, and imaging findings. Diagnosis typically relies on postoperative pathological assessment. This study investigated the diagnostic utility of the predilection sites and clinical characteristics of MEANTs. A retrospective analysis was conducted on clinical data from 10 patients with histologically confirmed MEANTs, admitted to Eye & ENT Hospital of Fudan University between March 2016 and March 2023. The median age of the patients at diagnosis was 39.3 years. Hearing loss (n = 8) and ear pain (n = 6) were the most prevalent clinical symptoms in the patients diagnosed with MEANTs. Endoscopic examination revealed diverse symptoms, predominantly presenting as non-pulsatile masses with distinct boundaries and quasi-circular shapes within the external auditory canal, often accompanied by abundant blood vessels (n = 4). Tumors were typically confined to the middle/lower tympanic chambers or eustachian tube and were frequently associated with tympanic sclerosis, particularly around the pharyngeal tube (n = 3). Pathologically, MEANTs exhibited CD56 positivity or weak positivity, along with positive staining for CKpan and Syn, negativity for S100, and Ki67 ≤3%. Personalized surgical interventions were chosen by all the patients based on lesion severity, with no subsequent radiotherapy or chemotherapy administered postoperatively. No tumor progression was noted during the postoperative follow-up. In addition, a noteworthy case was presented in which MEANT initially manifested in the middle or lower tympanic cavity and eustachian tubes. Over 2 years, the tumor progressively grew, invading the middle tympanum and surrounding ossicles, ultimately achieving complete resection with no recurrence observed during subsequent follow-up. A possible diagnosis of middle ear adenoma should be considered when encountering non-pulsatile tumors with clearly demarcated inner boundaries within the external auditory canal accompanied by abundant quasi-circular vessels and the presence of new bone or neoplasm at the pharyngeal tympanic canal orifice observed during preoperative examinations or surgical procedures.
中耳腺瘤样神经内分泌肿瘤（MEANTs）是罕见的中耳病变，其特征为非特异性症状、体征及影像学表现。诊断通常依赖术后病理评估。本研究调查了MEANTs的好发部位及临床特征的诊断价值。对2016年3月至2023年3月期间复旦大学附属眼耳鼻喉科医院收治的10例经组织学确诊的MEANTs患者的临床资料进行回顾性分析。患者确诊时的中位年龄为39.3岁。听力损失（n = 8）和耳痛（n = 6）是诊断为MEANTs患者中最常见的临床症状。内镜检查发现多种症状，主要表现为外耳道内边界清晰、形状近似圆形的非搏动性肿物，常伴有丰富血管（n = 4）。肿瘤通常局限于中/下鼓室或咽鼓管，常与鼓室硬化相关，尤其是在咽鼓管周围（n = 3）。病理上，MEANTs表现为CD56阳性或弱阳性，CKpan和Syn染色阳性，S100阴性，Ki67≤3%。所有患者均根据病变严重程度选择了个性化手术干预，术后未进行后续放疗或化疗。术后随访期间未发现肿瘤进展。此外，还介绍了1例值得注意的病例，MEANT最初表现于中或下鼓室及咽鼓管。2年多来，肿瘤逐渐生长，侵犯中耳及周围听小骨，最终实现完整切除，后续随访未观察到复发。术前检查或手术过程中，当遇到外耳道内边界清晰、伴有丰富近似圆形血管的非搏动性肿瘤，且在咽鼓管鼓口处发现新骨或肿瘤时，应考虑中耳腺瘤的可能诊断。
