Adenomatous Neuroendocrine Tumors of the Middle Ear: A Multi-institutional Investigation of 32 Cases and Development of a Staging System
Adenomatous Neuroendocrine Tumors of the Middle Ear: A Multi-institutional Investigation of 32 Cases and Development of a Staging System
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DOI：
10.1097/mao.0000000000001905
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发表时间：
2018-09-01
影响因子：
2.1
通讯作者：
Carlson, Matthew L.
中科院分区：
文献类型：
--
作者：
Marinelli, John P.;Cass, Stephen P.;Carlson, Matthew L.
目的：迄今为止，英文文献中报道的中耳腺瘤性神经内分泌肿瘤（MEANTs）不到150例。本研究的目的是提供一个当代分析这些罕见的病变，并制定一个共识驱动的stagingsystem.Study设计：多机构回顾性组织病理学，放射学和临床审查。设置：六个三级转诊中心。患者：32例患者病理证实MEANT。干预：手术切除，辅助治疗。主要结果措施：临床表现，结果，stagingsystem.Results：患者通常表现为进行性传导性或混合性听力损失（90%），耳满（50%），音调耳鸣（46%）。脉动性耳鸣（16%）、类癌综合征（4%）和面神经麻痹（4%）较少见。MEANTs常表现为颞骨副神经节瘤（31%）和中耳乳突瘤（15%）。根据本文提出的新的T/N/M/S分期系统（S=分泌性肿瘤），有6例（19%）T1，19例（59%）T2和7例（22%）T3 MEANT。与继发于重要神经血管结构（如岩部颈内动脉和面神经）粘连的低分期肿瘤相比，T3肿瘤更有可能接受次全切除或接近全切除（p=0.027）。与低分期肿瘤相比，T3 MEANTs患者更可能经历多次复发，需要生长抑素类似物辅助治疗，或发生永久性面神经麻痹。末次随访时，T1 MEANT患者均未复发，而T2 MEANT患者中37%（7/19）和T3 MEANT患者中57%（4/7）在大体全切除术（GTR）后复发或已知残留肿瘤再生长，需要额外手术，中位持续时间为72个月结论：MEANTs患者可表现出与更常见的中耳病变重叠的非特异性中耳炎。根据T分期，MEANT表现出复发的倾向，长期临床随访是必要的，特别是对于晚期肿瘤。
Objective:To date, less than 150 cases of middle ear adenomatous neuroendocrine tumors (MEANTs) have been reported in the English literature. The objective of this study was to provide a contemporary analysis of these rare lesions and develop a consensus-driven staging system.Study Design:Multi-institutional retrospective histopathologic, radiologic, and clinical review.Setting:Six tertiary referral centers.Patients:Thirty-two patients with pathologically confirmed MEANT.Intervention:Surgical resection, adjuvant therapy.Main Outcome Measures:Clinical manifestations, outcomes, staging system.Results:Patients commonly presented with progressive conductive or mixed hearing loss (90%), aural fullness (50%), and tonal tinnitus (46%). Pulsatile tinnitus (16%), carcinoid syndrome (4%), and facial nerve paresis (4%) were less commonly observed. MEANTs frequently mimicked temporal bone paraganglioma (31%) and cholesteatoma (15%) at presentation. According to a novel T/N/M/S staging system (S=secretory tumor) proposed herein, there were 6 (19%) T1, 19 (59%) T2, and 7 (22%) T3 MEANTs. T3 tumors were significantly more likely to undergo subtotal or near total resection compared with lower staged tumors secondary to adherence to critical neurovascular structures such as the petrous internal carotid artery and facial nerve (p=0.027). Patients with T3 MEANTs were more likely to experience multiple recurrences, require adjuvant therapy with a somatostatin analogue, or develop permanent facial nerve paresis compared with lower staged tumors. At last follow up, no patients with T1 MEANTs had developed recurrence, whereas 37% (7/19) of patients with T2 MEANT and 57% (4/7) of patients with T3 MEANT experienced either disease recurrence after gross total resection (GTR) or regrowth of known residual tumor requiring additional surgery at a median duration of 72 months (95% CI, 24-84).Conclusions:Patients with MEANTs can present with nonspecific symptomatology that overlaps with more commonly encountered middle ear lesions. MEANT exhibits a proclivity for recurrence according to T-stage and long-term clinical follow up is necessary, particularly for advanced stage tumors.