Middle ear adenomatous neuroendocrine tumors: suggestion for surgical strategy

INTRODUCTION: Middle ear adenomatous neuroendocrine tumors are extremely rare neoplasms with epithelial and neuroendocrine differentiation, accounting for fewer than 2% of all middle and inner ear tumors. Universal standard surgical procedures for different stages of these tumors remain elusive due to the limitation of the small number of case reports or investigations. OBJECTIVE(S): This study intends to investigate proper surgical strategies for patients with middle ear adenomatous neuroendocrine tumors. METHODS: Six patients with middle ear adenomatous neuroendocrine tumors who were treated at the Second Affiliated Hospital of Nanchang University (Nanchang, China) and the Eye, Ear, Nose, and Throat Hospital of Fudan University (Shanghai, China) respectively. Clinical characteristics and management strategies of patients were reviewed. The mean follow-up time was 63.7 months (range, 13–153 months). All the information was collected from medical records and prognosis postoperatively. RESULTS: Three patients underwent canal wall-up tympanomastoidectomy, including one patient with recurrence who underwent a previous tympanotomy; the other three patients underwent lateral temporal bone resection All of these patients were followed up with no evidence of recurrence or metastasis. Patients underwent canal wall-up surgery treatment accompanied with hearing function preservation measurements during follow-up periods. CONCLUSIONS: Complete surgical resection provided good results for patients with middle ear adenomatous neuroendocrine tumors. The ossicular chain should be removed. Because of the propensity for local recurrence and invasiveness, as well as regional or distant metastasis of these tumors, it is necessary to schedule long-term follow-up and an observation plan postoperatively.