Late onset middle ear neuroendocrine tumor presenting with distant metastasis

Tumors of the ear with neuroendocrine features are a very rare group neoplasms, adenoma representing more frequent entity found; prevalence middle tumors is so low that it has never been determined exactly. Herein we describe case 74 years old woman who presented pure moderately differentiated tumor after long history adhesive otitis media. Patient abruptly developed otalgia and sense plugged ear. Otomicroscopy revealed polypoid mass external auditory canal. On histopathologic exam neoplastic proliferation monomorphous epithelioid cells arranged in small nests clusters was present, being positive to cytokeratins, chromogranin, synaptophysin CD56, negative S100. Ki-67 index 20%. The lesion diagnosed as (NET grade 2). CT PET scans highlighted concurrent presence locoregional lymph nodes involvement but also at distance liver metastases. patient underwent chemotherapy repetitions were treated locally. At time writing good general condition. We an exceeding primitivity distant metastasis diagnosis. This represents clinical histological challenge, for rarity aspecific symptoms patient.