In the group of already rare middle ear neoplasms, around 2% can be classified as neuroendocrine tumors of the middle ear (MeNET). Patients with MeNET manifestation usually report rather nonspecific symptoms such as aural fullness, hearing loss and vertigo and clinical and radiological examinations do not provide the sensitivity and specificity disered for a reliable diagnosis. Therefore, MeNET are often found incidental in exploratory tympanotomy and even intraoperatively, their appearance is quite heterogenous. Consequently, a definitive diagnosis is predominantely based on histopathology and immunohistochemical examination. As rare findings and a comparatively small number of reported cases, it still remains unclear how the neurendocrine cells migrate into the tympanic cavity. It is much more common for neuroendocrine tumors to occur in the digestive tract or lungs. Interistingly, MeNETs can show similarities to those gastroenteropancreatic neuroendocrine neoplasms and may even show a multilocular occurence. In this case-report, we present a 65-year-old female patient with MeNET who complained about hearing loss and aural fullness in her left ear. After histological evaluation, a MeNET of a pancreatic origin was assumed. This is to our knowledge the first reported case, where additional staging with DOTATOC PET/CT scan showed an upregulated somatostatin expression in the pancreatic caput and cauda and also abdominal MRI showed a suspected correlate. This is a case with unexpected twists in diagnosis and treatment which gives an excellent example of how individualized treatment of patients with rare diseases remains an extremely challenging task.

Nein