2025 Poster Presentations
P056: NEUROENDOCRINE TUMOR OF THE TEMPORAL BONE WITH MIDDLE CRANIAL FOSSA SPREAD
Samantha Y Cerasiello, MD; Nina Dharmarajah; John P Leonetti, MD; Loyola University Medical Center
Introduction: Neuroendocrine tumors are generally found in the digestive and respiratory tracts, but are rare neoplasms of the ear and temporal bone. These tumors are typically low grade and conventionally thought to have a small risk of invasiveness and low metastatic potential. This case study is a report of a patient who presented with a right middle ear mass and middle fossa lesion.
Methods: Retrospective case presentation
Results: A 35 year old male with partially recovered right facial paralysis and progressive right-sided hearing loss, pain, and bloody otorrhea. The patient had a House-Brackmann Grade III facial function on the right and a soft tissue mass completely filling and extruding from the right external auditory canal (EAC). A temporal bone CT showed a soft tissue mass filling the middle ear and EAC and an MRI revealed a middle fossa lesion. The patient underwent a right temporal bone resection and ear canal oversew with findings including a large polypoid mass in EAC, soft tissue filling the middle ear attached to the promontory and a dehiscent tympanic segment of facial nerve. Pathology showed neuroendocrine tumor with lymphovascular invasion. The patient then underwent Dotatate PET/CT which showed focal uptake in the right middle cranial fossa at site of previously identified temporal lobe lesion. Additional uptake was noted in the left thyroid lobe, which may represented an additional focus of disease. Interval MRI showed an increase in size of the right middle cranial fossa dural based tumor. The patient subsequently underwent a right middle fossa craniotomy with intradural resection of this lesion. Intraoperative frozen section was consistent with neuroendocrine tumor. The patient then underwent radiotherapy over a six week time period.
Conclusions: Neuroendocrine tumors are rare primary ear tumors initially characterized as having low potential for metastasis, however several case series over the years have reported higher rates of local recurrence and distant metastasis. Surgical resection is the optimal primary treatment for these neoplasms, but adjuvant radiotherapy has been described in the literature for subtotal resection, recurrence, and distant metastasis. We present a young male initially thought to have right cholesteatoma with a separate right temporal lobe meningioma and was found to neuroendocrine tumor of the temporal bone with extension to middle cranial fossa. A systematic review of temporal bone neuroendocrine tumors will be included.