Neuroendocrine Adenoma of the Middle Ear: A Rare Histopathological Diagnosis

Abstract

Neuroendocrine tumours occur throughout the body but are rare in the head and neck region and particularly rare in the middle ear. Clinical findings are often nonspecific and therefore pose a diagnostic challenge. Furthermore, the nomenclature of neuroendocrine tumours of the middle ear is historically controversial. Herein a case is presented of a middle ear adenoma in a 33-year-old patient who presented with otalgia, hearing loss, and facial nerve palsy. A brief discussion is included regarding the histopathological features of middle ear adenomas and seeks to clarify the correct nomenclature for these tumours.

111In-Octreoscan SPECT/CT hybrid imaging and 68Ga-DOTANOC PET/CT in neuroendocrine adenoma of the middle ear (NAME)

“… 68Ga-DOTANOC PET/CT shows focal pathologic uptake in correspondence of the solid tissue reported in the left mastoid and tympanic airspaces, confirming the occurrence of high density of somatostatin receptors (SSRs 2, 3 5) in this region. (A) Fused PET/CT image on axial plane (B) CT image on axial plane (C) 68Ga- DOTANOC PET image on axial plane (D) 68Ga-DOTANOC PET maximum-intensity projection (MIP) …”

“…The nosologic distinction between middle ear neuroendocrine tumours from adenomatous tumours is not so unequivocal, given that someone keeps considering them two distinct entities while others believe them to represent a spectrum of the same disease. [ 3 ] Middle ear glandular neoplasms were classified by Saliba et al . in three different types based on the occurrence of immunohistochemical markers and/or metastasis.…”

Middle ear neuroendocrine tumours: Insight into their pathogenesis, diagnosis and management

“…Other associated symptoms include tinnitus, ear discharge, fullness or pain 4,[8][9][10][11][12][13][14] : rarely, they may present with facial nerve palsy, 4,12,13 although in our patient this only developed after extensive surgery.…”

Adenomatous neuroendocrine tumors of the middle ear (MEANTs) with local invasion and lymph nodes metastasis: A brief report of a rare neoplasm

“…More publications have showed these tumor metastasize at the time of the initial diagnosis or several years after surgical resection to local cervical lymph node, salivary glands, muscle, cranial nerves, and dura, 8,11,17,19 and distant to the liver and iliac crest 11,20 . Our case showed the tumor had locally invaded the temporal lobe of the brain and bone, facial nerve, with bilateral cervical lymph nodes metastasis.…”

“…Recurrent chronic otitis media and facial paralysis can also occur 15,16 . It is believed that facial paralysis is temporary and is related to local compression of tumors or local inflammation rather than invasion of tumors 9,17,18 . However, our case shows a tumor invasion of the facial nerve, which suggests a malignant pathologic behavior that needs long‐term follow‐up.…”