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

Abstract

Neuroendocrine tumours (NETs) represent a spectrum of tumours with a diverse range of molecular abnormalities, functionality and anatomical locations. 1 The common sites of neuroendocrine tumours include the gastrointestinal tract, pancreas and lungs, although other primary sites have been reported, such as the ovary, 2 thymus and C cells of the thyroid. 3 Very rarely, these tumours appear to arise from the middle ear and, although extremely uncommon, accounting for < 2% of all middle ear tumours, 4 they are important to differentiate from other middle ear tumours because the management of these tumours significantly differs from these other types of tumour. In addition, they raise significant questions as to the provenance of such tumours arising from an area not generally known to harbour neuroendocrine tissue.We describe two patients with middle ear NETs, one with multiple local recurrences and the other with distant metastases, discuss the management of these tumours, and review the extant literature on these tumours and their putative derivation.

Successful PRRT-rechallenge in a metastasized neuroendocrine tumor of the middle ear

“…Like many other patients with neuroendocrine tumors (NET) of the middle ear our patient suffered from a therapeutic odyssey of more than 4 years prior to final diagnosis. Moreover, our presented case confirms the observation that the traditionally called "ademomas of the middle ear" [1] do indeed have a relevant potential for aggressive local recurrence and metastasis just like many other NET as previously postulated by Saliba and Edvard [2]. Within the course of disease, a steady increase in proliferation rate from initially < 2 % (G1) to 15 % (G2) in the latest resected specimen had been observed.…”

Middle ear neuroendocrine tumor: a case report

“…The most frequent symptoms are hearing loss, which is commonly of the conductive type, ear fullness, tinnitus and/or ear pain. Rarely, they may present with facial nerve palsy [ 4 ].…”

“…The Ki-67 proliferation index may play an important role in prognostication and therapeutic decision. The other most important factor determined by the histology is the degree of differentiation of the cells [ 4 ].…”