Primary Lymphoma of Internal Acoustic Meatus Mimicking Vestibular Schwannoma—A Rare Diagnostic Dilemma

Abstract

Background/Setting A subject presenting with a unilateral sensorineural hearing loss and with vertigo/imbalance and a lesion of internal acoustic meatus (IAM) most often represents a vestibular schwannoma. Several alternative pathologies involving the region, with clinical and neuroradiological similarities, could lead to an error in judgement and management. Rare tumors of the IAM pose unique diagnostic difficulty. A rare case that we present here had a typical history and imaging findings suggestive of vestibular schwannoma. A primary central nervous system (CNS) lymphoma was diagnosed in later stages of brain involvement warranting a retrospective analysis of the entity. Case Summary An 80-year-old male presented with unilateral sensorineural hearing loss, vertigo, and imbalance. On imaging, he was found to have a lesion in the left internal auditory meatus, reported as a vestibular schwannoma and operated upon. Subject's condition worsened with time and a repeat imaging was suggestive of a CNS lymphoma with lesions involving bilateral cerebellum and subcortical white matrix. Conclusion To conclude, primary CNS lymphoma presenting an isolated lesion in the IAM with no other parenchymal lesions at presentation is a rare incidence; to our knowledge this is the first case of such unique presentation.

A Scoping Review of Otologic Manifestations of Hematologic Malignancies

“…Two hundred seventy-two articles reported on 553 patients with otologic manifestations of hematologic malignancies, of which 255 (93.8%) were case reports and of OECBM level V evidence and 17 (6.2%) were case series and of OECBM level IV evidence (Supplement 3, http://links.lww.com/MAO/B834; Supplement 4, http://links.lww.com/MAO/B835) (4,5,7–10,13,14,19–282). While the largest proportion of articles originated in the United States (n = 83, 30.5%), the literature spanned countries across all continents.…”

Neurolinfomatose Bilateral do Ângulo Ponto-Cerebeloso

“…10 A dificuldade em estabelecer em definitivo um diagnóstico desta raridade é incrementada pelo facto de a neurolinfomatose poder preceder o diagnóstico de linfoma sistémico em até 50% dos doentes, o que também justifica que referências a quadros como o aqui apresentado apenas surjam de modo esporádico na literatura -aspeto que, seguramente, corroboramos. 7,13,14 O tratamento da neurolinfomatose do SNC baseia--se, por analogia ao tratamento do linfoma primário do SNC (e na ausência de casuística suficiente para permitir uma definição terapêutica mais específica) na quimioterapia sistémica. 15 Existem algumas reservas quanto ao recurso a radioterapia e a quimioterapia intratecal, tendo como racional o pressuposto de que o envolvimento pela neurolinfomatose vá além dos limites do espaço subaracnoideu.…”

A Rare Case of Primary Central Nervous System Lymphoma Located in the Anterior Geniculate Ganglion Diagnosed by 18F-FDG PET/CT and MRI

“…Other, less frequent, etiologies to be considered in the differential diagnosis of tumor of GGA are epidermoid cyst [10], primary brain tumor, metastases, perineural spread of other malignancies, lymphoproliferative disorders [2].…”

“…Primary Central Nervous System Lymphoma (PNCSL) is a rare form of extranodal non-Hodgkin lymphomas, exclusively located in central nervous system, usually presenting as a supratentorial lesion [1]. When rarely occurs in geniculate ganglion, PNCSL can present non-specific neurological symptoms and have radiologic features that mimic, especially in the early stage, other pathologies more frequently involved in this area [2].…”