An unexpected, invasive disease of the mastoid bone and external auditory canal

• Main Authors: Iris Krebbers, Rens M. van de Goor, Roel J.W. van Kampen, Paul H.M.H. Theunissen, Verona E. Bergshoeff
• Format: Article
• Language: English
• Published: Elsevier 2020-06-01
• Series: Otolaryngology Case Reports
• Subjects: Systemic mastocytosis; Mast cell disorders; External auditory canal; Parotid gland
• Online Access: http://www.sciencedirect.com/science/article/pii/S2468548820300151
• ISSN: 2468-5488

A 65-year-old male patient with a history of Myelodysplastic Syndrome (MDS) and Systemic Mastocytosis (SM) presented with an invasive disease of the external auditory canal (EAC) and a parotid gland abscess. Abscess drainage and treatment for a malignant otitis externa (MOE) was started, which did not lead to clinical improvement. Computed Tomography (CT) of the petrous bone as well as a Positron Emission Tomography/Computed Tomography with 2-[fluorine-18]-fluoro-2-deoxy-d-glucose (FDG-PET-CT) showed bony erosion of the EAC with a symmetrical FDG distribution at the level of both EACs. Histopathological biopsies of the EAC revealed a rarely encountered localization of systemic mastocytosis (SM). Especially patients with a hematological disorder such as a MDS are at risk to develop SM. In case of a fulminant infectious disease of the EAC in these patients, a localization of SM should be considered as well. This is the first report on SM with a localization in the EAC.