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

Iris Krebbers; Rens M. van de Goor; Roel J.W. van Kampen; Paul H.M.H. Theunissen; Verona E. Bergshoeff

doi:10.1016/j.xocr.2020.100169

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

Iris Krebbers^*, Rens M. van de Goor, Roel J.W. van Kampen, Paul H.M.H. Theunissen, Verona E. Bergshoeff

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

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.

Original language	English
Article number	100169
Journal	Otolaryngology Case Reports
Volume	15
DOIs	https://doi.org/10.1016/j.xocr.2020.100169
Publication status	Published - 1 Jun 2020

Keywords

External auditory canal
Mast cell disorders
Parotid gland
Systemic mastocytosis

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10.1016/j.xocr.2020.100169Licence: CC BY

Cite this

@article{1a1c7240fe68489781e7a75f115806e5,

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

abstract = "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.",

keywords = "External auditory canal, Mast cell disorders, Parotid gland, Systemic mastocytosis",

author = "Iris Krebbers and {van de Goor}, {Rens M.} and {van Kampen}, {Roel J.W.} and Theunissen, {Paul H.M.H.} and Bergshoeff, {Verona E.}",

note = "Publisher Copyright: {\textcopyright} 2020 Elsevier Inc.",

year = "2020",

month = jun,

day = "1",

doi = "10.1016/j.xocr.2020.100169",

language = "English",

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T1 - An unexpected, invasive disease of the mastoid bone and external auditory canal

AU - Krebbers, Iris

AU - van de Goor, Rens M.

AU - van Kampen, Roel J.W.

AU - Theunissen, Paul H.M.H.

AU - Bergshoeff, Verona E.

PY - 2020/6/1

Y1 - 2020/6/1

N2 - 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.

AB - 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.

KW - External auditory canal

KW - Mast cell disorders

KW - Parotid gland

KW - Systemic mastocytosis

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DO - 10.1016/j.xocr.2020.100169

M3 - Article

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