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Erschienen in: Wiener klinische Wochenschrift 5-6/2017

01.03.2017 | case report

Middle ear adenoma with neuroendocrine differentiation

A pediatric case report

verfasst von: MD Elisabeth Sterrer, MD Franz Windisch, MD Kurt Frey, MD Klaus Rettensteiner, MD Benjamin Loader

Erschienen in: Wiener klinische Wochenschrift | Ausgabe 5-6/2017

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Summary

Background

Middle ear adenoma with neuroendocrine differentiation (MEA-ND), also known as carcinoid tumor of the middle ear, is an extremely rare neoplasm, especially when located within the tympanic cavity. To the best of our knowledge, this represents the first pediatric case of a MEA-ND described in Europe. Excluding the present case, only three other pediatric cases of this disease have been published globally.

Case report

We report on a 15-year-old female patient who presented with conductive hearing loss of the right ear. A tympanogram was flat. Ear microscopy revealed bulging of the right posterior superior tympanic quadrant. Computed tomography depicted an opacification of the middle ear and mastoid air cells without evidence of bone erosion. Paracentesis was performed assuming a serous otitis media. Since there was no liquid found the patient underwent an atticoantrotomy and mastoidectomy as therapy for cholesteatoma. Histologic examination revealed a highly differentiated neuroendocrine tumor. Immunohistochemical evaluation showed strong positivity for synaptophysin. On the basis of these results, the patient underwent a middle ear revision surgery. After 15 months, magnetic resonance imaging and octreotide scans suggested disease recurrence, prompting a second surgical revision. The histologic examination confirmed a recurrence of only 1 mm, but postoperative imaging still showed pathological diffuse enhancement. Eight years after the initial diagnosis there has been no evidence of recurrence or metastasis.

Conclusion

In our opinion a pathological radiological result is insufficient to identify recurrent or residual disease within the tympanic cavity.
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Metadaten
Titel
Middle ear adenoma with neuroendocrine differentiation
A pediatric case report
verfasst von
MD Elisabeth Sterrer
MD Franz Windisch
MD Kurt Frey
MD Klaus Rettensteiner
MD Benjamin Loader
Publikationsdatum
01.03.2017
Verlag
Springer Vienna
Erschienen in
Wiener klinische Wochenschrift / Ausgabe 5-6/2017
Print ISSN: 0043-5325
Elektronische ISSN: 1613-7671
DOI
https://doi.org/10.1007/s00508-016-1104-8