title: Middle ear adenoma with neuroendocrine differentiation : A pediatric case report.
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  In our opinion a pathological radiological result is insufficient to identify recurrent or residual disease within the tympanic cavity.
authors.affiliation: Department of Otorhinolaryngology and Head and Neck Surgery, Rudolfstiftung Teaching Hospital, Juchgasse 25, 1030, Vienna, Austria.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Sterrer
authors.affiliation: Department of Otorhinolaryngology and Head and Neck Surgery, Rudolfstiftung Teaching Hospital, Juchgasse 25, 1030, Vienna, Austria.
authors.forename: Franz
authors.initials: F
authors.lastname: Windisch
authors.affiliation: Department of Pathology, Rudolfstiftung Teaching Hospital, Vienna, Austria.
authors.forename: Kurt
authors.initials: K
authors.lastname: Frey
authors.affiliation: Department of Nuclear Medicine, Rudolfstiftung Teaching Hospital, Vienna, Austria.
authors.forename: Klaus
authors.initials: K
authors.lastname: Rettensteiner
authors.affiliation: Department of Otorhinolaryngology and Head and Neck Surgery, Rudolfstiftung Teaching Hospital, Juchgasse 25, 1030, Vienna, Austria. benjamin.loader@wienkav.at.
authors.forename: Benjamin
authors.identifier: http://orcid.org/0000-0003-4387-5993
authors.initials: B
authors.lastname: Loader
mesh_terms: D000236:Adenoma / Q000473:pathology* / Q000601:surgery*; D000293:Adolescent; D003937:Diagnosis, Differential; D004428:Ear Neoplasms / Q000473:pathology* / Q000601:surgery*; D004432:Ear, Middle / Q000502:physiology / Q000601:surgery; D005260:Female; D006801:Humans; D018358:Neuroendocrine Tumors / Q000473:pathology* / Q000601:surgery*; D016896:Treatment Outcome
keywords: Adenoma; Carcinoid; Middle ear; Neuroendocrine; Octreotide