Skip to main content
SpringerLink
Log in

Middle ear adenoma with neuroendocrine differentiation

A pediatric case report

  • case report
  • Published:
Wiener klinische Wochenschrift Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

References

  1. Murphy GF, Pilch BZ, Dickersin GR, et al. Carcinoid tumor of the middle ear. Am J Clin Pathol. 1980;73(6):816–23.

    Article  CAS  PubMed  Google Scholar 

  2. Thomson L, Wenig B. Diagnostic pathology: head and neck. Salt Lake City: Amirsys; 2011, pp 46–9.

    Google Scholar 

  3. Ramsey MJ, Nadol JB, Pilch BZ, McKenna MJ. Carcinoid tumor of the middle ear: clinical features, recurrences, and metastases. Laryngoscope. 2008;115(9):1660–6.

    Article  Google Scholar 

  4. Fundakowski CE, Chapman JR, Thomas G. Middle ear carcinoid with distant osseous metastasis. Laryngoscope. 2013;123(3):779–82.

    Article  PubMed  Google Scholar 

  5. Dogru S, Wilkinson E, Robinson RA, Smith RJ. Middle ear adenoma with neuroendocrine differentiation in the pediatric population. Int J Pediatr Otorhinolaryngol. 2009;73(6):895–8.

    Article  PubMed  Google Scholar 

  6. Torske KR, Thompson LD. Adenoma versus carcinoid tumor of the middle ear: a study of 48 cases and review of the literature. Mod Pathol. 2002;15(5):543–55.

    Article  PubMed  Google Scholar 

  7. Nikanne E, Kantola O, Parviainen T. Carcinoid tumor of the middle ear. Acta Otolaryngol. 2004;124(6):754–7.

    Article  PubMed  Google Scholar 

  8. Rieh E, Steigerwald C, Göbel H, Maier W, Offergeld C. Carcinoid of the middle ear. A case report. HNO. 2009;57(8):835–8.

    Article  CAS  PubMed  Google Scholar 

  9. Kambayashi J, Ishidoya M, Urae T, Saijo S, Yuasa R, Kimura N, Sasano N. A case of carcinoid tumor of the middle ear producing peptide hormones. Auris Nasus Larynx. 1988;15(3):155–63.

    Article  CAS  PubMed  Google Scholar 

  10. Pathology quiz case 1. Arch Otolaryngol Head Neck Surg. 2006;132:1156. doi:10.1001/archotol.132.10.115.

Download references

Author information

Authors and Affiliations

  1. Department of Otorhinolaryngology and Head and Neck Surgery, Rudolfstiftung Teaching Hospital, Juchgasse 25, 1030, Vienna, Austria

    Elisabeth Sterrer MD, Franz Windisch MD & Benjamin Loader MD

  2. Department of Pathology, Rudolfstiftung Teaching Hospital, Vienna, Austria

    Kurt Frey MD

  3. Department of Nuclear Medicine, Rudolfstiftung Teaching Hospital, Vienna, Austria

    Klaus Rettensteiner MD

Authors
  1. Elisabeth Sterrer MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Franz Windisch MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Kurt Frey MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Klaus Rettensteiner MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Benjamin Loader MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Benjamin Loader MD.

Ethics declarations

Conflict of interest

E. Sterrer, F. Windisch, K. Frey, K. Rettensteiner, and B. Loader declare that they have no competing interests.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Sterrer, E., Windisch, F., Frey, K. et al. Middle ear adenoma with neuroendocrine differentiation. Wien Klin Wochenschr 129, 208–211 (2017). https://doi.org/10.1007/s00508-016-1104-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00508-016-1104-8

Keywords

Search

Navigation