CC BY-NC-ND 4.0 · Laryngorhinootologie 2018; 97(S 02): S197
DOI: 10.1055/s-0038-1640393
Otologie: Otology
Georg Thieme Verlag KG Stuttgart · New York

Rare middle ear tumor

R Jadeed
1  Uniklinik RWTH Aachen University, Aachen
AK Otremba
2  Klinik für Hals-Nasen-Ohrenheilkunde und Plastische Kopf- und Halschirurgie, Uni, Aachen
T Braunschweig
3  RWTH Aachen University, Aachen
M Westhofen
2  Klinik für Hals-Nasen-Ohrenheilkunde und Plastische Kopf- und Halschirurgie, Uni, Aachen
› Author Affiliations
Further Information

Publication History

Publication Date:
18 April 2018 (online)



Carcinoid tumors of the middle ear are very rare and belong to neuroendocrine tumors. It has been classified as a indolent malignancy (well-differentiated neuroendocrine carcinoma). In the literature since Murphy et al. 1980 only about 54 cases were published.

Case report:

We report a 37-year-old patient who presented with a recurrent cholesteatoma at the richt ear.

The patient reported a recurrent otorrhea and a hearing loss on the right side. In audiometry showed a combined hearing loss with moderate inner ear involvement. Furthermore, a tympanoplasty type IIIa with radical cavity revision, myringotomy and long-term ventilation tube Insertion performed on the right ear. Intraoperatively showed a signal polyp in the tympanic cavity as well as a recurrent cholesteatoma with bone destruction.

Histological examination demonstrated a neuroendocrine adenoma of the middle ear (signal polyp biopsy) and a cholesteatoma. Immunohistochemical evaluation revealed a predominantly negative immune response for markers CK7 and p63. The mitosis rate per 2 square millimeters was less than 1 mitosis.

Postoperative staging did not reveal any cervical or distant metastases of the tumor, so further neck dissection was not required.


Complete surgical removal of the tumor is considered the treatment of choice for treating neuroendocrine tumors of the middle ear for primary and metastatic carcinoids. Tympanomastoidectomy or radical mastoidectomy is then recommended.

A modified radical or selective neck dissection is used to remove cervical lymphoid metastases in metastatic cases.