A CASE OF SECONDARY ACQUIRED CHOLESTEATOMA WITH CARCINOID TUMOR OF THE MIDDLE EAR
Abstract
Secondary acquired cholesteatoma may occur from trauma, surgical manipulation of the drum or acute otitis media. We presented a 73-year-old lady who had secondary acquired concholesteatoma with carcinoid tumor of the middle ear. While she underwent the myringotomy for right otitis media at another clinic, her hearing was not improved, she therefore visited our department. A perforation at the antero-inferior quadrant of the right tympanic membrane and a white mass both in the cavity of right middle ear and around of the edge of the perforation were observed. We diagnosed this case as secondary acquired cholesteatoma for these finding and performed the tympanoplasty. Most part of the tympanic membrane was adhered to the wall of the middle tympanum. A white mass was localized to the tympanic membrane and another yellowish mass was observed in the middle tympanum. The pathological diagnoses of the white and yellowish masses were cholesteatoma and carcinoid tumor, respectively. In this case, we thought that the blockade of the eustachian tube by carcinoid tumor caused otitis media with effusion, and secondary acquired cholesteatoma appeared at the edge of the perforation after the myringotomy. It is suggested that the careful evaluation would be needed when we see a case of secondary acquired cholesteatoma, since it might be accompanied by be other lesion such as carcinoid tumor.
Case
A 73 year-old lady visited the clinic located near her house, since she had suffed from right hearing loss. She was diagnosed as right otitis media with effusion and underwent the myringotomy twice. However, her hearing was not improved. She therefore visited our department again. On the otoscopic examination, a perforation was observed at the antero-inferior quadrant of the right tympanic membrane (Fig. 1). The white masses were also observed both in the cavity of right middle ear and around the edge of the perforation (Fig. 1). An au-diogram revealed mixed hearing loss, a pure-tone average of 76.7 dB on right ear (Fig. 2). The temporal bone computed tomographic scan (CT) revealed the soft tissue density area extending from right middle tympanum to the eustachian tube without any destruction of ossicles (Fig. 3). We diagnosed this case as secondary acquired cholesteatoma for these finding and planned the tympanoplasty. Most part of the tympanic membrane that we thought the perforation was retraction and adhered to the wall of the middle tympanum (Fig. 4A). A white mass was localized just behind the tympanic membrane and another yellowish mass was observed in the middle tympanum (Fig. 4B). Since the rapid pathological diagnosis of yellowish mass was low-malignant potential tumor, these masses were completely removed with a part of the tympanic membrane and the repair of the eardrum was not performed. The pathogenic examination of yellowish tumor revealed the proliferation of cuboidal cells without any nuclear irregularity arranged in glandular pattern (Fig. 5). Immnohistochemical
Fig. 1. Otoscopic examination. A perforation at the antero-inferior quadrant of the right tympanic membrane and the white masses both in the cavity of right middle ear and around the edge of the perforation were observed.
Fig. 2. Audiogram. The mixed hearing loss, a pure-tone average of 76.7 dB, on right ear was revealed.
Fig. 3. CT finding. The soft tissue density area extending from right middle tympanum to the eustachian tube without any destruction of ossicles was revealed.
stains for chromogranin and symaptophysin were positive. S-100 protein, Ki-67 and p53 were negative (Fig. 6). The pathological diagnoses of the white and yellowish tumors were cholesteatoma and carcinoid tumor, respectively. The recurrence finding was not observed about for 3 years after the operation (Fig. 7).
Discussion
Cholesteatoma is classified into congenital and acquired. Furthermore, acquired cholesteatoma is divided into primary and secondary according to the cause. Secondary acquired cholesteatoma may occur from a trauma,
Fig. 4. Otoscopic finding in the operation. A: The retraction at the antero-inferior quadrant of the right tympanic membrane was observed. B : The yellowish mass in the middle tympanum was observed.
Fig. 5. Pathological finding of yellowish tumor, hematoxylin-eosin stain (×400). The proliferation of cuboidal cells without any nuclear irregularity arranged in glandular pattern was observed.
surgical manipulation of the drum or acute otitis media. Several pathogenic theories to induce secondary acquired cholesteatoma have been reported follow as 1) implant theory1 2) metaplasia theory,2 3) epithelial invasion theory.2 It has been reported that the incidence of secondary acquired cholesteatoma following the myringotomy and the ventilation tube insertion in children was ranged from 0.48 to 1.1%.1,3,4 Carcinoid tumor was one of neuroendocrine tumors and occurs rarely in the middle ear.5 Carcinoid tumor was observed mostly around ossicles and sometimes extending to the eustachian tube such as this case.6 Any case of secondary acquired cholesteatoma with carcinoid tumor has been reported ever. In the present case, we thought the blockade of the eustachian tube by carcinoid tumor caused otitis media with effusion first and secondary acquired cholesteatoma then appeared at the edge of the perforation after the myringotomy.
Conclusion
A case of secondary acquired cholesteatoma with carcinoid tumor was presented. We thought the blockade of the eustachian tube by carcinoid tumor caused otitis media with effusion first, and secondary acquired cholesteatoma then appeared at the edge of the perforation after the myringotomy. It is suggested that the careful evaluation would be needed when we see a case of secondary acquired cholesteatoma, since it might be accompanied by be other lesion such as carcinoid tumor.
Fig. 6. Immnohischemical stain of yellowish tumor. Symaptophysin was positive.
Fig. 7. Otoscopic examination after the operation
References
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2.Kazuo M, Tetsuaki K, Hideki T, et al. Study of cholesteatomas with central perforation of the eardrum – a new theory of secondary acquired cholesteatoma. Oto-Rhino-Laryngol, Tokyo 44:345–350, 2001
3.Golz A, Goldenberg D, Netzer A, Westerman LM, Westerman ST, Fradis M, Joachims HZ. Cholesteatomas associated with ventilation tube insertion. Arch Otolaryngol Head Neck Surg 125(7):754–757, 1999
4.Spilsbury K, Miller I, Semmens JB, Lannigan FJ. Factors associated with developing cholesteatoma: a study of 45,980 children with middle ear disease. Laryngoscope 120(3):625–30, 2010
5.Soga J. Carcinoids and their variant endocrinomas. An analysis of 11842 reported cases. J Exp Clin Cancer Res 22:517–530, 2003
6.Ramsey MJ, Nadol JB Jr, Pilch BZ, McKenna MJ. Carcinoid tumor of the middle ear: clinical features, recurrences, and metastases. Laryngoscope 115(9):1660–6, 2005
Address for correspondence: Naoko Sakuma, M.D., Department of Otolaryngology, Head and Neck Surgery, Yokohama City University School of Medicine, 3–9 Fukuura, Kanazawa-ku, Yokohama, Japan. n_sakuma@yokohama-cu.ac.jp
Cholesteatoma and Ear Surgery – An Update, pp. 417–420
Edited by Haruo Takahashi
2013 © Kugler Publications, Amsterdam, The Netherlands