An aggressive papillary tumor of middle ear: A case report

Aggressive papillary tumors of the middle ear constitute a small percent of adenomatous middle ear tumors. Patients usually presentwith long-lasting tinnitus, hearing loss, and vertigo and occasionally facial paralysis. Computed tomography findings predominantlysuggest bone invasion. Thus, it is commonly misdiagnosed with chronic otitis media with a cholesteatoma. A 72-year-old manpresented with chronic ear pain and ear drainage. The patient had a 63 decibels (dB) mixed-type hearing loss and a mass lesion atthe temporal bone high resolution computed tomography. Explorative tympanotomy revealed a tumor with thick mucoid secretionand no bone erosion, for which an incisional biopsy revealed an adenoma. Radical modified mastoidectomy was performed andthe definite pathology result was compatible with an aggressive papillary tumor. The patient was free of disease at six months offollow-up.

___

1. Eby TL, Makek MS, Fisch U. Adenomas of the temporal bone. Ann Otol Rhinol Laryngol 1988;97:605-12.

2. el-Naggar AK, Pflatz M, Ordóñez NG, Batsakis JG. Tumors of the middle ear and endolymphatic sac. Pathol Annu 1994;29:199-231.

3. Gaffey MJ, Mills SE, Fechner RE, Intemann SR, Wick MR. Aggressive papillary middle-ear tumor. A clinicopathologic entity distinct from middle-ear adenoma. Am J Surg Pathol 1988;12:790-7.

4. Heffner DK. Low-grade adenocarcinoma of probable endolymphatic sac origin A clinicopathologic study of 20 cases. Cancer 1989;64:2292-302.

5. Michaels L, Beale T, Sandison A, Soucek S. Endolymphatic sac tumour. In: Barnes L, Eveson JW, Reichart P, Sidransky D, editors. Pathology and Genetics of Head and Neck Tumours. Lyon: IARC Press; 2005. p. 355-6.

6. Schipper J, Maier W, Rosahl SK, van Velthoven V, Berlis A, Boedeker CC, et al. Endolymphatic sac tumours: surgical management. J Otolaryngol 2006;35:387-94.

7. Chetty R, Kennedy M, Ezzat S, Asa SL. Pancreatic endocrine pathology in von Hippel-Lindau disease: an expanding spectrum of lesions. Endocr Pathol 2004;15:141-8.

8. Friedrich CA. Von Hippel-Lindau syndrome. A pleomorphic condition. Cancer 1999;86:2478-82.

9. Thompson LD. Update From the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Tumours of the Ear. Head Neck Pathol 2017;11:78-87.

10. Kawabata S, Hollander MC, Munasinghe JP, Brinster LR, Mercado-Matos JR, Li J, et al. Epidermal growth factor receptor as a novel molecular target for aggressive papillary tumors in the middle ear and temporal bone. Oncotarget 2015;6:11357-68.

11. Pollak A, Böhmer A, Spycher M, Fisch U. Are papillary adenomas endolymphatic sac tumors? Ann Otol Rhinol Laryngol 1995;104:613-9.

12. Elhefnawy NG. Aggressive low grade middle ear adenocarcinoma with multiple recurrences: a case report. Diagn Pathol 2011;6:62.

13. Arava S, Soumya RM, Chitragar S, Safaya R, Chandrashekhar SH, Thakar A. Papillary endolymphatic sac tumor: a case report. Case Rep Otolaryngol 2012;2012:163851.