Case report
Middle ear adenoma with neuroendocrine differentiation (MEA-ND) in the pediatric population

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Abstract

Middle ear adenoma with neuroendocrine differentiation is an uncommon tumor of the tympanic cavity. The terminology of this entity has suffered due to a poor understanding of the differentiation of these neoplasms, and has included both “adenoma” as well as “carcinoid tumor.” Immunohistochemical techniques have helped clarify that these tumors all share elements of neuroendocrine differentiation. Occurrences of this tumor in the pediatric population are extremely rare. In the world literature, only three pediatric cases of middle ear adenoma with neuroendocrine differentiation have been described. We report the youngest case to date of middle ear adenoma with neuroendocrine differentiation, in a 13-year-old boy. We discuss the differential diagnosis, surgical management, and pathology of this entity.

Introduction

Middle ear adenoma with neuroendocrine differentiation (MEA-ND) is a rare tumor that was first reported in 1980. It can be difficult to classify histologically as middle ear adenomas may show purely epithelial (adenoma), purely endocrine (carcinoid) or hybrid differentiation [1]. In spite of this variability, some authors suggest that all middle ear adenomas have elements of neuroendocrine differentiation. Support for this viewpoint comes from immunohistochemical studies with neuroendocrine-specific markers. Torske and Thompson, for example, reviewed 48 middle ear adenomas and demonstrated chromogranin positivity in all, implying some neuroendocrine differentiation despite indistinguishable morphology on routine stains [2].

Historically, MEA-ND has been referred to as a carcinoid tumor, reflecting its histologic similarity to intestinal carcinoids, which are classified by site of origin as foregut, midgut or hindgut lesions [3], [4], [5]. Middle ear carcinoids are associated with the foregut tumors, which generally produce only small amounts of peptide hormones. As a consequence, the typical carcinoid syndrome of cramping, flushing and diarrhea is absent in MEA-ND.

MEA-ND was considered an indolent lesion until 1999 when Mooney et al. [6] reported the first case with metastases. In this paper, we present the youngest case to date of MEA-ND in a 13-year-old boy. We discuss the surgical management of this patient, including findings at the original surgery and on re-exploration one year later. We also review the pathology and differential diagnosis of this tumor.

Section snippets

Case report

A 13-year-old boy followed for chronic otitis media with effusion presented with a left tympanic membrane perforation. His past medical history was significant for ventilation tube placement on several occasions and for recurrent tonsillitis necessitating tonsillectomy at age 11 years.

Examination of the left tympanic membrane was remarkable for a perforation in the attic region and a posterior-superior quadrant white mass. The right tympanic membrane examination was significant only for

Usual presentation

MEA-ND usually presents with hearing loss, as documented by Nyrop et al. in their 1994 review of 30 cases in which nearly all patients had progressive conductive hearing loss. Other complaints on presentation may include tinnitus, aural fulness, otorrhea, and otalgia. Transient facial paresis has also been reported. Age at diagnosis has ranged from 16 to 59 years [7].

On otoscopy, erythema or lateral bulging of an intact tympanic membrane is typical, and at surgery, MEA-ND appears solid and

Conflict of interest

There is no financial or other relationship that might lead to a conflict of interest for any authors.

This study was funded by departmental resources.

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    This work was originally presented at the meeting of the American Society of Pediatric Otolaryngology at COSM, Chicago, IL, May 20–22, 2006.

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