Tumors originating from ceruminous glands are rare lesions of the external auditory canal. The lack of specific clinical and radiological signs makes their diagnosis challenging. We report the case of an exceptionally rare benign tumor, a syringocystadenoma papilliferum (SCAP), in an atypical location in the bony segment of the external auditory canal with uncommon clinical signs. The special traits of the case included the following: the most lateral component of the tumor was macroscopically cystic and a granular myringitis with an obstructing keratin mass plug was observed behind the mass. The clinical, audiological, radiological, and histological characteristics of the neoplasm are consequently presented. Intraoperative diagnosis of the epidermal cyst was proposed. The final diagnosis of SCAP was determined only by histological analysis after the surgical excision. The educational aspects of the case are critically discussed. 1. Introduction Syringocystadenoma papilliferum (SCAP) was first described in the dermatological literature in the beginning of the 20th century as a “naevus syringadenomatosus papilliferus” [1]. Occasionally, this type of tumor may arise in the external auditory canal (EAC). The masses in this location can be a real diagnostic challenge because of a nonspecific clinical presentation and the lack of experience of the clinician or pathologist [2]. SCAP is an extremely rare benign tumor that originates from modified apocrine sweat (ceruminous) glands with extensive papillary growth of epithelial elements down into dermis [3]. In 1894, Haugh described the first ceruminous gland tumor of the cartilaginous segment of the EAC [4]. He proposed the term ceruminoma. Later, Wetli et al. tried to classify these tumors into four groups: adenoma, pleomorphic adenoma, adenoid cystic carcinoma, and adenocarcinoma [5]. According to the World Health Organization, the ceruminous gland neoplasms are currently classified as benign ceruminous adenoma, chondroid syringoma, SCAP and malignant adenocarcinoma, adenoid cystic carcinoma, and mucoepidermoid carcinoma [6]. To date, fewer than 150 case reports of ceruminous gland tumors originating in the EAC can be found in the literature [4]. Among them, only 11 cases of SCAP have been described [7]. In this report, we present a case with an atypical clinical presentation. As far as we know, this is the first case of an SCAP occurring simultaneously with chronic granular external otitis and myringitis and a keratin mass plug of the bony part of the EAC. Diagnostic modalities and the problem of
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