# Introduction eruminous glands are specialized apocrine glands located in the cartilaginous part of the external auditory canal (EAC). Primary malignant tumors arising from these glands are infrequent, and they may present a diagnostic dilemma because of their varied clinical and histological manifestations. Here we report a patient with an extended ceruminous adenocarcinoma. This article highlights the importance of early diagnosis for optimal management of this pathology. # II. # Case Report A 37 years old man, without a history of diabetes mellitus or immunesupression, presented to our head and neck department with one (01) year history of left purulent otorrhea with the decreased hearing of the left ear and facial paralysis without vertigo. He denied experiencing dizziness, tinnitus, vertigo or earache. On physical examination, there was a retro-auricular fistula with local inflammatory signs. Otoscopy showed a large polyp on the cartilaginous part of the left EAC, with mucopurulent discharge in the EAC. The tympanic membrane was not visible. Audiometry revealed conductive hearing loss on the left. The 4-frequency pure tone average was 50 dB for air conduction and 10 dB for bone conduction. We didn't note any palpable cervical lymph nodes. Using retro-auricular approach, the mass of the EAC was removed (figure3), and histological analysis showed a poorly differentiated carcinomatous proliferation primarily suggestive of a neuroendocrine tumor. We completed with immunohistochemistry that showed an intense and diffuse positivity of cytokeratin 7 and CD117. The final diagnosis was ceruminous adenocarcinomas. # 12 Year 2019 Because of the extension of the mass, the decision was to propose for intensity-modulated radiotherapy; he received a total of 60 Gy. # Global # III. # Discussion Ceruminous adenocarcinomas are a rare malignant tumor that arises from the ceruminous gland found in the cartilaginous part of EAC. It is the 2 nd most common type of carcinoma arising from ceruminous-glands [1]. Most affected patients are in their fifth or sixth decades of life and average age at diagnosis of 48 years. Its incidence is equal between males and females [1,2]. There are no known risk factors for the development of these tumors, although the otitis externawas implicated in animal models [2]. Ceruminous adenocarcinoma exhibits a prolonged subclinical phase, often lasting years, before presentation [3]. The most common symptoms reported in the literature are: mass of the EAC, hearing changes, otorrhea, bleeding, headaches, equilibrium changes, pain and facial paralysis, and less frequently lymphadenopathy or tinnitus. Although cervical lymph node metastasis is uncommon, distant metastasis to the bones, lungs, and brain have been described [4]. Imaging plays important role in predicting and delineating the EAC tumor extent. Axial and coronal Computed tomographic scans help in detecting bony erosion and disease extension as well as in excluding a primary parotid or middle ear tumor [3]. Histologically, ceruminous adenocarcinoma is classified as high-grade and low-grade tumors based on the extent of glandular differentiation and the proportion of solid areas [6,8]. Low-grade tumors may mimic the histopathologic features of the benign ceruminous adenomas but are distinguished from the latter by the presence of stromal invasion and a desmoplastic stromal response. High-grade adenocarcinomas extensively infiltrate the surrounding tissues as irregular glands, cords, and sheets of overtly malignant cells. You marked nuclear pleomorphism and abundant mitotic figures. High-grade tumors may exhibit minimal or no evidence of apocrine derivation and needs to be distinguished from metastatic adenocarcinoma originating from other sites [6]. The mainstay of management of this tumor is combined surgery and radiotherapy. Radiotherapy has a limited role in treatment as a conjunctive therapy with surgery and in cases of distant metastasis. As a surgical procedure, Hicks has proposed initial en bloc surgical resection. And then, if tumor have an extension to the middle ear, resection of the temporal bone and adjacent structures is necessary [4,7]. In our case, there is a tumor extension to the carotid canal, complete resection was impossible. Surgical margin positivity at initial surgery, bone extension, perineural invasion, and local recurrence seem to be prognostic factors for cases in the literature. Chemotherapy did not prevent the development of distant metastasis. Prognosis may be difficult to predict, 25% of patients with ceruminous adenocarcinoma died of disease a mean of 2 years after presentation. The disease-specific mortality is 35% [8]. IV. # Conclusion Ceruminous adenocarcinoma is a rare malignant subtype of ceruminous gland neoplasm; its diagnosis is most often challenging, comprehensive evaluation including physical examination, multimodal imaging studies and thorough histologic analysis of an adequate biopsy that includes surrounding tissue is necessary to arrive at the correct diagnosis. Prognosis may be difficult to predict but complete surgical excision coupled with radiation yields the best long-term prognosis. 1![Figure 1: CT scan showing the collection in the left EAC, with osteolysis and osteitis of the temporal bone (Arrows).](image-2.png "Figure 1 :") 2![Figure 2: Per-operative view showing the tumor arising fronEAC (Arrow).](image-3.png "Figure 2 :") * Ceruminous gland carcinomas: a clinicopathologic and immunophenotypic study of 17 cases NCrain B LNelson E LBarnes LDThompson Head Neck Pathol 3 1 2009 * Ceruminous adenocarcinoma with extensive parotid, cervical, and distant metastases: case report and review of literature J CJan C PWang P CKwan 10.1001/archotol.134.6.663 Arch Otolaryngol Head Neck Surg 134 2008 * Ceruminous adenocarcinoma: a rare tumour of the external auditory canal S LSoon MBullock MEPrince J Otolaryngol 30 6 2001 * Gülben Erdem Huq. Ceruminous Adenocarcinoma of External Auditory Canal: A Case Report SuatBilici F?ratOnur AhmetVolkanSünter ÖzgürYi?it 10.5152/iao.2016.1249 J Int Adv Otol 2016 * A case of ceruminous adenocarcinoma of the external auditory canal presenting as an aural polyp CWKim American Journal of Otolaryngology-Head and Neck Medicine and Surgery 29 2008 * Atlas of Tumor Pathology. 3 rd series, fascicle 26 S EMills M JGaffey HFFrierson Armed Forces Institute of Pathology Roasi J, Sobin LH 2000 Tumors of the ear * Tumors arising from the glandular structures of the external auditory canal GHicks Laryngoscope 93 1983 * Ceruminous Neoplasms of the Ear Head and Neck Pathology PriyadharsiniNagarajan 10.1007/s12105-018-0909-3 2017