Carcinoma Ex Pleomorphic Adenoma of the Uvula - Case Report

Joana Borges Da Costa,Delfim Duarte,Miguel Viana,Ana Isabel Goncalves,Andre Carcao,Joana Santos

doi:10.37871/jbres1274

Abstract

Introduction: Carcinoma Ex Pleomorphic Adenoma (CEPA) results from the malignant transformation of a benign tumor of the Salivary Glands (SG), the Pleomorphic Adenoma (PA). PA is considered the most common salivary tumor with a 5% risk of malignant transformation and its excision is recommended. CEPA is a rare tumor, corresponding to 3.6% of all salivary tumors and 11.6% of all GS carcinomas. About 18% of CEPAs affect minor SG, with the palate being the most common location. The present work serves to describe a case of a CEPA of the Uvula Minor SG (UMSG). Case Report: We present a case report of a 57-year-old patient, with no relevant medical history, referred to the ENT consultation due to the appearance and progressive growth of a painless uvula lesion. The objective ENT examination showed a 15 mm ulcerative-vegetating lesion with apparent origin on the posterior face of the uvula. The lesion was biopsied and histopathological examination identified the presence of a neoplasm of the minor SG, probably NOS adenocarcinoma. The patient underwent Computed Tomography (CT) scan that showed an irregularity of the uvula, with no signs of invasion of the remaining soft palate, without other significant pharyngo-laryngeal changes. The patient underwent partial pharyngectomy and bilateral selective cervical ganglion dissection, and the histopathology of the surgical specimen confirmed that it was an invasive CEPA, the malignant component of the tumor corresponding to a NOS adenocarcinoma of the UMSG. The patient has been followed up in the ENT consultation, with no signs so far of loco-regional recurrence. Discussion/Conclusion: In the presented case, the patient probably developed an undiagnosed PA that had become malignant over time. Given that it is a poor prognosis neoplasm, it’s essential that the ENT specialists are aware of this disease, in order to facilitate and anticipate the diagnosis and treatment as much as possible.

Highlights

Carcinoma Ex Pleomorphic Adenoma (CEPA) results from the malignant transformation of a benign tumor of the Salivary Glands (SG), the Pleomorphic Adenoma (PA)
Patients with CEPA have a poor prognosis but an accurate diagnosis and aggressive surgical management of the tumor may increase the survival rates [9,14] Since the CEPA clinical presentation are quite similar to a PA, it is important that clinicians maintain a high level of clinical suspicion, which can be challenging considering the rarity of this cancer
Carcinomas arising from PA may present a wide morphological spectrum but, in most cases, CEPA is usually a high-grade adenocarcinoma or undifferentiated carcinoma that characteristically shows as histological features a capsule invasion, haemorrhage and necrosis alternating with areas with PA benign classical features [17]

Summary

INTRODUCTION

Carcinoma Ex Pleomorphic Adenoma (CEPA) is a rare and aggressive epithelial tumor that arises from the malignant transformation of a benign Pleomorphic Adenoma (PA) [1,2,3,4], which is the most common Salivary Glands (SG) tumor, representing 45-74% of all salivary tumors and about 40% of Minor SG (MSG) tumors [5,6]. The most common clinical presentation of CEPA is the appearance of a bulky, slow-growing, painless mass, in the majority of cases in the parotid or submandibular glands [9]. We describe the clinical and histopathological aspects in a case of a CEPA of the uvula MSG. The patient remains asymptomatic and with no signs of locoregional or distant recurrence of the pathology (Figure 8)

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DISCUSSION

CONCLUSION