Abstract

Introduction: adenoid cystic carcinoma account 1% for all cancers of the ear nose and throat. Despite slow growth, their recurrence is frequent. This study concerns 5 cases of cystic adenoid carcinoma with atypical ear nose and throat locations (outside the main salivary glands).
 Materials and methods: Our work is a study of 5 cases of cylindromes with atypical localizations collected at the ear nose throat and cervico-facial surgery department of Mohamed IV university hospital of Marrakesh: 1 case located at the external auditory meatus, 1 case at the infratemporal fossa, 1 case on the inside of the cheek, 1 case in the soft palate and one laryngotracheal case.
 Results and discussion: The management of adenoid cystic carcinoma remains delicate because of their insidious growth, their neurological tropism and their metastatic potential. These associated elements delay the diagnosis that is often made while those tumors are locally advanced, which can make surgery difficult, and be responsible of a lot of late recurrences. The treatment of these tumors has long relied on exclusive surgery. Cystic adenoid carcinomas were considered radio resistant. Retrospective studies have shown the benefit of treatment combining surgery and radiotherapy, in terms of local control and disease-free survival, compared to surgery or radiotherapy alone.
 Conclusion: The essential problem of adenoid cystic carcinoma remains the long-term control of the disease. Our results confirm that the gold-standard treatment should be both surgery and radiotherapy.

Highlights

  • Cystic adenoid or cylindroid carcinomas were first described by Robin and Laboulbene in 1852 [1]

  • Retrospective studies have shown the benefit of treatment combining surgery and radiotherapy, in terms of local control and disease-free survival, compared to surgery or radiotherapy alone

  • Our results confirm that the gold-standard treatment should be both surgery and radiotherapy

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Summary

Introduction

Cystic adenoid or cylindroid carcinomas were first described by Robin and Laboulbene in 1852 [1]. The cystic adenoid carcinomas are an infiltrating, very malignant tumor They are rare and developed usually at the expense of the salivary glands. They represent the main histological type of accessory salivary gland tumors and account for approximately 10-20% of all salivary gland tumors [2]. They account for approximately 1% of all ear, nose and throat cancers, it can be found more rarely in all sites that contain secretory glands (breast, cervix, colon, prostate ...) [3]. The combination of all these elements delays the diagnosis, which is often made when they are locally advanced, making surgery difficult, and late recurrences frequent The treatment of these tumors has long relied on exclusive surgery. Retrospective studies have shown the benefit of treatment combining surgery and radiotherapy, in terms of local control and disease-free survival, compared with surgery or radiotherapy alone [7]-[9]

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