Abstract

Fatima-Zahra Kahouadji*, Ouail Benzerouale, Hafsa Elouazzani, Nadia Cherradi, Saber Boutayeb, Ibrahim El Ghissassi, H. M’rabti and H. Errihani

ABSTRACT

Adenoid cystic carcinoma (ACC) of submandibular gland is a cancer rarely encountered in clinical practice. Here we presented ACC of submandibular gland in a 57 years old male discovered at a metastatic stage. Distant metastasis from submandibular ACC most commonly involves the lungs, and regional metastasis from submandibular ACC is more common compared to other major salivary glands due to the proximity of the draining lymph nodes. in the absence of metastasis, surgery and adjuvant radiotherapy are the mainstay of curative treatment. Systemic treatments are used for locally recurrent or metastatic disease. The best objective response rates were obtained with cisplatin-vinorelbine dual therapy. Monotherapy with targeted therapies could lead to prolonged stabilization. Two phases II immunotherapy trials were conducted with pembrolizumab, in patients with recurrent or metastatic ACC, one of which showed no objective response and the other is still ongoing. The aim of this case report is to present the particularities of this tumor and to provide a brief overview on systemic treatments for locally recurrent or metastatic diseases.