Mammary Analogue Secretory Carcinoma of Submandibular gland

INTRODUCTION: Mammary Analogue Secretory Carcinoma of salivary glands (MASC) is a low-grade carcinoma of salivary glands of the head-neck region. It bears histological resemblance to Secretory Carcinoma of the breast and Acinic Cell Carcinoma (ACC) of the parotid gland. Its clinical behaviour and aggressiveness vary amongst individuals and experience in MASC of the submandibular gland are limited. CASE REPORT: We report a 16-year-old female with binary neck swelling in the submandibular region. The hard swelling in the submandibular region was a MASC and the soft cystic mass was a synchronous congenital lymphatic cyst in the neck. We report two unusual features, an extremely rare involvement of MASC of submandibular salivary gland and the presence of a congenital lymphatic cyst in the area adjacent to the main tumour mass. Treatment was done by surgical excision of both the neck masses in-toto and ipsilateral selective neck dissection (Level I-IV). CONCLUSIONS: While MASC's histological pattern has been described in previous studies, its clinical picture is rarely documented. This report aims to shed light on the clinical presentation of this under-diagnosed entity and the aggressive management protocol required during preoperative workup, intraoperative disease clearance and post-operative follow up of such patients. MASC of the submandibular salivary gland is an uncommon cause of neck swelling in the adolescent age group, but due to its occasional aggressive nature, should be borne in mind as a possible differential diagnosis of salivary gland tumours.