An Unusual Presentation of Adenoid Cystic Carcinoma.

Kurren S Gill,Mark A Frattali

doi:10.1155/2015/826436

Abstract

Adenoid cystic carcinoma (ACC) is a relatively rare tumor of epithelial cell origin, most commonly arising from major salivary glands. It is uncommonly found outside the major or minor salivary glands and is especially rare when located in the nasal cavity. Diagnosis and treatment of ACC pose numerous challenges, partly due to its biological behavior of slow growth, high tendency of local recurrence, and perineural invasion. We present the case of a 67-year-old male with complaints of facial pain and swelling, with a CT scan showing a soft tissue mass extending from the right nasal cavity with osseous destruction. Biopsy revealed ACC with perineural invasion. ACC of the nasal cavity continues to pose diagnostic and therapeutic challenges to physicians. Because this rare pathology presents in a vague manner, early diagnosis requires a high index of suspicion for this disease and close follow-up care. Since ACC of the nasal cavity is seldom reported in the literature, it is our hope that reporting these rare instances as case reports will heighten physician awareness of this rare disease, allowing for early diagnosis and treatment.

Highlights

Adenoid cystic carcinoma (ACC) is a rare tumor of epithelial cell origin, comprising 1% of malignant tumors of the head and neck region [1]
We describe a case of ACC of the nasal cavity, present the clinical and radiologic features, and discuss treatment options, with a thorough review of the world literature
Presence of lymphadenopathy is uncommon since ACCs do not usually spread to regional lymph nodes

Summary

Introduction

ACC is a rare tumor of epithelial cell origin, comprising 1% of malignant tumors of the head and neck region [1]. The most common symptom is a slow growing mass followed by pain, and it has a relentless course with usually a fatal outcome It most commonly arises from major or minor salivary glands, contributing to 10% of salivary gland tumors [2]. It is rarely seen in the nasal cavity, wherein the most common site is the lateral nasal wall. ACC tends to spread locally through bony destruction and perineural and perivascular invasion It is characterized by high rates of local recurrence and distance metastasis, occurring as late as 10 to 20 years following initial treatment [4]. We describe a case of ACC of the nasal cavity, present the clinical and radiologic features, and discuss treatment options, with a thorough review of the world literature

Case Report

Findings

Discussion