A Young Woman with a Cystic Mass in the External Auditory Canal

Abstract

Introduction: Adenoid cystic carcinoma (ACC) is a malignancy that primarily affects the salivary glands. Primary cutaneous ACC (PCACC) is a rare variant that arises from ceruminous glands of the ear and other glands of apocrine origin. Case Presentation: We presented a case of a 26-year-old woman evaluated for a slow-growing mass in her ear for several years that was painful with bloody discharge. Physical exam demonstrated a subcutaneous nodule with overlying telangiectasias protruding from the external auditory canal. Excisional biopsy revealed a dermal proliferation of basaloid cells forming islands in a cribriform architecture. Discussion: PCACC grossly mimics other cysts and tumors, so diagnosis must be histologically confirmed. Basal cell carcinoma, mucinous carcinoma, and spiradenoma can mimic PCACC on microscopy, making differentiation important. PCACC is not associated with common risk factors for malignancy, thus it is important to include on the differential with any auricular tumor presenting as a pearly nodule with blue and purple hues. Despite treatment with wide local excision, postoperative radiation, and less commonly chemotherapy, recurrence rates are high. Conclusion: It is important to accurately diagnose PCACC, due to the need for long term follow up, as these tumors commonly recur decades later. This case contributes to the growing literature how PCACC can present and how the treatment will shape the trajectory of the patient’s follow up, as there is currently no consensus on definitive treatment.