Adenoid cystic carcinoma of the orbit: a single institute experience

Abstract

Abstract Introduction/Objective Adenoid cystic carcinoma (ADCC) is a malignant neoplasm usually arising from the major and minor salivary glands. Orbital ADCC arising from the lacrimal gland is rare. They present with morphology and immunohistochemical properties identical to their salivary gland counterparts. Due to their rarity, orbital ADCCs are poorly studied. Here, we describe our institutional experience of diagnosing ADCC in orbit. Methods/Case Report We queried the electronic laboratory information system for primary tumors arising in the orbit that resulted in the diagnosis of ADCC in the last twenty years. Clinical history, radiology, and immunohistochemical profile were obtained and summarized for all cases. Results (if a Case Study enter NA) Eight cases were with the diagnosis of orbital ADCC. The age at diagnosis ranged between 38 to 79 years, with most patients diagnosed between 41 to 50 years. The male to female ratio was 1:1. The tumor size varied between 1.5 to 8.7 cm. Most tumors were graded as grade III and showed perineural invasion. Five out of eight tumors showed predominantly solid architecture. The immunohistochemical pattern was similar to salivary gland ADCC, with one case showing MYB rearrangement. Conclusion Our data suggest that orbital ADCC closely resembles their salivary gland counterparts. Although small, our study broadens the general understanding of this rare group of malignancies.