Recurred Adenoid Cystic Carcinoma of Lacrimal Gland with Aggressive Local Invasion to the Maxillary Bone Marrow without Increased Uptake in PET-CT

Dear Editor, This report describes a patient with locally aggressive ad-enoid cystic carcinoma (ACC) of the lacrimal gland that recurred after neoadjuvant intra-arterial chemotherapy (IAC), local surgery and postoperative radiotherapy. A 47-year-old female presented with painless swelling of her right eyelid that had persisted for 3 months. Physical examination revealed 3-mm exophthalmos of the right eye. Orbit magnetic resonance imaging (MRI) with contrast revealed an approximately 3-cm-sized enhancing lesion in the right lacri-mal gland with bony invasion (Fig. 1A). Incisional biopsy verified ACC. Neoadjuvant intracarotid adriamycin and cis-platinum was started, but the patient was switched to the intravenous regimen due to facial swelling and tenderness. After two chemoreduction cycles, the patient underwent orbital exenteration. Because the resected margin of the lateral exenterated orbit and zygoma were positive for carcinoma, additional lateral zygoma bone was removed (Fig. 1B). The pathology report verified T4bN0M0 stage ACC, cribriform type, with lymphovascular invasion (Fig. 1C). The patient then received postoperative 59.4 Gy radio-therapy. An MRI taken 1 year after exenteration revealed an approximately 7-mm, focal enhancing lesion in the right anterior maxilla bone, suggestive of possible tumor recurrence. However, positron emission tomography-computed tomography (PET-CT) did not show increased uptake of fludeoxyglucose (FDG) (Fig. 1D). The patient refused to have a bone biopsy. A repeat MRI performed 2 years after surgery indicated that there was an increase in the extent of the enhancing lesion inferior to the right orbit and right pterygomaxillary fissure and suspicious tumor spread along the right foramen rotundum, but again no increased FDG uptake was observed on PET-CT (Fig. 1E). A bone biopsy was recommended which the patient refused. A PET-CT taken 3 years postoperatively again showed a stable disease state, however, the patient presented with an elevated pig-mented mass along the inferolateral margin of the exenter-ated orbit after 6 months. An MRI revealed a markedly increased infiltrating mass along the entire maxillary sinus wall with perineural spread to the cavernous sinus via the foramen rotundum. At that time, PET-CT finally showed consistent findings of high FDG uptake (Fig. 1F). An exci-sional biopsy of the elevated pigmented lesion (Fig. 1G) confirmed the pathology as recurred ACC (Fig. 1H). The patient was referred to the head and neck surgery department and a salvage operation was planned, however the patient refused to undergo the operation and she did not return until she presented to the emergency room in a confused mental status with …