Case report: Adenoid cystic carcinoma of the lacrimal gland with intracranial invasion and single lung metastasis

Case presentation: A 52-year-old woman had right eye ptosis about 2 years prior evolving with worsening of symptoms during the last months associated a lacrimal mass presented with exophthalmos, local pain and visual disturbance in the right eye. Orbital MRI showed a well-circumscribed mass at lateral aspect of the right orbit with bone involvement that was extended to periorbital tissue, temporal muscle, cavernous sinus extending to the infratemporal fossa and clivus. A biopsy was performed which confirmed the histological diagnosis as high grade adenoid cystic carcinoma of the lacrimal gland. Surgical approach was discarded due to involvement of the central nervous system. The patient was referred for radiosensitizing chemotherapy, with cisplatin, concomitant with radiotherapy. The patient underwent radiation therapy with two different planning target volumes: 60 Gy to extracranial portion and 30 Gy to intracranial portion. After completing the proposed treatment, patient showed considerable improvement in pain and staging tests were performed showing important primary lesion reduction and evidence of a single mass measured 4 × 3 cm in axial diameter in the left lung. Computed tomography-guided percutaneous biopsy of the chest confirmed that it was a metastatic focus. Currently, the patient is scheduled for surgical resection of the residual primary lesion and treatment for oligometastatic disease. Discussion: Lacrimal gland adenoid cystic carcinoma accounts for 25–40% of all epithelial tumors of the lacrimal gland and has an aggressive behavior and is associated with significant morbidity and mortality. The traditional surgical treatment is orbital exenteration followed by radiotherapy. In our case, the lesion was unresectable because the mass presented into intracranial invasion. Chemotherapy may occasionally be clinically efficacious for the recurrence of adenoid cystic carcinoma. As the lesion was unresectable, our patient received radiotherapy concomitantly with exclusive chemotherapy and was presented a significant reduction in the mass. Despite presenting distant metastasis at the end of the treatment, due to the excellent response, resection of the residual primary lesion and treatment of the single lung metastasis will be scheduled. Final comments: To our knowledge, lacrimal gland adenoid cystic carcinoma seems to be associated with high rates of local recurrence, metastases and death. Therefore, the instituted treatment must be carried as soon as possible in order to control the disease.