Multiple Metastases of the Adenoid Cystic Carcinoma Associated with Intracranial Metastasis

ABSTRACT

OBJECTIVE: Adenoid cystic carcinoma is a relatively slowly growing malignant tumor. Probably at least 40-50% of patients eventually develop distant metastases. We present the natural history and treatment modality of this malignancy from our experience and review of literature. METHODS: We report a case of a 30-year-old man who complained of a headache, facial pain and hearing disturbance in the right ear. Physical examination revealed soft, protruded mass and narrowed external auditory canal. The cranial MRI showed a well defined mass in the extradural middle cranial fossa. RESULTS: The patient underwent subtotal resection of the lesion after tumor embolization was performed. The pathological diagnosis was adenoid cystic carcinoma. The patient received postoperative radiation therapy(56Gy) resulting in a complete neurological recovery. Fifteen months later, the patient was readmitted for severe back pain. Bone scan disclosed hot uptakes at the upper cervical spine, the 4th lumbar vertebra, and the pelvic area. These areas were irradiated(40Gy) and the presenting symptoms were relieved. Twenty months later, he complained of respiratory difficulty and was found to have a multiple nodule(s) in the lung. He was given 10 cycles of chemotherapy but discharged because of unresponsivencess and development of new lesions. Brain MRI and bone scan were checked 10 months after his discharge due to more aggravated lung metastasis although local tumor was controlled. Adjuvant radiation therapy(18Gy) was performed and he continued to be functional independently, although he was no longer working as a registered nurse. Two months later, dyspnea and hemoptysis were more improved and chest X-ray showed decreased mass. During the follow up period, he complained of intercostal pain, chest X-ray showed more aggregated lung mass. Chest CT scan showed multiple lung metastases and liver metastasis. In spite of adjuvant radiotherapy, the patient died of multiple systemic metastases 47 months after the first operation. CONCLUSION: Surgery is essential in the treatment of adenoid cystic carcinoma. Radiotherapy has gained acceptance as a palliative therapy, as it reduces tumor bulk and relieves symptoms. The combined treatment do not, however, prevent further recurrence and distance metastasis.