Adenoid cystic carcinoma with intracranial extension

Abstract

Adenoid cystic carcinoma (cylindroma) usually arises from the salivary, lacrimal, or other exocrine glands, and is rarely encountered by neurosurgeons. The authors describe three cases involving intracranial extension. An intraorbital tumor in a 71-year-old man extended directly into the epidural space of the frontal base and destroyed the orbital roof. In a 53-year-old woman, the tumor arose from the area adjacent to the eustachian tube and invaded the Gasserian ganglion. In the third patient, a 58-year-old man, the tumor originated in the maxillary sinus and extended directly into the middle cranial fossa. In all these cases, the tumors were removed to the fullest extent possible. Although residual tumor was markedly reduced by radiation therapy, recurrence and metastases occurred within a few years. Thus, adenoid cystic carcinoma appears to be radiosensitive, but not curable by irradiation. In treating a recurrent tumor in one patient, we applied the so-called "two-route" chemotherapy (cisplatin and its antidote) in combination with radiation therapy. The tumor responded well to this therapy, although multiple pulmonary and bone metastases eventually led to the patient's death.

Stereotactic radiosurgery for skull base adenoid cystic carcinoma: A report of two cases

“…[ 4 ] The 5-year overall survival rate is approximately 90%; however, the cumulative risks of local recurrence and metastases are 40% and 40–60%, respectively. [ 3 , 4 , 6 , 7 , 9 , 11 , 20 , 21 ] Although, surgical resection is the mainstay primary treatment for ACC,[ 2 , 3 , 6 , 21 ] the main approach for ACC undergoing subtotal or partial resection is postoperative fractionated radiotherapy as a radical intent almost regardless of location. [ 1 , 2 , 16 , 22 ] Same applies to recurrent lesions, radiotherapy is the main approach with systemic treatment (chemo and targeted therapy) having a limited role.…”

“…[ 2 , 3 , 22 ] Metastatic ACC rarely occurs in the skull base; however, as the main tumor often extends alongside critical neurovascular structures, gross total resection with neurological preservation is challenging. [ 17 , 20 ]…”