Skull Base Adenoid Cystic Carcinoma – Clinical Series and Outcomes

Background: From the first description as 'cylindroma' 160 years back by Robin to the current era of advanced molecular genetics, we have come long way in understanding pathophysiology, molecular biology, patterns of spread of adenoid cystic carcinomas. Although there are lots of questions that still need to be answered, few aspects completely clear about these tumors are the progressive, indolent, relentless growth of these tumors with multiple local recurrences, peri neural spread and dismal 20year survival. Diffuse infiltration, peri neural spread along the skull base with difficult total resection has been attributed as the cause of grave prognosis in skull base adenoid cystic carcinomas. In our poster we are presenting 4 interesting cases of skull base adenoid cystic carcinomas managed in our institute that highlight importance of team approach, aggressive management, and long term follow up.

Methods: We retrospectively reviewed the charts of 4 patients with adenoid cystic carcinomas involving skull base. Data on patient demographics, clinical presentation, radiology, pathology, modality of treatment, complications and survival is presented.

Case Series: Case 1–39 years. old female, presented with acute vision loss and retro-orbital headache and facial weakness. Diagnosed in 2005 as nasopharyngeal adenoid cystic, extending to orbital apex, skull base infra temporal fossa, treated with craniofacial resection and radiation. Some improvement in vision was noticed post-op. Recurrence involving mandible in 2005. Treated with surgery followed by radiotherapy. Alive and tumor free for 8 years.

Case 2–63 years. old female presented with headaches, nasopharyngeal adenoid cystic extending to sphenoid and skull base diagnosed in 2002. Initial endoscopic resection followed by anterolateral craniofacial resection in 2003 and post op radiotherapy. Recurrence in 2010, another endoscopic resection. Radiotherapy in 2011 for another recurrence. Alive but still has active disease.

Case 3–51yrs old female with headaches had naso septal adenoid cystic carcinoma, treated with wide resection and radiotherapy in 2004. Developed metastasis in cervical, thoracic, lumbar spine in 2009. Treated with stereotactic radiosurgery to spine. No recurrence of primary. Alive with active metastasis.

Case 4–63yrs old female presented with unilateral vision loss and facial numbness. Scans and endoscopic biopsy in January 2013 confirmed adenoid cystic carcinoma of posterior nasal cavity, ethmoids, sphenoid and central skull base. Trans cranial, Trans nasal endoscopic excision and orbital decompression were done. Athough vision improved post-op, patient suffered a stroke after discharge from the hospital. She underwent radiotherapy. She is alive going for rehabilitation but still has residual disease.

Conclusion: Aggressive surgical intervention, adjuvant treatment, good rehabilitation and long term follow up are essential components of the management protocol for these complex tumors. Surgical intervention has a role in palliative improvement of local symptoms like vision and pain. All patients had severe pain so pain team plays a critical part in the management as well. In cases of complications after surgical or non surgical management we cannot stress enough the importance of good rehabilitation team.