title: Adenoid cystic carcinoma of the skull base: results with an aggressive multidisciplinary approach.
abstract: OBJECT Adenoid cystic carcinoma (ACC) is a locally aggressive tumor of salivary gland origin. Little data exist to guide treatment when this tumor extends to involve the structures of the skull base. METHODS Fifty-one patients with a diagnosis of ACC affecting the skull base were identified from a prospective database at MD Anderson Cancer Center (from 1992 to 2010). RESULTS Median follow-up for study patients was 6.75 years. The 5- and 10-year overall survival (OS) rates were 78% and 50%, respectively. Sixty-six percent of patients had progression of their disease. The 5- and 10-year progression-free survival (PFS) rates were 46.7% and 21.0%, respectively. Gross-total resection was achieved in 75% of patients, with 49% having microscopically negative margins at the time of first operation. On univariate analysis, resections with microscopically negative margins were associated with a significant OS advantage (20.1 ± 3.3 years) compared with resections that left residual disease, even if microscopic (10.3 ± 1.6 years, p = 0.035). In patients who underwent reoperation, the effect persisted, with improved OS in those with negative margins (21.4 ± 0.0 vs 16.7 ± 4.0 years, p = 0.06). The use of adjuvant radiotherapy was associated with an OS advantage (16.2 ± 2.5 vs 5.5 ± 2.2 years, p = 0.03) at initial diagnosis and improved PFS (7.8 ± 1.0 vs 2.1 ± 0.62 years, p = 0.005), whereas repeat irradiation provided no benefit. The use of adjuvant chemotherapy at diagnosis or at recurrence was not associated with any significant advantage. Multivariate analysis revealed margin-negative resection at initial operation and at recurrence retained OS significance, even after controlling for age, radiation therapy, and T stage. CONCLUSIONS ACC of the skull base is best treated with a multidisciplinary approach aimed at maximal, safe resection. Adjuvant radiotherapy should be offered, whereas chemotherapy does not confer benefit.
authors.affiliation: Departments of 1 Neurological Surgery and.
authors.forename: Rohan
authors.initials: R
authors.lastname: Ramakrishna
authors.affiliation: Departments of 1 Neurological Surgery and.
authors.forename: Shaan M
authors.initials: SM
authors.lastname: Raza
authors.affiliation: Head and Neck Surgery, MD Anderson Cancer Center, Houston, Texas.
authors.forename: Michael
authors.initials: M
authors.lastname: Kupferman
authors.affiliation: Head and Neck Surgery, MD Anderson Cancer Center, Houston, Texas.
authors.forename: Ehab
authors.initials: E
authors.lastname: Hanna
authors.affiliation: Departments of 1 Neurological Surgery and.
authors.forename: Franco
authors.initials: F
authors.lastname: DeMonte
mesh_terms: D003528:Carcinoma, Adenoid Cystic / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D059248:Chemoradiotherapy / Q000706:statistics & numerical data; D016208:Databases, Factual; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D018365:Neoplasm, Residual; D011446:Prospective Studies; D018714:Radiotherapy, Adjuvant; D019292:Skull Base Neoplasms / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D016019:Survival Analysis
keywords: ACC = adenoid cystic carcinoma; EGFR = epidermal growth factor receptor; ICA = internal carotid artery; OS = overall survival; PFS = progression-free survival; VEGF = vascular endothelial growth factor; adenoid cystic carcinoma; bifrontal craniotomy; endonasal endoscopy; lateral rhinotomy; neuro-oncology; oncology; radiation; skull base