Management Of Adenoid Cystic Carcinoma Research Articles

Approaches to the Management of Metastatic Adenoid Cystic Carcinoma.

High rates of recurrence and distant metastasis are a foremost challenge in the management of adenoid cystic carcinoma (ACC), occurring in approximately 40% of all ACC patients. Despite the morbidity and mortality resulting from recurrent/metastatic (R/M) disease, there are no FDA-approved systemic agents for these patients. In this review, we summarize pertinent ACC pathophysiology and its implications for different systemic treatment regimens in R/M ACC. We review the evidence for the most widely used systemic agents - cytotoxic chemotherapy and tyrosine kinase inhibitors (TKIs) targeting VEGFR - in addition to immune checkpoint inhibitors and non-TKI biologic agents. Exciting emerging targets for R/M ACC, including inhibitors of Notch signaling, stemness, PRMT5, and Axl, are also discussed. Lastly, we review local therapies for small-volume lung disease in patients with oligometastatic ACC, specifically pulmonary metastasectomy and stereotactic body radiation therapy (SBRT). Future development of targeted molecular agents which exploit the underlying biology of this disease may yield novel therapeutic options to improve clinical outcomes in patients with R/M ACC.

Adenoid cystic carcinoma of maxillary antrum: A case report.

IntroductionAdenoid cystic carcinoma rarely involves the nose and paranasal sinuses. It is a slowly growing, aggressive malignant tumor with a predisposition for perineural invasion. The study highlights the importance of timely diagnosis and management of adenoid cystic carcinoma.Presentation of caseHerein, we report a case of adenoid cystic carcinoma of maxillary antrum in a 69 years old female, who presented with an insidious onset of painful right cheek swelling for three months. Biopsy showed the glandular pattern of tumor cells with perineural invasion, suggestive of adenoid cystic carcinoma. Contrast-enhanced computed tomography of paranasal sinuses showed a well-differentiated, irregularly outlined, non-enhancing heterogeneous lesion in the right maxillary sinus with bony destruction, suggesting the malignant nature of the lesion. The mass was surgically excised and histopathological examination further confirmed the diagnosis.DiscussionAdenoid cystic carcinoma is a rare malignancy of the nose and paranasal sinus, although it is quite common in salivary glands. It should be considered in the differentials in a patient presenting with sinonasal mass, pain, nasal obstruction, epistaxis, and auditory symptoms.ConclusionAs the tumor can be missed clinically owing to its rarity in paranasal sinuses and can present at an advanced stage, early diagnosis and management of adenoid cystic carcinoma are important.

Cystic Adenoid Carcinomas in Ear, Nose and Throat: Unusual Localizations and a Literature Review

Introduction: adenoid cystic carcinoma account 1% for all cancers of the ear nose and throat. Despite slow growth, their recurrence is frequent. This study concerns 5 cases of cystic adenoid carcinoma with atypical ear nose and throat locations (outside the main salivary glands).
 Materials and methods: Our work is a study of 5 cases of cylindromes with atypical localizations collected at the ear nose throat and cervico-facial surgery department of Mohamed IV university hospital of Marrakesh: 1 case located at the external auditory meatus, 1 case at the infratemporal fossa, 1 case on the inside of the cheek, 1 case in the soft palate and one laryngotracheal case.
 Results and discussion: The management of adenoid cystic carcinoma remains delicate because of their insidious growth, their neurological tropism and their metastatic potential. These associated elements delay the diagnosis that is often made while those tumors are locally advanced, which can make surgery difficult, and be responsible of a lot of late recurrences. The treatment of these tumors has long relied on exclusive surgery. Cystic adenoid carcinomas were considered radio resistant. Retrospective studies have shown the benefit of treatment combining surgery and radiotherapy, in terms of local control and disease-free survival, compared to surgery or radiotherapy alone.
 Conclusion: The essential problem of adenoid cystic carcinoma remains the long-term control of the disease. Our results confirm that the gold-standard treatment should be both surgery and radiotherapy.

Management of Adenoid Cystic Carcinoma of the Breast: A Single-Institution Study.

ObjectiveThe purpose of our study was to analyze the clinicopathologic features and surgical and oncological outcomes of adenoid cystic carcinoma (ACC) of the breast and to provide the basis for a clinical therapeutic schedule.MethodsA total of 14 patients with primary breast adenoid cystic carcinoma treated at Cancer Hospital of the Chinese Academy of Medical Sciences from January 2000 to December 2017 were included. Data on clinical presentation, treatment strategy, and outcome, as well as the pathological features of ACC, were reviewed and analyzed.ResultsFourteen patients were diagnosed with ACC of the breast, out of 23205 total patients treated for breast cancer (0.06%). All but three patients were postmenopausal, with a median age at diagnosis of 60.5 years (range, 39–73 years). The most common clinical presentation was a palpable mass (85.7%), and the imaging characteristics of all patients on color Doppler ultrasound and mammography were nonspecific. Six patients (42.9%) were suspected of having ACC by fine-needle aspiration cytology (FNAC) and were confirmed by postoperative histology and immunohistochemistry. All 14 patients underwent surgery, and no patient had a positive lymph node status. Median tumor size was 1.75 cm (range, 1–3 cm). Eight/14 (57.1%) patients were hormone receptor negative (HR−) and HER-2/neu (−) (HER2−). The remaining patients were hormone receptor positive (HR+). There was no significant difference in clinicopathological characteristics between the HR+ group and the HR- group (P>0.05). The mean follow-up period was 57 months. Local recurrence occurred in 14.3% of patients, 1.7% of patients had distant metastasis, all patients with local recurrence or distant metastasis were in the HR (-) group, and all patients were alive at the last follow-up.ConclusionACC of the breast cannot be simply summarized as triple-negative breast cancer because it also includes a small number of hormone receptor-positive breast cancers. Establishing a preoperative diagnosis is difficult on the basis of clinical imaging examination, FNAC may be useful tool in the diagnosis. the final diagnosis can only be assessed based on the results of the histopathological and immunohistochemical examination. Breast-conserving surgery may be an alternative treatment strategy, and axillary lymph node dissection or sentinel node biopsy may not be necessary in some cases.

Management of Adenoid Cystic Carcinoma of the Head and Neck: Experience of the National Cancer Institute, Egypt

This study presents the experience of the National Cancer Institute, Cairo University, in diagnosis and management of ACC of the head and neck.This is a retrospective review of 57 patients with ACC managed during the period from January 2011 to January 2016. Data about the characteristics and management of the disease were recorded. All patients were followed up to detect the development of local recurrence and distant metastasis and their management.The mean age was 45.5 ± 15.1, with a femaleto-male ratio of 1.5:1. The minor salivary glands were affected in 61.4% of cases. Four patients (7%) were metastatic at presentation. The main presenting symptom was swelling, followed by pain. Surgical resection was performed in 48 patients (84.2%) followed by adjuvant radiotherapy in 36 of them. Four patients received radical radiotherapy. Treatment failed in 3 patients. Recurrences were recorded in 21 out of the 50 cured patients; 9 had locoregional recurrence, 9 had distant metastases, and 3 had both. The overall survival (OS) and disease-free survival (DFS) at three years were 79% and 57.1%, respectively. Surgical resection improved OS (p<0.001). Advanced T-stage, lymph node invasion, solid tumors, close or positive margins worsened OS. Adjuvant radiotherapy was associated with better DFS (p = 0.003), while solid tumors were associated with worse DFS.Despite aggressive management with radical surgery and adjuvant radiotherapy, recurrence affects 42% of the patients within three years. Patients with unresectable tumors have a poor prognosis. Adjuvant radiotherapy improves DFS but not OS.

Management of adenoid cystic carcinoma of the head and neck: a single-institute study with over 25-year follow-up

BackgroundAdenoid cystic carcinoma is a rare malignant tumor arising from exocrine glands such as the major and minor salivary glands of the paranasal sinuses or the external auditory canal. Although multiple retrospective clinical studies of ACC have been reported to date, clinical questions, such as 1) long-term prognosis beyond 20 years, 2) usefulness and suitability for treatment of therapeutic interventions, 3) therapeutic goal to aim for, and 4) prognosis by recurrence sites, are still unclear.MethodsTo improve understanding and management of adenoid cystic carcinoma of the head and neck (ACC), a retrospective study with 58 new ACC cases between 1991 and 2016 was performed. The median observation period was 66.8 months (range 3–316 months). The overall clinical stages were as follows: I, 6.9%; II, 25.9%; III, 19.0%; and IV, 48.2%. Histology was cribriform/tubular type (C-T type) in 62.0% and solid type in 27.5%. The main treatment strategy was definitive surgery, which was performed in 75.2% of cases.ResultsOverall 10-year, 20-year, and 25-year survivals were 63.7, 27.3, and 20.0%, respectively. Similarly, disease-specific survival (DSSs) was 65.7, 51.2, and 38.4%, respectively, and disease-free survival was 25.2, 9.4, and 9.4%, respectively. Conducting surgery (HR: 0.19, 95% CI: 0.06–0.61, p = 0.005) and C-T type (HR: 0.32, 95% CI: 0.11–0.93, p = 0.036) were independent prognostic predictors of DSS. DSS was significantly prolonged after salvage surgery for both locoregional recurrence (p = 0.004) and lung metastatic recurrence (p = 0.012, vs best supportive care).ConclusionsIn ACC cases, both initial surgical treatment and repetitive surgical resection of resectable recurrent lesions, including both locoregional and lung metastases, resulted in longer survival. The major goal of treatment for ACC may be long-term survival including cancer-bearing survival, resulting in either natural death or intercurrent-disease death, since judging cure of ACC is almost impossible.Trial registrationRetrospectively registered.

Management of advanced adenoid cystic carcinoma infiltrating the skull base: a contemporary review.

This article provides an overview of the natural history and management of adenoid cystic carcinoma infiltrating the skull base (SB ACC). A comprehensive review of the available literature and discussion regarding the current management of SB ACC. This review describes the unique pathology, appropriate diagnostic work-up and contemporary management options in SB ACC. There is a specific focus on the role of surgical resection with post-operative radiotherapy, given the literature suggesting a superior locoregional control rate. The importance of surgical decision making and varying surgical approaches are detailed. In addition, the emerging role of biological agents is discussed. The gold standard for SB ACC is surgical resection and post-operative radiation, given the superior locoregional control. In patients not amendable to surgery, palliative radiation should be considered. The role of biological agents is still evolving. The complexity of SB ACC management mandates experienced multi-speciality management.

Evaluation of Multimodality Management of Adenoid Cystic Carcinoma of the Head and Neck.

Adenoid cystic carcinoma is a relatively rare tumour arising from salivary glands of head and neck region. Surgery and radiotherapy form standard treatment modalities in the management of this tumor. In this analysis we present results of multimodality treatment in our cohort of patient. This retrospective analysis evaluated results of treatment in forty patients diagnosed with adenoid cystic carcinoma ofthe head and neck. Evaluation was done to identify prognostic factors affecting the disease free survival. A median disease free survival of 34 ± 2.42 (Median ± SE) versus 10 ± 5.45months was seen in patients undergoing surgery followed by post operative radiotherapy versus radiotherapy alone (P = 0.01). A radiotherapy dose more than 60Gy was associated with a better disease free survival compared with patients receiving less than 60Gy (P = 0.01). Positive surgical margins and perineural invasion were associated with a poor treatment outcome (P = 0.02) Patients with c-kit positive status showed a poor local control rate (P = 0.05).

Percutaneous CT-fluoroscopic–guided radioisotope seed placement for the management of adenoid cystic carcinoma of the trachea

PurposeTracheal and paratracheal malignancies present challenges in radiotherapeutic management due to their proximity to sensitive structures, central location, and because of the potential for catastrophic radiation induced fistula. The use of external beam radiation therapy and intraluminal brachytherapy has a limited number of reports in the literature. We have searched for a technique for treating tracheal and paratracheal malignancies allowing application of a high dose of radiation while minimizing dose to normal tissues. Our group has considerable experience in the use of percutaneous CT-guided radioisotope seed implants for intrathoracic malignancies. We have previously reported our technique for percutaneous CT-fluoroscopic–guided radioisotope seed implants in the management of thoracic malignancies. We have now treated several tracheal and paratracheal malignancies with our technique and report our results here. This is to our knowledge the first report of treatment of tracheal malignancy with CT-guided permanent seed implant. Methods and MaterialsThree patients with tracheal adenoid cystic carcinoma were implanted with 103Pd under CT-fluoroscopic guidance utilizing percutaneous approach. ResultsAll patients tolerated the procedure well, and at 9-month average followup, all show disease regression, symptom improvement, and no sign of toxicity. ConclusionsWe believe this to be the first published series on CT-directed permanent seed brachytherapy for tracheal malignancies. Review of PubMed literature to 1990 discloses no prior writings on the use of permanent seed implantation for tracheal cancers. Tracheal malignancies provide a vexing radiation therapy challenge to stay within the therapeutic window. CT-directed permanent seed brachytherapy allows a high dose to be delivered to the tumor with a rapid falloff to the surrounding tissues. Short-term results from seed implant are excellent. Our 3 patients responded well in the short term to permanent seed brachytherapy with no chronic side effects and with reduction or relief of cough and pain. CT-fluoro–guided permanent seed implantation is an effective and low morbidity treatment for tracheal malignancies. Long-term followup is needed to further elucidate durability of response and toxicity.

BH3-mimetic small molecule inhibits the growth and recurrence of adenoid cystic carcinoma

To evaluate the anti-tumor effect of BM-1197, a new potent and highly specific small molecule inhibitor of Bcl-2/Bcl-xL, in preclinical models of human adenoid cystic carcinoma (ACC). Low passage primary human adenoid cystic carcinoma cells (UM-HACC-2A,-2B,-5,-6) and patient-derived xenograft (PDX) models (UM-PDX-HACC) were developed from surgical specimens obtained from 4 patients. The effect of BM-1197 on cell viability and cell cycle were evaluated in vitro using this panel of low passage ACC cells. The effect of BM-1197 on tumor growth, recurrence and tumor cell apoptosis in vivo was evaluated with the PDX model of ACC (UM-PDX-HACC-5). Exposure of low passage primary human ACC cells to BM-1197 mediated an IC50 of 0.92-2.82 μM. This correlated with an increase in the fraction of apoptotic cells (p<0.0001) and an increase in caspase-3 activity (p<0.0001), but no noticeable differences in cell cycle (p>0.05). In vivo, BM-1197 inhibited tumor growth (p=0.0256) and induced tumor cell apoptosis (p=0.0165) without causing significant systemic toxicities, as determined by mouse weight over time. Surprisingly, weekly BM-1197 decreased the incidence of tumor recurrence (p=0.0297), as determined by Kaplan-Meier analysis. These data demonstrated that single agent BM-1197 induces apoptosis and inhibits tumor growth in preclinical models of adenoid cystic carcinoma. Notably, single agent BM-1197 inhibited tumor recurrence, which is considered a major clinical challenge in the clinical management of adenoid cystic carcinoma. Collectively, these results suggest that patients with adenoid cystic carcinoma might benefit from therapy with a BH3-mimetic small molecule.

Ongoing challenges in the treatment of adenoid cystic carcinoma of the head and neck.

Adenoid cystic carcinoma is a malignant tumour of major and minor salivary glands. Distant metastasis and poor survival are persistent in the literature, with recent publications aimed at understanding molecular pathogenesis and development of pharmaceutical therapeutic options. Provide an update of recent studies in the management of adenoid cystic carcinoma of the head and neck. Literature search using Medline, Scopus, Google Scholar, the Cochrane Database of Systematic Reviews and the Cochrane central register of controlled trials for articles on adenoid cystic carcinoma from January 2005 to January 2015. Adenoid cystic carcinoma is characterized by a slow growing mass, with distant metastasis independent of local or regional control. Primary tumour resection remains the preferred option with radiotherapy having an adjuvant role. Recent advances have been made with novel targeted therapies however, limited to clinical trials and advanced disease.

Long-term treatment outcomes and prognostic features in adenoid cystic carcinoma of the head and neck

PurposeThe aim of this study was to identify independent predictors of outcome and to characterize the patterns of failure in a long-term, single-institution experience with the management of adenoid cystic carcinoma (ACC) of the head and neck using survival analyses. Patients and methodsWe investigated prognostic factors, such as gender, age, primary tumor site, TNM stage, treatment modalities, perineural invasion, metastases, surgical margins, histological grade, cervical lymph node metastasis, and postoperative RT spread through 86 cases of ACC diagnosed by Seoul National University Dental Hospital (SNUDH) from 1984 to 2013. The overall survival and disease-free survival were evaluated. Survival rates were predicted and Kaplan–Meier's survival curves were generated for each factor. Cox proportional hazard models were used for multivariate survival analysis. ResultsSurvival analysis shows that overall survival (OS) at 5 years, 10 years, and 20 years was 75%, 55%, and 41%, respectively. Disease-free survival (DFS) at 5 years, 10 years, and 20 years was 58%, 44%, and 28%, respectively. Univariate analysis revealed that age, primary tumor site, histological subtype, clinical stage, cervical lymph node metastasis, and presence of distant metastasis were significant predictors while perineural invasion, margin involvement, and gender did not demonstrate statistical difference between the treatment groups. Our multivariate analysis revealed that advanced clinical stage (stages III and IV), distant metastasis, and age over 60 years were independent significant prognostic factors. ConclusionsRecurrence and metastasis were the main cause of treatment failure of ACC in head and neck. T stage, age, primary tumor site, histologic type, cervical lymph node metastasis, and distant metastasis were the independent prognostic factors of ACC in head and neck. Radical surgery and reasonably postoperative radiotherapy were the main treatment strategy.

English

Benign or malignant tumors of mouth and oropharyngeal region often result in the maxillofacial defects. The acquired maxillectomy defects results in the impairment of speech, mastication, swallowing and facial esthetics. The Prosthodontist plays a significant role in the rehabilitation of the acquired maxillectomy defects with the special emphasis to the reduction of the weight of the prosthesis and thereby enhancing the retention and stability. The weight of the obturator can cause a dislodging force. It must therefore be as light as possible. A suitable technique of fabrication should be selected to achieve this. This case report describes the fabrication of a hollow bulb obturator for a hemi- maxillectomy patient (Aramany's class 1 defect) who had undergone wide excision and alveolar resection squeal of management of adenoid cystic carcinoma of the right palate. This article describes the step by step clinical and laboratory procedure involved in the rehabilitation of a hemimaxillectoy dentate patients using obturator lined with soft liner to restore the functions such as esthetics, mastication, deglutition and speech.

Management of Adenoid Cystic Carcinoma Metastatic to Lung with CT-Fluoroscopic Guided Brachytherapy

Management of Adenoid Cystic Carcinoma Metastatic to Lung with CT-Fluoroscopic Guided Brachytherapy

Management of Adenoid Cystic Carcinoma of Distal Trachea

SummaryTracheal neoplasms occur infrequently, the majority of primary tracheal tumours in adults are malignant, and in 40% of cases it is adenoid cystic carcinoma, which can be asymptomatic for a long period of time. Surgical treatment can be justified even in advanced disease, because progression of tracheal tumour usually causes obstruction of airways. Improved long-term outcome seems to follow combined surgical resection and full-dose radiotherapy. Patients after resection of adenoid cystic carcinoma need careful and long-term observations to detect a relapse

Orbitocranial Approach for Treatment of Adenoid Cystic Carcinoma of the Lacrimal Gland

Multiple treatment options exist for management of adenoid cystic carcinoma of the lacrimal gland. Our objective was to perform an analysis of outcomes in a cohort of patients with adenoid cystic carcinoma of the lacrimal gland treated identically with an orbitocranial approach. We performed a retrospective review of 7 consecutive patients who presented to a tertiary care academic medical center between 1995 and 2009 with adenoid cystic carcinoma of the lacrimal gland. All patients were treated with an orbitocranial approach to tumor resection followed by postoperative radiotherapy. The mean and median follow-up times were 39 and 19 months, respectively (range, 7 to 138 months). Six patients had orbital reconstruction using free tissue transfer, and 1 patient had a split-thickness skin graft to line the orbital cavity. Two patients developed distant metastases 18 months and 29 months after surgery and ultimately died with disease. Five patients are alive without disease. The orbitocranial approach followed by postoperative irradiation achieves excellent local and regional control rates for adenoid cystic carcinoma of the lacrimal gland, although patients remain at risk long-term for distant metastases. Orbital bone removal to obtain adequate margins should be a routine part of tumor resection for these malignancies.

Management of Adenoid Cystic Carcinoma of the Breast: A Rare Cancer Network Study

Mammary adenoid cystic carcinoma (ACC) is a rare breast cancer. The aim of this retrospective study was to assess prognostic factors and patterns of failure, as well as the role of radiation therapy (RT), in ACC. Between January 1980 and December 2007, 61 women with breast ACC were treated at participating centers of the Rare Cancer Network. Surgery consisted of lumpectomy in 41 patients and mastectomy in 20 patients. There were 51(84%) stage pN0 and 10 stage cN0 (16%) patients. Postoperative RT was administered to 40 patients (35 after lumpectomy, 5 after mastectomy). With a median follow-up of 79 months (range, 6-285), 5-year overall and disease-free survival rates were 94% (95% confidence interval [CI], 88%-100%) and 82% (95% CI, 71%-93%), respectively. The 5-year locoregional control (LRC) rate was 95% (95% CI, 89%-100%). Axillary lymph node dissection or sentinel node biopsy was performed in 84% of cases. All patients had stage pN0 disease. In univariate analysis, survival was not influenced by the type of surgery or the use of postoperative RT. The 5-year LRC rate was 100% in the mastectomy group versus 93% (95% CI, 83%-100%) in the breast-conserving surgery group, respectively (p = 0.16). For the breast-conserving surgery group, the use of RT significantly correlated with LRC (p = 0.03); the 5-year LRC rates were 95% (95% CI, 86%-100%) for the RT group versus 83% (95% CI, 54%-100%) for the group receiving no RT. No local failures occurred in patients with positive margins, all of whom received postoperative RT. Breast-conserving surgery is the treatment of choice for patients with ACC breast cancer. Axillary lymph node dissection or sentinel node biopsy might not be recommended. Postoperative RT should be proposed in the case of breast-conserving surgery.

Advanced adenoid cystic carcinoma of the cervix: a case report and review of the literature

IntroductionAdenoid cystic carcinoma is a malignant epithelial neoplasm derived from the salivary glands. Primary adenoid cystic carcinoma of the cervix is extremely rare, accounting for less than 1% of all cervical carcinomas. In this paper we report a case of primary adenoid cystic carcinoma and a review of the related literature.Case presentationA 68 year-old woman was admitted with signs and symptoms suggestive of a cervical cancer. The radiological and pathological investigations confirmed the diagnostic of primary adenoid cystic carcinoma of the cervix at Stage IIIB according to the International Federation of Gynaecology and Obstetrics classification. The patient was managed successfully by concurrent chemo-radiotherapy.ConclusionThe optimal management of adenoid cystic carcinoma cannot be established for certain. From our case and from the literature, it appears that combined treatment (surgery, radiotherapy, and chemotherapy) is necessary for achieving a long-term remission. Concurrent chemo-radiotherapy appears to be a logical option for locally advanced disease.

Epithelial tumors of the lacrimal gland: an update

The goal of this article is to offer an update on the treatment and prognosis of the most common epithelial tumors of the lacrimal gland, report on new pathological entities and offer a review of the classification of lacrimal gland tumors. Improvements have been made in the understanding of lacrimal gland lesions with the knowledge that lacrimal gland tumors compare to the more common counterparts of the major salivary glands. Therefore, the WHO's classification of salivary gland tumors has been adapted to the lacrimal gland pathology. Until recently, primary adenocarcinomas of the lacrimal gland were not further subclassified, but they can now be divided into low-grade and high-grade malignancies. The adjunctive use of intra-arterial cytoreductive chemotherapy for the management of adenoid cystic carcinoma is one of the most important advancements on the management of these aggressive tumors. Another important step forward has been taken on carcinoma ex pleomorphic adenoma of the lacrimal gland, which is subclassified into noninvasive carcinoma, with an excellent prognosis after complete excision and invasive carcinoma for which the prognosis is still guarded despite adjunctive radiotherapy. This article offers an update on diagnosis, classification and treatment of common and rare epithelial lacrimal gland tumors.

Adenoid cystic carcinoma of the head and neck treated by surgery with or without postoperative radiation therapy: Prognostic features of recurrence

This study sought to review a single-institution experience with the management of adenoid cystic carcinoma of the head and neck. Between 1960 and 2004, 140 patients with adenoid cystic carcinoma of the head and neck were treated with definitive surgery. Ninety patients (64%) received postoperative radiation to a median dose of 64 Gy (range, 54-71 Gy). Distribution of T stage was: 26% T1, 28% T2, 20% T3, and 26% T4. Seventy-eight patients (56%) had microscopically positive margins. Median follow-up was 66 months (range, 7-267 months). The 5- and 10-year rate estimates of local control were 88% and 77%, respectively. A Cox proportional hazards model identified T4 disease (p = 0.0001), perineural invasion (p = 0.008), omission of postoperative radiation (p = 0.007), and major nerve involvement (p = 0.02) as independent predictors of local recurrence. Radiation dose lower than 60 Gy (p = 0.0004), T4 disease (p = 0.005), and major nerve involvement (p = 0.02) were predictors of local recurrence among those treated with surgery and postoperative radiation. The 10-year overall survival and distant metastasis-free survival were 64% and 66%, respectively. Combined-modality therapy with surgery followed by radiation to doses in excess of 60 Gy should be considered the standard of care for adenoid cystic carcinoma of the head and neck.