Sinonasal Adenoid Cystic Carcinoma Research Articles

Clinical Outcomes in Adenoid Cystic Carcinoma of the Nasal Cavity and Paranasal Sinus: A Comparative Analysis of Treatment Modalities.

This study aimed to present the treatment patterns and outcomes for adenoid cystic carcinoma (ACC) arising in the nasal cavity and paranasal sinus. Sixty-one sinonasal ACC patients were retrospectively reviewed: 31 (50.8%) underwent surgery followed by postoperative radiation therapy (S+PORT), and 30 (49.2%) received definitive radiation therapy (D(C)RT). T4 disease was significantly more frequent in the D(C)RT group (25.8% vs. 80.0%, p < 0.001), where all T4b disease patients underwent D(C)RT. The 5-year local failure-free survival (LFFS), distant metastasis-free survival (DMFS), progression-free survival (PFS), and overall survival were 61.8% versus 37.8% (p = 0.003), 64.8% versus 38.1% (p = 0.036), 52.6% versus 19.3% (p = 0.010), and 93.2% versus 73.4% (p = 0.001) in the S+PORT and D(C)RT groups, respectively. The absolute differences in 5-year rates of LFFS, DMFS, and PFS between the two groups were smaller in the T3-4 subgroup. The univariate analysis showed that T4b disease, neurologic symptoms, longest diameter of tumor, radiological evidence of nerve involvement, and undergoing D(C)RT were associated with worse clinical outcomes, but the significance disappeared in the multivariate analysis, except for in the case of radiological evidence of nerve involvement. In conclusion, most patients with extensive disease underwent upfront D(C)RT and generally exhibited inferior clinical outcomes when compared to those with less extensive disease and who underwent S+PORT.

Overall and disease-specific survival of sinonasal adenoid cystic carcinoma: a systematic review and meta-analysis.

This meta-analysis aims to investigate the outcome of sinonasal adenoid cystic carcinoma (snAdCC). We followed PRISMA guidelines and included studies reporting 5-year overall survival (OS) rates for snAdCC. Eligible studies were identified through a literature search and assessed using JBI critical appraisal checklist. A total of 17 studies were included comprising 2259 patients (mean age: 58.1 years, 52.7% female, 47.3% male). The meta-analysis demonstrated that the 5-year OS, 10-year OS, and 5-year disease-free survival (DFS) were 68%, 40%, and 47.2%, respectively. Descriptive statistics on study level showed high rates of locally advanced tumor stages at diagnosis: 23% cT3, 53% cT4, 3.4% N+, and 4.2% M+. 29.7% of the tumors were in the nasal cavity, 67.6% in the paranasal sinuses. The maxillary, ethmoid, sphenoid, and frontal sinus were affected in 50.9%, 7.2%, 4%, and 0.5%, of cases. A combination of surgery and radiotherapy was used in 45.4% of the patients and 19.3% of patients received surgery only. In conclusion, these findings emphasize the significance of thorough surveillance for individuals with snAdCC to identify any potential recurrence or progression of the disease.

Clinical and genomic characterisation of adenoid cystic carcinoma of the head and neck, lung, and breast.

6094 Background: Adenoid cystic carcinoma (ACC) arises most commonly within the major salivary glands and other exocrine glands. ACC is typically a slow growing disease with high rates of recurrence many years after diagnosis. To inform post-operative surveillance and to provide comparator data for non-randomised studies, we sought to perform clinical and genomic characterisation of a cohort of 450 ACC patients, comparing outcomes between salivary ACC and other primary sites. Methods: 450 patients with ACC underwent clinical review at 4 UK NHS institutions. Electronic records were reviewed to extract clinical and demographic data. In 225 patients, DNA-based next-generation sequencing was performed to identify alterations in the most frequently altered genes in ACC (NOTCH1, ARID1A, KDM6A, SETD2, or TP53). Kaplan-Meier survival analyses calculated the time to first confirmed recurrence (TTFR) and overall survival from diagnosis (OS-diag) and from first confirmed recurrence (OS-rec). Results: Of 450 ACC patients, the primary site was most frequently the major salivary gland (44.7%; 201/450) comprising parotid (86), submandibular (96), sublingual (9) and salivary NOS (12). Other primary sites were sinonasal (99/450; 22.0%), upper aerodigestive (69/450; 15.3%), tracheobronchial (46/450; 10.2%), breast (16/450; 3.6%), skin (10/450; 2.2%), lacrimal (7/450; 1.6%), and other (2/450; 0.4%). Complete survival data were available in 440 patients. Recurrent/metastatic disease was confirmed in 69.8% (314/450). For these patients, the median TTFR was 4.5 yrs and for those with recurrence, the median OS from recurrence (OS-rec) was 4.8 yrs. The median TTFR in major salivary ACC was 5.8 yrs (95% CI 4.3 – 7.0). In comparison, the shortest median TTFR was seen in tracheobronchial (2.9 yrs, 95% CI 1.6 – 6.8; p = 0.019) and sinonasal ACC (3.8 yrs, 95% CI 2.9 – 5.0; p = 0.004). Breast ACC showed a differing survival pattern compared with major salivary ACC. Although the OS-diag in these two groups was not significantly different (9.75 yrs and 11.3 yrs respectively, p = ns), breast ACC showed a significantly increased TTFR (9.8 versus 4.4 yrs, p = 0.067), and a significantly reduced OS-rec (0.75 versus 4.8 yrs, p = &lt; 0.001). KDM6A mutation was seen more frequently in primary sites outside the major salivary glands (p = 0.005). However, there was no relationship between primary tumour site and mutations in NOTCH1, ARID1A, SETD2 or TP53. Conclusions: In this real-world multi-centre UK study, tracheobronchial and sinonasal ACC have significantly shorter TTFR than major salivary ACC. This study suggests that breast ACC has a similar OS from diagnosis to major salivary ACC, but has an increased TTFR and shorter OS following recurrence. This may have implications for follow-up protocols after primary treatment of breast ACC, and initiation of systemic therapy following recurrence.

Analysis of the efficacy of endoscopic transnasal surgery for sinonasal and skull base adenoid cystic carcinoma

Objective: To evaluate the efficacy of endoscopic transnasal surgery for sinonasal and skull base adenoid cystic carcinoma (ACC), and to analyze the prognostic factors. Methods: Data of 82 patients (43 females and 39 males, at a median age of 49 years old) with sinonasal and skull base ACC who were admitted to XuanWu Hospital, Capital Medical University between June 2007 and June 2021 were analyzed retrospectively. The patients were staged according to American Joint Committee on Cancer (AJCC) 8th edition. The disease overall survival(OS) and disease-free survival(DFS) rates were calculated by Kaplan-Meier analysis. Cox regression model was used for multivariate prognostic analysis. Results: There were 4 patients with stage Ⅱ, 14 patients with stage Ⅲ, and 64 patients with stage Ⅳ. The treatment strategies included purely endoscopic surgery (n=42), endoscopic surgery plus radiotherapy (n=32) and endoscopic surgery plus radiochemotherapy (n=8). Followed up for 8 to 177 months, the 5-year OS and DFS rates was 63.0% and 51.6%, respectively. The 10-year OS and DFS rates was 51.2% and 31.8%, respectively. The multivariate Cox regression analysis showed that late T stage and internal carotid artery (ICA) involvement were the independent prognostic factors for survival in sinonasal and skull base ACC (all P<0.05). The OS of patients who received surgery or surgery plus radiotherapy was significantly higher than that of patients who received surgery plus radiochemotherapy (all P<0.05). Conclusions: Endoscopic transonasal surgery or combing with radiotherapy is an effective procedure for the treatment of sinonasal and skull base ACC. Late T stage and ICA involvement indicate poor prognosis.

The outcome of transnasal endoscopic total maxillectomy in the treatment of sinonasal adenoid cystic carcinoma

Objective:To assess the prognosis of sinonasal adenoid cystic carcinoma with hard palatine invasion treated by transnasal endoscopic total maxillectomy. Methods:Clinical data of twenty-six patients with sinonasal adenoid cystic carcinoma invading hard palatine treated by transnasal endoscopic total maxillectomy between May 2014 and December 2020 was analyzed retrospectively. Survival rate, local recurrence and distant metastasis were analyzed using Kaplan-Meier method. Cox regression was used to investigate the prognosis factors. Masticatory function after maxillectomy has also been assessed using the questionnaire of patients' satisfaction about masticatory function. Results:Margins in 8 patients（30%） were positive. The median time of follow-up was 38 months（6 to 85 months）. Twenty-five patients recurred. Four patients died of distant metastasis. The 5-year overall survival rate and relapse-free survival rate was 79.5% and 89.1%, respectively. Independent predictors of outcome on multivariate analysis were positive margin（P=0.018）, recurrence（P=0.006） and distant metastasis（P=0.04）. Conclusion:Transnasal endoscopic total maxillectomy could be performed for the treatment of the sinonasal adenoid cystic carcinoma with hard palatine invasion. Positive margin, local recurrence and distant metastasis were important predictors for patients' prognosis.

The Role of Induction Therapy for Sinonasal Cancers.

The role of induction chemotherapy in sinonasal cancers is promising; however, prospective studies with higher grades of evidence are needed. With the currently available literature, the authors would advocate for the use of induction chemotherapy (IC) in locally advanced sinonasal squamous cell carcinoma (T3-T4) for organ preservation and potentially for improved survival outcomes. In sinonasal undifferentiated carcinoma (SNUC), IC should be considered in all patients given its tendency for aggressive invasion and poor outcomes. In SNUC, response to IC may direct the modality of definitive treatment to follow. In responders (partial or complete), chemoradiation therapy should be strongly considered. In non-responders or in those with progression of disease, surgical therapy is favored. For esthesioneuroblastoma, surgical resection with negative margins and adjuvant radiation therapy remains the gold standard. However, IC may be considered for locally advanced disease especially with orbital invasion or in recurrent/distant disease. There is no definite indication for IC in sinonasal adenoid cystic carcinoma or sinonasal adenocarcinoma. Recommendations are summarized in Table 1.

134 Case Report and Review of Literature: Advanced Sino-Nasal Adenoid Cystic Carcinoma with Intracranial and Intradural Extension

Abstract Introduction Adenoid cystic carcinoma (ACC) is a rare cancer of the head and neck that primarily occurs in the salivary glands. Sino-nasal ACC (SNACC) is a much rarer entity; this paper presents an exceedingly rare case of SNACC with both intracranial and intradural extension. In addition to this, a review of the literature has been performed. Case Study Our case describes the diagnosis of SNACC with both intracranial and intradural extension in a 55yr old female which was subsequently managed with palliative radiotherapy. Review of literature and Conclusions Currently, there are only few larger studies that have been conducted on SNACC and only isolated case reports of SNACC with intracranial extension. Aggregated data suggests that SNACC tends to present late at T3-T4 stage, has a 5-year survival of 60% and holds the best prognosis when surgical resection is feasible. From our case and the four other cases published on SNACC with intracranial extension it can be seen there is a clear distinction with management of patients with or without intradural extension. Intradural extension is a contraindication to surgical resection and once found deems the disease stage as primarily palliative. The focus for treatment of SNACC particularly with intracranial extension should catch disease early and prevent intradural spread. This maximises the number of patients who can undergo surgical resection and improves prognosis.

Is prophylactic neck irradiation indicated for patients with cN0 adenoid cystic carcinoma of the paranasal sinuses?

PurposeTo study the prevalence of nodal metastases in sinonasal adenoid cystic carcinoma (SNACC) and to evaluate whether prophylactic neck irradiation (PNI) should be performed in patients with clinical N0 (cN0) disease. Patients and methodsBetween April 1992 and November 2020, 166 patients with SNACC who had undergone radiotherapy at our department were retrospectively analyzed. The median follow-up time was 71.3 months. ResultsAmong 166 cases of SNACC, a total of 13 (7.8%) had retropharyngeal or cervical nodal metastasis and 93% (12/13) cases occurred in patients with advanced T stage (T3-T4). Levels VIIa, Ib, and IIa were the most common sites of initial nodal involvement. Only 1.2% (2/166) of patients presented late neck recurrence.Lymph node metastasis independently predicted a poor progression-free survival (PFS) (P = 0.017) but had no impact on overall survival (OS) (P = 0.38).PNI was performed on 36% (55/153) of cN0 patients. The OS (P = 0.42), PFS (P = 0.59), nodal recurrence-free survival (NRFS) (P = 0.46) and distant metastasis-free survival (DMFS) (P = 0.63) rates showed no significant difference between cases with and without PNI. Furthermore, cN0 patients with T4b (P = 0.53; P = 0.61), tumor origin from maxillary sinus (P = 0.55; P = 0.53) or nasopharynx involvement (P = 0.56; P = 0.60) showed no extended OS or PFS associated with PNI. ConclusionsRegardless of the T stage or the site of origin, prophylactic neck irradiation (PNI) for cN0 patients did not provide any benefit on OS and PFS, suggesting that its application on such patients is not warranted unless there is clinical suspicion.

Treatment and prognosis analysis of perineural invasion on sinonasal adenoid cystic carcinoma

Objective: To analyze the efficacy of sinonasal adenoid cystic carcinoma (ACC) with perineural invasion (PNI), and explore the prognostic value of PNI on sinonasal adenoid cystic carcinoma. Methods: The clinical data of 105 patients with sinonasal ACC admitted to Cancer Hospital, Chinese Academy of Medical Sciences from January 2000 to December 2016 were retrospectively reviewed. All patients were restaged according to American Joint Committee on Cancer 8th edition. Follow-up visits were conducted to obtain information of treatment failure and survival outcome. The Log rank test was used for univariate analysis of prognostic factors, and Cox regression model was used for multivariate prognostic analysis. Results: The maxillary sinus (n=59) was the most common primary site, followed by the nasal cavity (n=38). There were 93 patients with stage Ⅲ-Ⅳ. The treatment modalities included surgery alone (n=14), radiotherapy alone (n=13), preoperative radiotherapy plus surgery (n=10), and surgery plus postoperative radiotherapy (n=68). The median follow-up time was 91.8 months, the 5-year local control (LC), distant metastasis-free survival (DMFS), progression-free survival (PFS), and overall survival (OS) rates were 72.6%, 73.0%, 52.9% and 78.0%, respectively. There were 33 patients (31.4%) with PNI-positive. The 5-year DMFS, PFS, and OS rates of PNI-positive group were 53.7%, 29.4% and 56.5%, respectively, which were significantly inferior to those of PNI-negative group (80.8%, 63.0% and 86.8%, respectively, P<0.05), while there was no significant difference in the 5-year LC rate between both groups (64.5% vs 76.5%, P=0.273). The multivariate Cox regression analysis showed PNI was one of the poor prognostic factors of DMFS (HR=3.514, 95%CI: 1.557-7.932), PFS (HR=2.562, 95%CI: 1.349-4.866) and OS (HR=2.605, 95%CI: 1.169-5.806). Among patients with PNI-positive, the 5-year LC, PFS and OS rates of patients received surgery combined with radiotherapy were 84.9%, 41.3% and 72.7%, respectively, which were significantly higher than 23.3%, 10.0% and 26.7% of patients receiving surgery or radiotherapy alone (P<0.05). Conclusion: The presence of PNI increases the risk of distant metastasis in patients with sinonasal ACC. Compared with patients with PNI-negative, the prognosis of patients with PNI-positive is relatively poor, and surgery combined with radiotherapy for PNI-positive sinonasal ACC results in good clinical outcomes.

A rare case of advanced sinonasal adenoid cystic carcinoma with intracranial and intradural extension

Adenoid cystic carcinoma (ACC) is a rare cancer of the head and neck that primarily occurs in the salivary glands. Sino-nasal ACC (SNACC) is a much rarer entity; this paper presents an exceedingly rare case of SNACC with both intracranial and intradural extension which was ultimately treated with palliative radiotherapy due to its extensive invasion. In addition to this, a review of the literature has been performed to delineate specific learning points for the management of intracranial SNACC.

Omitting elective neck irradiation in clinically N0 sinonasal adenoid cystic carcinoma: A propensity score-matched analysis

ObjectiveTo explore the effects of elective neck irradiation (ENI) in clinically node-negative (cN0) sinonasal adenoid cystic carcinoma (SNACC). Materials and methodsBetween January 2000 and December 2016, 60 patients with cN0 sinonasal adenoid cystic carcinoma receiving surgery combined with radiotherapy were analyzed retrospectively, there were 39 received ENI (ENI group) and 21 with no ENI (non-ENI group). Propensity score matching (PSM) was performed to balance clinical factors and match patients. Kaplan-Meier method and Cox proportional hazard model were used to evaluate the impact of ENI on regional relapse risk and survival outcomes. ResultsThe median follow-up time for our cohort was 82.0 months (12.4–190.7 months). Four patients (6.7%) developed neck nodal relapses, with a median time to progression of 38.8 months. Among them, two patients in ENI group but failed out-field. After PSM, 21 patients were matched in each group. The 7-year regional relapse-free survival (RRFS), distant metastasis free survival (DMFS), progression-free survival (PFS), and overall survival (OS) between ENI group and non-ENI group were 85.4% vs 73.3% (p = 0.845), 65.2% vs 65.6% (p = 0.548), 68.3% vs 66.2% (p = 0.425), and 87.3% vs 84.0% (p = 0.953). Multivariate Cox analysis showed ENI was not an independent prognostic factor associated with RRFS, DMFS, PFS and OS. ConclusionOur findings firstly demonstrated the omission of elective neck irradiation in the management of cN0 sinonasal adenoid cystic carcinoma might be safe without compromising disease control and should be further investigated.

Expanded Endoscopic Endonasal Approach and Adjuvant Radiotherapy for the Treatment of Locally Advanced Sinonasal and Skull Base Adenoid Cystic Carcinoma: Case Report and Literature Review

Expanded Endoscopic Endonasal Approach and Adjuvant Radiotherapy for the Treatment of Locally Advanced Sinonasal and Skull Base Adenoid Cystic Carcinoma: Case Report and Literature Review

Particle Beam Radiation Therapy for Adenoid Cystic Carcinoma of the Nasal Cavity and Paranasal Sinuses.

Background: Sinonasal adenoid cystic carcinoma (SNACC) presents a challenge to oncologists due to its complex anatomy and poor prognosis. Although radiation therapy, either definitive or adjuvant to surgery, is an important part of the multidisciplinary management of SNACC, photon-based radiotherapy yielded suboptimal local control. The purpose of this study was to report the clinical results of a large patient cohort treated with particle beam radiation therapy.Methods: Patients with SNACC that received proton beam therapy (PBT), carbon-ion radiotherapy (CIRT) or a combination of CIRT and PBT between May 2015 and May 2019 were included in the analysis. Three patients were treated with PBT, 17 with CIRT and 18 received PBT and a CIRT boost. Overall survival (OS), progression-free survival (PFS), local control (LC), regional control (RC), and distant metastasis-free (DMF) rates were calculated using the Kaplan-Meier method. Toxicities were reported using the CTCAE (version 4.03).Results: A total of 38 patients were included in this analysis. Of these patients, 12 had recurrent disease, including 10 whose previous photon-based RT had failed. The most common primary tumor site was the maxillary sinus. Thirty-six patients (94.7%) suffered from locally advanced disease (T3-4). After a median follow-up of 27.2 months, the 3-year OS, PFS, LC, RC, and DMF rates were 96.7, 80.6, 90.0, 100, and 88.7%, respectively. No acute toxicities of grade 3 or above were observed. Two patients experienced grade 3 xerostomia or vision decreased, and one patient died of hemorrhage.Conclusion: PBT, CIRT or a combination of CIRT and PBT appeared to be a promising treatment option for SNACC and produced satisfactory local control and toxicity profile. Longer follow-up is needed to verify the long-term benefit of particle-beam radiation therapy (PBRT) for patients with SNACC.

Adenoid Cystic Carcinoma of Nasal Cavity A Rare Case Report

Adenoid cystic carcinoma (ACC) was first described by billroth in 1856. It is common in minor salivary glands around the mouth, uncommon in parotids and in Nose and paranasal sinuses. Sinonasal ACC accounts for 10-25% of all head and neck ACC. We are reporting a case of nasal cavity adenoid cystic carcinoma in a 70 year old male patient who was presented with bilateral nasal block, nasal bleeding and loss of vision. On examination polypoidal growth was present in bilateral nasal cavity. Biopsy was taken and sent for histopathological examination. Histopathological findings were suggestive of adenoid cystic carcinoma of nasal cavity. ACC is a slow-growing but highly malignant neoplasm with a remarkable capacity for recurrence. They show no sex predilection and may involve patients of any age, although they usually present in middle-aged or older patients. It has a high risk of recurrence and distant metastasis. Features of diagnostic and therapeutic evaluation are described here with.

Endoscopic resection of sinonasal adenoid cystic carcinoma with radiofrequency coblation. A clinical report with literature review

Introduction Sinonasal cancer is very rare and accounts for under 1% of all malignancies of which sinonasal adenoid cystic carcinoma (SNACC) is about 5%. The maxillary sinus is the most common site of SNACC. Misleading symptomatology which overlaps with rhinosinusitis contributes to the delay of diagnosis. Recently, the endoscopic approach is commonly applied with equal or better results to the open approach. Radiofrequency coblation might be used to reduce blood loss. Material and Methods We present a case of an adenoid cystic carcinoma arising from the left inferior turbinate. The patient received endoscopic surgical treatment with the application of radiofrequency coblation in Clarós Clinic at the beginning of 2019 and remains followed up with no sign of recurrence. Afterward, we review the literature on the subject. Conclusion SnACC is a rare malignancy, characterized by slow growth and perineural infiltration with spreading towards the skull base, cavernous sinus, and orbit. The regional nodal involvement is rare. Frequent and delayed distant metastasis in lung, bone, liver, and brain are also typical. Prognosis is poor with the 5-year overall survival between 41%-86%. Surgery with or without complementary radiotherapy provides the best outcomes for the majority of patients. The significance of prognostic factors remains unclear. Lifelong surveillance is obligatory as very late recurrences are common.

Adenoid Cystic Carcinoma.

Sinonasal adenoid cystic carcinoma is a rare malignancy characterized by an insidious growth pattern and a tendency for perineural spread along major and minor nerves, resulting in invasion of the skull base and intracranial extension. Therefore, many patients present with advanced disease and involvement of critical structures, making treatment difficult and potentially associated with high morbidity. Surgery represents the mainstay of treatment of the primary tumor. Complete resection of the tumor with negative margins, whenever feasible, is associated with better survival outcomes. However, in the case of extensive involvement of vital structures (e.g., carotid artery, cavernous sinus, optic nerve, Meckel's cave) or when radical surgery could seriously affect the patient's quality of life, a function-preserving subtotal removal of the tumor followed by irradiation can be proposed. The role of surgery is limited to a biopsy in unresectable lesions that are more suitable for non-surgical treatments (e.g., exclusive chemoradiation). Given the difficulty in obtaining negative margins and the propensity for submucosal and perineural spread, adjuvant radiotherapy is strongly recommended. Recently, heavy-particle radiotherapy using protons or carbon ions has emerged as a promising treatment with improved local control. Local failures (60%) and distant metastases (40%) are common and can occur even decades after definitive treatment. The 5-year overall survival ranges from 55 to 70% and it exceeds that of other sinonasal malignancies, but dramatically drops down at 10 years (40%) and further decreases at 20 years (15%). Therefore, a prolonged follow-up of at least 15 years, and possibly lifelong, is mandatory.

Treatment Outcome of 227 Patients with Sinonasal Adenoid Cystic Carcinoma (ACC) after Intensity Modulated Radiotherapy and Active Raster-Scanning Carbon Ion Boost: A 10-Year Single-Center Experience.

We aimed to evaluate the treatment outcome of primary and postoperative bimodal radiotherapy (RT) including intensity modulated photon radiotherapy (IMRT) and carbon ion radiotherapy (CIRT) for sinonasal adenoid cystic carcinoma (ACC) patients. Medical records of 227 consecutive patients who received either a primary (n = 90, 40%) or postoperative (n = 137, 60%; R2, n = 86, 63%) IMRT with doses between 48 and 56 Gy in 1.8 or 2 Gy fractions and active raster-scanning carbon ion boost with 18 to 24 Gy (RBE, relative biological effectiveness) in 3 Gy (RBE) fractions between 2009 and 2019 up to a median total dose of 80 Gy (EQD2, equivalent dose in 2 Gy single dose fractions, range 71–80 Gy) were reviewed. Results: Median follow-up was 50 months. In univariate and multivariate analysis, no significant difference in local control (LC) could be shown between the two treatment groups (p = 0.33). Corresponding 3-year LC rates were 79% for primary bimodal RT and 82% for postoperative bimodal RT, respectively. T4 stage (p = 0.002) and solid histology (p = 0.005) were identified as independent prognostic factors for decreased LC. Significant worse long-term treatment tolerance was observed for postoperatively irradiated patients with 17% vs. 6% late grade 3 toxicity (p < 0.001). Primary radiotherapy including IMRT and carbon ion boost for dose-escalation results in adequate LC with less long-term grade 3 toxicity compared to postoperative bimodal radiotherapy in sinonasal ACC patients. The high rate of macroscopic tumor disease in the postoperative group makes the interpretation of the beneficial results in LC for primary RT difficult.

Positron emission computed tomography and magnetic resonance imaging features of sinonasal small round blue cell tumors.

The sinonasal tract hosts numerous types of undifferentiated neoplasms, having small round cell morphology. The aim of this study was to determine whether sinonasal small round blue cell tumors (SRBCT) have distinct imaging features on computed tomography (CT), magnetic resonance imaging (MRI), and 18-fluorodeoxyglucose positron emission tomography (18F-FDG PET)/CT. Seventy-three patients (43 male; Mage = 61.2 years) with histopathologically proven sinonasal SRBCT were retrospectively reviewed. Imaging features of SRBCTs including location, maximum dimension, margin characteristics, presence of calcification, sclerotic bone changes, intratumoral necrosis, tumor extension, bone destruction, bone remodeling, perineural spread, T1- and T2-weighted MRI signal intensity, qualitative features on diffusion-weighted imaging and 18F-FDG PET/CT, and pattern of contrast enhancement were analyzed using Fisher's exact test or the chi-square test. The maximum standardized uptake values (SUVmax) and apparent diffusion coefficient (ADCmean) values of SRBCT were compared by utilizing the Kruskal-Wallis test. There was a significant difference between SRBCT type regarding the tumor location (p = 0.006), 18F-FDG uptake pattern (p = 0.006), involvement of the orbit (p = 0.016) and pterygopalatine fossa (p = 0.043), the presence of perineural spread (p < 0.001), bone destruction (p = 0.034), and intratumoral necrosis (p = 0.022). Bone destruction and necrosis were more common in rhabdomyosarcoma. Perineural spread was common in sinonasal adenoid cystic carcinoma (ACC). Qualitative 18F-FDG uptake features as well as tumor location were significantly different between sinonasal ACC and sinonasal undifferentiated carcinoma. The ADCmean and SUVmax values were not statistically different between SRBCT types. Sinonasal SRBCTs have numerous distinct imaging features on CT, MRI, and 18F-FDG PET/CT that could be useful in the differentiation between lesions when the histopathologic diagnosis is inconclusive.

OC-0391 Treatment outcome of 265 patients with sinonasal adenoid cystic carcinoma (ACC)

OC-0391 Treatment outcome of 265 patients with sinonasal adenoid cystic carcinoma (ACC)

Adenoid cystic carcinoma of the sinonasal tract: a review of the national cancer database.

Sinonasal adenoid cystic carcinoma (SNACC) is a rare malignancy with a propensity for distant metastasis. In this study we describe the incidence and determinants of survival among patients with SNACC between the years 2004 and 2012 using the National Cancer Database (NCDB). This was a retrospective, population-based cohort study performed at a tertiary academic medical center. All participants were diagnosed with SNACC between 2004 and 2012 within the NCDB. The main outcome was overall survival (OS). A total of 793 patients were identified. The cohort was composed of 46.9% males. Mean age at diagnosis was 59.6 years. The maxillary sinus was the most common primary site (49.7%). Nodal disease was seen in 3.6% of the patients, whereas 3.7% had distant metastases. Stage IV disease was seen in 49.1% of cases. In total, 77.4% of patients underwent surgery, 68.2% received radiation therapy, and 16.4% had chemotherapy. Median OS was 78.5 months; OS at 1, 2, and 5 years was 91%, 83%, and 61%, respectively. On multivariate analysis, advanced age (p = 0.001), frontal sinus primary site (p < 0.001), positive margins (p < 0.001), Charlson comorbidity index >0 (p = 0.01), residing in an urban setting (p = 0.04), poorly differentiated or undifferentiated tumor grade (p = 0.003), and advanced tumor stage (p = 0.01) were associated with worse OS, whereas surgery (p < 0.001), but not radiation therapy (p = 0.52) or chemotherapy (p = 0.57), predicted improved OS. Predictors of survival in SNACC include age, comorbidity status, grade, and stage. Surgery is associated with improved survival and remains the mainstay of therapy, whereas the roles of radiation therapy and chemotherapy require future investigation.