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Adenoid cystic carcinoma infrequently affects paranasal sinuses. It's a slowly progressing, aggressive cancer with a tendency to invade nerves. The research underscores the significance of prompt diagnosis and effective management of adenoid cystic carcinoma. Two cases of adenoid cystic carcinoma of the maxillary sinus are presented. The first, a 73-year-old woman, presented with right nasal obstruction. The second, a 53-year-old woman, presented with a hard palatal mass and right nasal obstruction. The biopsy confirmed adenoid cystic carcinoma in the first patient. Given the extent of the tumor, she was referred to radiotherapy to complement the therapeutic treatment. The second patient underwent mass excision, also confirmed to be adenoid cystic carcinoma, followed by radiation therapy. Adenoid cystic carcinoma of the maxillary sinus is often diagnosed at an advanced stage due to its slow growth and local regional spread, making its diagnosis and therapeutic management particularly challenging., Competing Interests: The authors declare no competing interests., (Copyright: Anouar Ben Ameur El Youbi et al.)

Introduction: Adenoid cystic carcinoma (ACC) is a rare tumor, with an annual incidence of 3-4.5 cases/million., Materials and Methods: We conducted a descriptive and retrospective study. We included patients diagnosed with ACC of head and neck of a single third-level referral center, from January 2008 to December 2013., Results: A total of 23 patients were included in the study, 10 males (43.5%) and 13 females (56.5%) (F:M ratio 1.3:1). Median age was 52 years. Localization was predominantly observed in the oral cavity (26%) followed by the palate (17.4%). At presentation, 15 patients (65.2%) had clinical Stage II. The most frequent histology was the cribriform type in 10 patients (43.5%). Following resection, positive margins were observed in 15 (65.2%), neural invasion in 8 (34.8%), and lymphovascular invasion in 2 (8.7%) patients. The median time to recurrence in our population was 23 months. Recurrence at 5 years was observed in 15 patients (65%). At 2 years, recurrence was predominantly local in 7 patients (46%); whereas after 2 years, it was predominantly pulmonary 6 patients (40%). Overall, 5 year-survival was 78%., Conclusions: Cystic adenoid carcinoma is characterized by a high recurrence rate. Nevertheless, it has a high overall 5-year survival, which justifies an aggressive treatment., (Copyright: © 2020 Permanyer.)

We report an extremely rare case of a hybrid tumour of the maxillary sinus. A 51-year-old man presented with a 6-week history of nasal congestion and epiphora. Radiological imaging demonstrated a maxillary sinus tumour, with extensive local invasion. Surgical excision included maxillectomy, left eye exenteration and free flap closure. Histology of the excised specimen showed a rare hybrid tumour containing adenoid cystic carcinoma, salivary duct carcinoma, epithelial-myoepithelial carcinoma and basal cell adenoma. Hybrid tumours are very rare tumour entities which are composed of at least two distinct tumour types. Each tumour entity conforms with a defined tumour type. The tumour entities of a hybrid tumour are not separated but have an identical origin within a definite topographical area. Diagnosis and appropriate management requires high index of suspicion, pathological endeavour to look for a more aggressive accompanying tumour and adequate oncological treatment according to the highest grade of tumour., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: Adenoid cystic carcinomas (ACC) are rare malignant tumours arising in the major and minor salivary glands. Involvement of the nasal cavity and paranasal sinuses is rare and poorly described. The purpose of this study was to define the clinical and prognostic criteria of ACC of the paranasal sinuses based on the review of a series of 25 cases., Material and Methods: Single-centre retrospective study of 25 cases of ACC of the paranasal sinuses managed between 1998 and 2011, evaluating epidemiological, clinical, diagnostic and prognostic criteria. Factors influencing survival (Kaplan-Meier/Log Rank test) and the patient's quality of life (EORTC QLQ-C30 questionnaire) were also analysed., Results: Most patients (72%) had a locally-advanced tumour (stage T3 or T4) at diagnosis. Tumour sites, in decreasing order of frequency, were the maxillary sinus, nasal cavities and ethmoid sinus. The most common presenting complaints were maxillary pain or heaviness, unilateral blocked nose, and repeated epistaxis. When the tumour was resectable, treatment comprised a combination of surgery and adjuvant radiotherapy. The 5-year overall survival rate was 63% and the 5-year disease-free survival rate was 43%. The TNM stage at diagnosis (P=0.03), the histological subtype (P=0.023), the possibility of combined surgery and radiotherapy (P=0.03), and local control (P=0.05) were significant factors of improved 5-year overall survival. Positive surgical margins were associated with a trend towards poorer 5-year disease-free survival (ns)., Conclusions: ACC are rare malignant tumours associated with a poor prognosis, characterized by a high recurrence rate. Recommended treatment is a combination of surgery and adjuvant radiotherapy whenever possible. Five-year survival varies as a function of TNM stage, histological subtype, treatment options and local control., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Background: The efficacy of concurrent intra-arterial infusion chemoradiotherapy for adenoid cystic carcinoma (ACC) has been described in only a few reports. Herein, we report on 2 patients with unresectable ACC of the maxillary sinus treated with this approach., Methods: Patients received concurrent chemoradiotherapy. External beam radiation was administered 5 times a week at 2 Gray (Gy) per fraction for a total of 60 Gy. Chemotherapy consisting of docetaxel and cisplatin was administered by superselective intra-arterial infusion via a superficial temporal artery., Results: After the completion of all treatments in both patients, biopsy specimens of the primary lesions showed complete disappearance of all viable and nonviable tumor cells and new bone formation at the bony walls of the maxillary sinus. Although complications such as mucositis, neutropenia, and anorexia were observed, they were well-tolerated and manageable., Conclusion: Concurrent chemoradiotherapy using superselective intra-arterial infusion is a potential option for patients with unresectable maxillary ACC., (Copyright © 2011 Wiley Periodicals, Inc.)

Adenoid cystic carcinoma (ACC) is a rare malignant neoplasm, arising from glandular tissues, found mainly in the head and neck. Generally, it presents insidiously but can behave aggressively making its course unpredictable. Surgery and adjuvant radiotherapy continue to be the cornerstone for its treatment. ACC remains extremely difficult to treat. The authors report a case of a 37-year-old woman with bloody rhinorrhea for 6 months. She was diagnosed with a left nasal cavity lesion that was biopsied, and the anatomopathological result showed ACC. The patient was submitted to a left extended maxillectomy, microsurgical reconstruction and radiotherapy.

Objective: To study the clinical characters, the outcomes of treatments and the factors affecting long-term treatment results of adenoid cystic carcinoma (ACC) of the maxillary sinus., Methods: The clinical data of 80 patients with ACC of the maxillary sinus treated initially were analyzed retrospectively. Survival rate, local recurrence and distant metastasis were analyzed using Kaplan-Meier method. Prognosis factors were analyzed by Log-rank test and Cox regression., Results: The 5-, 10- and 15-year cumulative overall survival rates were 65.2%, 37.1%, 26.3% respectively and 5-, 10-, and 15-year disease-free survival rates 50.7%, 30.7% and 24.5% respectively. The 5-, 10- and 15-year cumulative local control rates were 68.5%, 47.3% and 47.3% respectively and the cumulative distant metastasis rate were 32.8%, 48.8% and 48.8% respectively. Prognostic factors affecting survival included T stage, pathologic grade and the modes of treatment (P < 0.05). Patients with combined therapy composed of surgery and radiation had a better local control, compared with surgery or radiation alone (χ(2) = 18.33, P < 0.01), and surgery combined with postoperative radiation was prior to preoperative radiation combined with surgery (χ(2) = 6.64, P < 0.05). Patients treated with surgery combined with preoperative radiation, either with doses of ≥ 60 Gy or with negative margins, had a better local control, compared with doses < 60 Gy and with positive margins (χ(2) = 5.06, P < 0.05). The most of patients (62.8%) died of local recurrence., Conclusions: The most of failure was due to recurrence. Combined therapy composed of surgery and radiation improves the local control and survival in patients with ACC of the maxillary sinus, compared with surgery or radiation alone. Surgery combined with postoperative radiation provides the best overall survival and local control and should be the first choice of treatments.

Objective: To study the clinical characters, management and the prognosis of patients with adenoid cystic carcinoma of the maxillary sinus., Methods: The clinical data were analyzed retrospectively for 36 patients with adenoid cystic carcinoma of the maxillary sinus to evaluate the treatment results of different modalities. The contribution of every factors influencing on survival were also analyzed. Survival analysis was performed by life table method, comparison among/between groups was performed using log-rank test, and multivariate analysis was carried out using Cox proportional hazard model., Results: The 5-year survival rate was 58.33% in all patients, while they were only 75.0% and 42.9% in stage III and stage IV lesions respectively. The 5-year survival rate of 66.7% was obtained in patients who received surgery combined with radiotherapy,71.4% and 12.5% respectively in those treated by surgery and by radiotherapy alone. Multivariate analysis indicated that stage, treatment modality, and the tumour residues in the primary treatment were the predict factors for the prognosis., Conclusions: Advanced adenoid cystic carcinoma should be treated by combined surgery and radiotherapy. Stage, treatment approach and short-term therapeutic response are the most important factors affecting the prognosis of the patients with adenoid cystic carcinoma of the maxillary sinus.

Background & Objective: Five-year survival rate of patients with maxillary malignant neoplasms is low, the prognostic factors of these neoplasms were unclear. This study was to investigate prognostic factors of maxillary sinus malignant neoplasms., Methods: Records of 151 inpatients with malignant neoplasms of maxillary sinus initially treated at Cancer Center of Sun Yat-sen University from Sep. 1983 to Mar. 1999 were reviewed. Of 151 cases, 72 were squamous cell carcinoma (SCC), 44 were adenocarcinoma, 16 were sarcoma, and 19 were other histological types; according to 1997 UICC classification, 7 were stage II, 55 were stage III, and 89 were stage IV; 66 patients received combined therapy of surgery and radiotherapy, 14 received surgery alone, 25 received radiotherapy alone, 39 received other treatments, and 7 gave up treatment. All patients were followed up for more than 5 years. Influences of clinicopathologic factors on prognosis of patients with maxillary sinus malignant neoplasms were analyzed by Kaplan-Meier method, and Cox regression model with SPSS10.0 software., Results: Five-year overall survival rate of patients of 40 years old was 33.3%(P=0.030); that of patients with SCC was 30.2%, of patients with adenocarcinoma was 57.5%, of patients with sarcoma was 24.3%, of patients with tumor of other histological types was 50.7% (P=0.011); that of patients with tumor of stage II, III, and IV were 85.7%, 45.8%, and 32.7%, respectively (P=0.029); that of patients with cervical metastases was 14.4%, of patients without cervical metastases was 44.1% (P=0.005); that of patients with distant metastases was 14.3%, of patients without distant metastases was 41.1% (P=0.011); that of patients without treatment was 14.3%, of patients treated with surgery alone was 42.9%, of patients treated with radiotherapy alone was 32.3%, of patients treated with combined therapy of surgery and radiotherapy was 50.8%, of patients treated with other treatments was 29.1% (P=0.004). Univariate survival analysis showed that the above 6 factors were prognostic factors of patients with maxillary sinus malignant neoplasms. Multivariate analysis showed that combination of surgery and radiotherapy (P=0.004, OR< 1), clinical stage (P=0.025, OR >1), SCC (P=0.016, OR >1), and sarcoma (P=0.003, OR >1) were independent prognostic factors of patients with maxillary sinus malignant neoplasms., Conclusion: For maxillary sinus malignant neoplasms, patients with SCC or sarcoma had poorer survival than patients with adenocarcinoma or other histological types of tumor; patients with sarcoma had poorer survival than patients with SCC. The higher the patient's clinical stage was, the worse his prognosis was. Combination of surgery and radiotherapy may be the best treatment for patients with maxillary sinus malignant neoplasms.

Purpose: To investigate characteristic clinical features and outcome for patients with adenoid cystic carcinoma (ACC) of the maxillary antrum., Patients and Methods: Twenty-two patients with ACC of the maxillary antrum were initially treated with surgery alone (3 patients), radiation alone (9 patients), or a combination of surgery and radiation (10 patients). Salvage treatment for initial failure was individualized. Patterns of failure, survival, and prognostic factors were retrospectively analyzed., Results: The most frequent site of failure was local recurrence at the primary site (72.7%). All patients treated with either surgery alone or radiation alone experienced one or more local recurrences, whereas patients who received planned combined surgery and radiation had a much lower local recurrence rate (40%). Neck node failure (4.6%) was an uncommon event, whereas distant metastases were clinically documented in seven patients (32%). Most of the treatment failures appeared within 5 years, but treatment failures after 5 years were not uncommon. The overall survival and disease-free survival rates at 10 years were 37.6% and 13.6%, respectively. Clinicopathological factors, such as location of primary tumor, tumor stage, and histological grade were of no value in predicting a favorable survival. The significant prognostic factors influencing 10-year survival were the pathological finding of perineural invasion and the initial mode of treatment., Conclusion: ACC of the maxillary antrum represented a unique natural history characterized by a more aggressive tumor behavior and an unfavorable prognosis. Combined surgery and radiotherapy is recommended for optimal local control and survival.

At Akita University Hospital (from 1971 to 1987) and at Chiba University Hospital (from 1983 to 1988), 17 patients with adenoid cystic carcinoma of the nose and paranasal sinuses were treated by en bloc tumor resection followed by primary reconstruction of the maxilla. Pre- and postoperative radiation was combined in 5 and 6 patients, respectively. Ten-year cancer-free survival rates were 59.3% in the 12 patients with maxillary sinus tumors and 50% in the 4 patients with nasal tumors. One patient with a sphenoid sinus tumor died within 5 years. Ten-year cancer-free survival was affected markedly by grade of tumor extension. Among T1N0M0 and T2N0M0 patients (of which there were 1 and 7, respectively), only 1 died of unrelated causes, and 6 patients survived cancer-free for more than 10 years. However, 4 of the 6 T3N0M0 patients died, and the cause of death was distant metastasis in 2, intracranial tumor extension in 1, and unrelated causes in 1. All 3 T4N0M0 patients died, 2 due to intracranial tumor extension and 1 of unrelated causes. The cause of death was distant metastasis in 3, local recurrence in 3, 2 of whom had intracranial tumor extension, and unrelated causes in 2. Prevention of distant metastasis and intracranial tumor extension was considered to be crucial for improving treatment results after en bloc tumor resection. Preoperative radiation was thought to decrease incidence of distant metastasis. In 5 patients who had preoperative radiation. 4 survived cancer-free for more than 10 years, and only 1 patient died of unrelated cause. However. of the 6 patients who had postoperative radiation, 2 died of distant metastasis and another 2 died of intracranial tumor extension. Of the 6 patients who did not have radiation therapy, the causes of death were local recurrence in 1, distant metastasis in 1 and unrelated causes in 1. Preoperative radiation in 5 patients showed histopathologically moderate or marked degeneration and necrosis of tumors in all patients. Although the number of patients in this study is too small to allow statistical analysis of the data, our present modality of treatment for adenoid cystic carcinoma of the nose and paranasal sinuses is routine combination of preoperative full dose radiation, en bloc tumor resection and primary reconstruction, including en bloc resection of the cranial base in selected T4 patients.

We report a case of adenocystic carcinoma (cylindroma) of the maxillary sinus with intracranial extension in a 35-year-old man with no previous clinical history. The patient underwent primary neurological and maxillofacial surgery. He then developed two successive recurrences of the tumor in the temporo-parietal area of the brain treated by surgery and radiotherapy for the first one and surgery alone for the second. The patient finally died 4 years and 2 months after the diagnosis. Adenoid cystic carcinomas are malignant epithelial neoplasms that arise mainly from salivary glands (14% of the salivary tumors), and less frequently from other structures. Their histological pattern can be either tubular, cribriform or solid, with different prognosis. They invade local areas (perineural spaces) and also metastasize to the lung, liver, bone, regional lymph nodes. Treatment mainly consists in combined surgery and radiation therapy. Chemotherapy might be also used.

A patient with an adenoid cystic carcinoma of the maxillary sinus was treated by adoptive immunotherapy involving intra-arterial injection of lymphokine-activated killer cells (a total number of 7 x 10(7) cells) and recombinant interleukin-2 (a total dose of 2.75 x 10(7) units) in combination with radiotherapy (60Co; total irradiation dose of 5,000 rads). Consequently, it was found that bone formation was induced in the treated tumor, which was then replaced completely by lamellar bone tissue with myxomatous stroma. This finding indicates that the tumor cells composing certain adenoid cystic carcinoma can be converted into normal-appearing, bone-forming cells by current therapy and this differentiation phenomenon leads to cure of the tumor.