CASE REPORT ACC VÒM HẦU

Các khối u tuyến nước bọt chiếm vào khoảng 0,2 – 0,6% của tất cả các loại khối u và khoảng 2 – 4% khối u vùng đầu cổ. Ở Việt Nam, ước tính có khoảng 0,6 – 0,7 trường hợp u tuyến nước bọt mới mắc100.000 dân. • U tuyến nước bọt chủ yếu là u lành tính (80%), chỉ có 20 % các u tuyến nước bọt là ác tính. Trong đó ung thư tuyến mang tai chiếm 78% trong số ung thư tuyến nước bọt. • Sự phân bố rải rác của các u tuyến nước bọt phụ làm khó khăn cho việc chẩn đoánUng thư tuyến nước bọt là một bệnh ung thư hiếm gặp, nó được phát sinh trong bất kỳ các tế bào nào trong tuyến nước bọt như ở: cổ, miệng hay cổ họng.

CASE REPORT ACC VÒM HẦU BS TRƯƠNG NGỌC LỄ CASE REPORT Bệnh nhân :Phương T.A,nữ,77 tuổi Địa chỉ:Quận LDĐK:Nghẹt mũi,ù tai NỘI SOI VÒM HẦU THANH QUẢN GPB Các tuyến tiết nước bọt cho vùng miệng, họng bao gồm: tuyến mang tai, tuyến hàm, tuyến lưỡi tuyến nước bọt phụ phân bố rải rác khoang miệng, họng quản U tuyến nước bọt gặp nhiều tuyến mang tai sau tuyến hàm, lưỡi, u tuyến nước bọt phụ gặp tỷ lệ ác tính lại cao so với u tuyến nước bọt • Các khối u tuyến nước bọt chiếm vào khoảng 0,2 – 0,6% tất loại khối u khoảng – 4% khối u vùng đầu cổ Ở Việt Nam, ước tính có khoảng 0,6 – 0,7 trường hợp u tuyến nước bọt mắc/100.000 dân • U tuyến nước bọt chủ yếu u lành tính (80%), có 20 % u tuyến nước bọt ác tính Trong ung thư tuyến mang tai chiếm 78% số ung thư tuyến nước bọt • Sự phân bố rải rác u tuyến nước bọt phụ làm khó khăn cho việc chẩn đoánUng thư tuyến nước bọt bệnh ung thư gặp, phát sinh tế bào tuyến nước bọt ở: cổ, miệng hay cổ họng Nguyên nhân yếu tố nguy cơ: • Ngun nhân :khơng rõ ràng xảy số tế bào tuyến nước bọt phát triển đột biến DNA • Các yếu tố nguy : - Lớn tuổi Mặc dù xảy lứa tuổi, ung thư tuyến nước bọt thường chẩn đoán người lớn tuổi (thường 40 tuổi) - Phơi nhiễm xạ chẳng hạn xạ sử dụng để điều trị ung thư đầu cổ, làm tăng nguy ung thư tuyến nước bọt - Tiếp xúc với chất hóa học:các hợp kim niken bụi silica Triệu chứng : • thường gặp khiến bệnh nhân phải nhập viện sưng phồng vùng tuyến nước bọt, nuốt vướng, nuốt đau, liệt mặt(là dấu hiệu gợi ý u ác tính tuyến mang tai) Nghẹt mũi chảy máu mũi thường tập trung khối u vịm họng, hốc mũi • bao gồm: - Sưng phần hàm ,cổ miệng - Tê phần mặt - Yếu bên mặt - Đau dai dẳng vùng tuyến nước bọt - Khó nuốt - Khó mở miệng rộng -Nghẹt mũi,chảy máu mũi Điều trị: -Phụ thuộc vào kích thước, phân loại giai đoạn ung thư tuyến nước bọt -Điều trị ung thư tuyến nước bọt thường liên quan đến phẫu thuật, kèm xạ trị ACTA otorhinolaryngologica italica 2007;27:216-219 Case report A rare case of adenoid cystic carcinoma of the SUMMARY Adenoid cystic carcinoma is a malignant tumour involving nasopharynx manifesting as Horner’s syndrome: discussion the salivary glands, rarely developing in the nasopharynx The biological and review of the literature behaviour of adenoid cystic carcinoma is characterized by slow growth Un raro caso di carcinoma adenoido-cistico del rinofaringe manifestatosi sindrome di Horner: discussione e revisione della letteratura F Soprani, V Armaroli, A Venturini1 , E Emiliani2 , D Casolino Department of Otolaryngology, Head and Neck Surgery, Santa Maria delle Croci Hospital, Ravenna; Department of Radiation Oncology, Villa Maria Cecilia Hospital, Cotignola (RA); Department of Radiation Oncology, Santa Maria delle Croci Hospital, Ravenna, Ital rate, high tendency to local recurrence and metastatic spread Its histological features are particularly important for prognostic prediction: solid pattern has the worst out come Initial presentation of nasopharyngeal adenoid cystic carcinoma with paresis of cranial nerves and Horner’s syndrome is infrequent The Authors present a case of a rare adenoid cystic carcinoma of the nasopharynx, at admission with Horner’s syndrome, in a 66-year-old male Magnetic resonance imaging showed an expansive submucosal lesion of the nasopharynx involving the para-pharyngeal space, tensor and levator veli palati muscles and the apex of petrous bone Positron emission tomography excluded distant metastasis Definitive histopathological examination revealed an adenoid cystic carcinoma with mixed cribriform and solid pattern The patient was treated exclusively with radiotherapy (70 Gy) in 35 fractions with partial reduction of the neoplastic mass Introduction Adenoid cystic carcinoma (ACC) is a malignant tumour of the exocrine glands It most commonly arises in the salivary glands (it accounts, in fact, for 12% to 14% of malignant parotid neoplasms, for 31% to 58% of malignant tumours of the submandibular gland and for 33% to 55% of malignant tumours of the minor salivary glands), even if localizations have been described in prostate, lacrymal glands, cervix uteri, breast and bronchial mucosa 1–5 Nasopharyngeal localization is uncommon, accounting for approximately 0.5% to 4% of all the carcinomas of the nasopharynx and for 2.4% to 3.7% of all head and neck ACC 6–8 Typical features of ACC are: slow growth rate, high propensity for perineural spread, for local recurrence and for distant metastasis, usually involving lung, bones and liver 10 Histologically, three growth patterns can be recognized for ACC: cribriform, tubular and solid This is particularly important for prognostic purposes since the predominant tubular pattern has the best prognosis, the predominant solid pattern the worst, being associated with the highest incidence of distant metastasis and perineural infiltration with, consequently, 15 years survival rate of 5% 11–13 The reported incidence of invasion of the skull base ranges from 4% to 22% and can realize through various ways of spread: the peritubaric space, the branches of the trigeminal nerve and the internal carotid artery 14–16 These tumours have a long natural history characterized by a typical slow growth rate responsible for the delay in seeking early medical consultation The interval between onset of the disease and onset of the first symptoms is estimated to be between and years 17 The symptoms most commonly found are epistaxis, progressive nasal stenosis, dysfunction of the Eustachian tube and, in relation to the invasion of the skull base, disorders of ocular motility, diplopia, facial pain, dysfunction of IX, X, XI and XII pairs of cranial nerves and, more rarely, Horner’ s syndrome 17 Imaging of ACC is based on computed tomography (CT) scan, particularly helpful in detecting bony erosions of the skull base, and on Magnetic Resonance Imaging (MRI) with gadolinium, effective in demonstrating possible involvement of infra-temporal fossa, cavernous sinus, and perineural or perivascular infiltration Case report U.R.B., a 66-year-old male, was referred to the ENT Department, complaining of a visual deficit in the lateral look associated with progressive hoarseness, dysphonia and dysphagia both for liquid and solid food which had been present for months Clinical history showed chronic lung emphysema More-over, the patient was in follow-up at the Department of Gastroenterology for a gastric carcinoid tumour successfully removed by endoscopic surgery two years earlier Otolaryngologic examination revealed paralysis of the left soft palate and of the left vocal fold Investigation with flexible endoscopy was negative for macroscopic disease of the nasopharynx Neurological evaluation revealed anisocoria due to constriction (miosis) of the left pupil, drooping of the left upper eyelid (ptosis) and light sinking of the eyeball into the bony cavity of the eye (enophthalmos) All these signs led to the diagnosis of Horner’ s syndrome High resolution computed tomography (HRCT) scan of the skull base revealed vast erosion of the foramen lacerum extended to the foramen rotundum and ovale, to the apex of petrous bone and to the internal carotid artery MRI of the skull base confirmed the presence of an expansive submucosal lesion with involvement of the left para-pharyngeal space reaching the omolateral petrous bone with infiltration of tensor and levator veli palati muscles (Fig (Fig.11 According to the indications of the HRTC scan and of MRI, multiple biopsies were performed, deeply into the mucosal surface which was healthy in appearance, following the posterior margin of the left Eustachian tube Intra-operative frozen section examination of nasopharyngeal tissue demonstrated an infiltrating adenocarcinoma Definitive histological examination confirmed the diagnosis; in particular, the morphological pattern (mixed cribriform and tubular) and immunohistochemical profile (CK7+, CK20-, S100+), indicated an adenoid cystic carcinoma of the minor salivary glands (Fig (Fig.22) Considering the site and the extension of the lesion, treatment with exclusive radiation therapy was prescribed The patient was immobilized with a thermoplastic mask Treatment was delivered with conformal high energy photons (6 MV) that included the nasopharynx and skull base A total dose of 70 Gy, in 35 fractions, was administered Treatment was not interrupted with good clinical tolerance; we detected grade mucositis (WHO scale) and weight loss, resolved with adequate therapy Post-treatment MRI, performed months after the radiation therapy, showed a reduction of the neoplastic mass (Fig (Fig.33) Fig Fig Definitive histologic examination indicating adenoid cystic carcinoma of minor salivary glands with mixed cribriform and tubular pattern Post-treatment MRI, performed months after radiation therapy, showing a slight reduction of neoplastic mass This radiological finding was the same as that at the MRI control performed 18 months after radiotherapy The patient reported improvement in swallowing function Otolaryngologic revaluation did not show any significant variations in the clinical aspects found at the time of diagnosis Discussion The best treatment for ACC is unanimously considered radical surgical resection followed by radiotherapy 13 16–20 In the case of ACC with intracranic extension, it, therefore, appears important to evaluate the real benefits of surgery, also considering that this pathological condition has a slow clinical progression which allows long-term survival of many patients, even with advanced or metastatic disease 13 14 22–24 In cases of nasopharyngeal ACC, the frequent perineural and perivascular infiltrations, associated with the anatomical characteristics of the nasopharynx, however, make the surgical approach risky on account of technical difficulties, due substantially to the proximity of surgical margins to critical neural and vascular structures 13 In the literature, moreover, many studies have shown that survival rates, over a long period of time, in patients with nasopharyngeal ACC not seem to be significantly influenced by different (more or less aggressive) types of treatment 13 18 21 Surgical treatment, therefore, is often characterized by incomplete oncological radicality, consequently with increased frequency of local recurrence within years of the initial treatment 13 21 Given the proven radiosensitivity of the ACC, this tumour is, in fact, regarded as a radiosensitive, even if not radiocurable, neoplasm, thus exclusive radiotherapy can determine a reduction in tumour volume with a meaningful improvement in the clinical symptoms 13 17 20 25 26 This aspect is of great importance considering the study of Sur et al., who, analysing the prognostic factors influencing the clinical control of the disease, indicate, in the portion of the residual tumoural mass, the only parameter indicating therapeutic effectiveness 20 Vikram et al reported regression of the tumoural mass in 96% of 49 patients treated only with radiotherapy, although in 93% of the cases they observed recurrence of the disease within years 24 In cases of ACC, moreover, it should be emphasized that, also when good local control of the disease has been reached (complete resection of the tumoural mass), the presence of distant metastases is possible in 39% of the patients Another aspect to be taken into consideration is that surgical treatment of these neoplasms with extension to the skull base is associated with a significant morbidity rate, due to the frequent appearance of sequels and complications, due to vascular Although the surgical approach with attempts of oncological radicality, followed by radiotherapy, remains the treatment of choice for ACC, also in the case of a nasopharyngeal localization, exclusive radiotherapy, in those cases in which surgery is contraindicated, for general reasons or for technical difficulties, offers a valid therapeutic alternative guaranteeing good control of the disease and minimal side-effects Biomedical Research 2017; 28 (14): 6441-6445 Adenoid cystic carcinoma of nasopharynx: a single-center Retrospective study Shujun Ding1#, Tongai Jin2#, Min Xu3#, Yong Liang4, Shoufeng Wang2, Lingyun Lv5, De Huai2* Abstract Purpose: To summarize the characteristics of Adenoid Cystic Carcinoma (ACC) arising from nasopharynx by analysing 28 cases in a single center, in order to enhance the care of patients with these unusual neoplasms Patients and methods: This was a retrospective analysis of 28 patients with nasopharyngeal ACC who were treated in Huai’an cancer hospital between 1997 and 2011 Kaplan-Meier estimation was used to calculate Overall Survival (OS) Results: Most patients had locally-advanced lesions at the time of diagnosis The most common presenting complaints were nasal obstruction and blood-stained snot The peak incidence of the patients in our study is the 5th decade In this study, 11 of 28 patients received radical surgery, and the rest received non-surgery treatment (chemoradiotherapy or radiotherapy alone) The y OS rate in surgical treatment group was higher than in nonsurgical treatment group (100% vs 47.1%) and the survival curves showed OS in surgical group was better than that in non-surgical group (χ 2=3.844, P=0.05) Conclusion: Adenoid Cystic Carcinoma (ACC) in the nasopharynx is an extremely rare entity Surgery followed by postoperative radiotherapy is the main stay of treatment Locally advanced patients may also benefit from surgical treatment Our data showed that treatment approach was an important factor affecting the prognosis Introduction Adenoid Cystic Carcinoma (ACC) is an uncommon malignant tumor, accounting for 3-5% of all neoplasms of the head and neck and approximately 22% of all salivary gland cancers [1-3] ACCs are rare variants of adenocarcinoma that most frequently arisen from major salivary glands included the parotid, submandibular, sublingual salivary glands, and minor salivary glands, which were located within the upper aerodigestive tract [4] As demonstrated in the previous studies, ACC is most commonly found in the palate, followed by the paranasal sinuses and other sites of the oral cavity, while it’s rarely found in the nasopharyngeal cavity Because previous studies have shown that primary nasopharyngeal adenocarcinoma (including ACC) represents less than 0.48% of all types of nasopharyngeal cancers [5,6] In 1859, Billroth was first described this tumor as the term cylindroma Subsequently, the current name of ACC introduced by Spies has gained general acceptance [7] ACC has a distinct propensity for frequent early Perineural Invasion (PNI), and late haematogenous distant metastasis, while rarely spread to the lymph nodes Furthermore, this tumor has a strong tendency to local invasion, which undoubtedly increases the difficulty of radical resection [2,8-12] In general, planned combined surgery and radiation therapy is the accepted standard of care for minor salivary gland malignancy Some studies revealed that the lesions in the nasal cavity or maxillary sinus were generally discovered at a late stage at the time of ISSN 0970-938X www.biomedres.info Biomed Res- India 2017 Volume 28 Issue 14 Biomedical Research 2017; 28 (14): 6441-6445 6441diagnosis, which increased the difficulty of surgery or even lost the chance of surgery [13,14] ACC stands for a special diagnostic and therapeutic challenge due to its extraordinary nature This study aimed to review our long-term experience based on the study of twenty-eight patients with nasopharyngeal ACC in the light of the most recent knowledge based on a review of the literature Discussion ACC is an uncommon malignant tumor Several reports in recent years have occasionally shown ACC originates from other than the common sites such as cardia, breast, lung, esophagus [15-18] While the primary site of the nasopharynx is exceedingly rare The vast majority of nasopharyngeal carcinomas are keratinizing or non-keratinizing squamous cell carcinoma Primary nasopharyngeal adenocarcinomas (included ACC) as a group are reported to occupy 0.48% in all types [5,6] ACC is a tumor of an uncertain age Not only the old but also the young can be involved The peak incidence of the patients in our study was the fifth decade (47 y), which was similar to most studies in the literature [19-21] Controversial also exists about the ratio of male to female A meta-analysis by Martin Rodriguez and the colleagues revealed that ACC was found to be more common among women [22] While, our data revealed that there is no gender predominance between man and woman Nasopharyngeal ACC was a rare malignancy with special biological features, which has different treatment strategies compared with the common nasopharyngeal cancers The benefit of concomitant chemoradiotherapy for squamous and poorly differentiated cancers of head and neck has been proven However, the clinical effort for locally advanced or inoperable salivary gland carcinomas is poorly understood Taking its indolent clinical behaviour into consideration, surgery does represent a potential treatment option for the lesion ACC also has distinct propensities for frequent early perineural invasion, local invasion and late haematogenous distant metastases, which is considered to be high-grade malignancy and should be treated with combined-modality therapy Some efforts have been made on the optimal treatment approaches for patients experienced with nasopharyngeal ACC Nasopharyngeal malignancies, no matter what kind of pathological types, often faced with a common problem that patients often present without local symptoms In addition, ACC is characteristic by slow and insidious growth, often discovered at a late and locally advanced stage, as our results have described above [2,8-12] Radical surgical resection is the main stay of treatment for early nasopharyngeal ACC However, advanced lesions in the nasopharynx basically lost the chance of surgery Even so, Schramm and Imola study revealed that patients with locally advanced stage can also benefit from surgical treatment [23] They enrolled 23 patients presenting with T3-T4 salivary gland malignancies in the nasopharynx who received surgical resection and radiotherapy (6 patients received preoperative radiotherapy and 17 patients received postoperative radiotherapy) The outcomes were as follows: the and 10 y disease free survival rates were 67% and 48%, respectively, and the y local control rate was 77% A retrospectively study conducted by Iseli et al reviewed 183 patients with ACC, revealed that the 10 y local recurrence-free survival rate of radiotherapy alone was significantly worse (0%), compared with surgery alone (41.8%, P=0.002) and surgery followed by postoperative radiotherapy (43.5%, P=0.001) [24] In the present study, our data revealed that the y OS rate in surgical treatment group was higher than in nonsurgical treatment group (100% vs 47.1%; χ2=3.844, P=0.05), which mainly because the small number of cases failed to highlight the advantages of the operation In addition, what should to be mentioned was that patients receiving radiotherapy alone were more likely to have the lesions in the primary sites of pharyngeal or hypopharyngeal In addition, many studies reported in the literature are recommended surgery followed by postoperative radiotherapy for the management of ACC [25-27] Posteroperative Radiotherapy (PORT) was mainly applied to patients with poor prognostic factors Miglianico et al in a retrospective study revealed that patients in PORT group had a higher y locoregional control rates (78% vs 44% for surgery alone, patients received surgical treatment, and the rest received non surgical treatment The and 10 y OS in non-surgery group were quite poor compared with the patients in surgery group Our data showed that the 10 y OS rates in nasopharyngeal ACC were lower than those reported in the literature, which mainly cause by the advanced stage was considered medically unfit for surgery or patients refused surgery Another reason could explain the poor prognosis of nasopharyngeal ACC, as the outcome described above Because the lesion in the nasopharynx was proximity to surrounding essential structures such as the parapharyngeal space, skull base, cranial nerves, paranasal sinus, orbit or infratemporal fossa, there is a need to balance functional and cosmetic with oncologic consequences The complex anatomy undoubtedly increased the difficulty of surgery, interfered with a complete surgical resection and even lost the chance of surgery Previous reports have also indicated that tumors arising closer to the base of the skull, such as nasopharyngeal ACC, have worse outcomes, which resulted in low local control rate Cervical lymph node status is an important prognostic predictor for salivary glands tumors Recent and past studies are consistent in revealing a reduced survival in patients with positive lymph node at time of primary therapy, and the y survival rate was significant different with or without cervical lymph node metastasis (44-48% vs 73-77%) [28-30] In spite of the low lymph node metastasis rate of ACC, for patients with clinically positive cervical lymph node, therapeutic neck dissection is still strongly recommended at the time of primary surgery followed by adjuvant RT, regardless of histology or site [20] In our study, four patients had clinical or radiologic evidence of cervical node metastasis, however, only one patient accepted neck dissection with the evidence of metastatic ACC, the other three patients received radiotherapy This may be another reason for relatively low survival Conclusion ACC in the nasopharynx is an extremely rare entity and continues to pose diagnostic and therapeutic challenges to physicians due to its biological behaviour Surgery followed by postoperative radiotherapy may be the mainstay treatment, especially for patients presenting with early stage disease Radiotherapy alone was used if the primary was considered unresectable, or if the patient refused surgery or was considered medically unfit for surgery Our data showed that treatment approach might be an important factor affecting the prognosis of patients with advanced the nasopharyngeal ACC ADENOID CYSTIC CARCINOMA OF THE NASOPHARYNX: AN INTERESTING CASE Case report by Camilo Reyes, MD and J Kenneth Byrd, MD Case Presentation – A 46-year-old female presented to a local otolaryngologist with complaints of left ear fullness and decreased hearing Her otolaryngologist noted fluid in the middle ear space on the left side only She had no history of otitis media or significant allergies Nasal endoscopy was performed, which revealed a large, smooth, submucosal mass of the left nasopharynx obstructing the Eustachian tube A biopsy was performed under anesthesia, which was described as a “basaloid neoplasm” by the pathologist She was referred to the Head and Neck Multidisciplinary Tumor Board at Augusta University for further evaluation and management Figures and are axial and sagittal MRI images demonstrating an enhancing, submucosal tumor x x cm in size, medial to the internal carotid artery and abutting the medial pterygoid muscle, with erosion of the floor of the sphenoid sinus Enhancement of the descending palatine and vidian nerves was noted on imaging The head and neck pathologist reviewed the outside biopsy, and the diagnosis of adenoid cystic carcinoma, cribriform pattern, stage T3N0M0 was made The patient was taken to the operating room in November 2015 for endoscopic resection A right-sided nasoseptal flap was raised due to planned sacrifice of the left nasoseptal artery during the transpterygoid approach Wide bilateral sphenoidotomies and a small posterior septectomy were made to allow for visualization and four-handed dissection by two surgeons A left-sided medial maxillectomy was performed prior to entering the pterygopalatine fossa, where the descending palatine and vidian nerves (Figure 3) were biopsied by frozen section The pterygoid plates were drilled to allow for entrance into the infratemporal fossa, and the vidian canal and sphenoid floor were drilled posteriorly to the cartilage of the foramen lacerum After bone removal was adequate, tumor debulking was performed to facilitate manipulation The tumor was removed with a margin of prevertebral musculature off the lower clivus posteriorly, and dissection was taken laterally through the palatal musculature to the medial pterygoid muscle After V3 was identified in the infratemporal fossa, the internal carotid artery was localized with a Doppler probe Using these landmarks, the tumor was dissected free laterally and superiorly Transorally, the soft palate was elevated into the nasopharynx, allowing resection of the involved portions The nasoseptal flap was rotated over the defect and secured with nasal packing (Figure 4) Discussion – Adenoid cystic carcinoma (ACC) accounts for to percent of all head and neck malignancies, arising most frequently in minor salivary glands It is the third most common sinonasal malignancy overall, following squamous cell carcinoma and adenocarcinoma Within the sinonasal tract, 47-60 percent arise from the maxillary sinus and 25-30 percent from the nasal cavity.1,2 ACC of the nasopharynx is very rare, making up less than 0.5 percent of nasopharyngeal cancers and with fewer than 100 cases reported in the literature.3 Surgery followed by postoperative radiation is the treatment of choice for adenoid cystic carcinoma of the skull base Complete resection with negative margins, when possible, is associated with better outcome A recent meta-analysis of skull base ACC concluded that positive or close tumor margins and tumor located in the ethmoid or sphenoid sinuses were significant predictors of poor outcome.6 Surgical approach, endoscopic vs open, must be selected according to patient tumor location in relation to neurovascular structures Although no study has directly compared approaches, the endoscopic approach to the anterior cranial base appears to produce acceptable oncologic outcomes in appropriately selected patients.7 Intensity-modulated radiotherapy is the most frequently used modality postoperatively, although there may be a role for stereotactic radiosurgery or proton radiation in some cases.8 There is no clear benefit to chemotherapy in the treatment of ACC, as in other salivary malignancies Regional lymphatic metastases are rare, and therefore, elective neck dissection is not warranted However, hematogenous spread can be seen in up to 3040 percent of cases to the lungs and long bones and may occur even after 10 years With aggressive, multidisciplinary treatment, five- and 10-year overall survival may be as high as 78 percent and 50 percent, respectively, although a significant number of these patients will be alive with disease.9 Local recurrences and isolated metastases may be re-resected endoscopically and/or treated with stereotactic radiosurgery Histologically, cribriform (most common) and tubular patterns exhibit low-grade features, and the solid, high-grade pattern confers a worse prognosis Perineural invasion is common in adenoid cystic carcinoma and should be assessed preoperatively by thin-section, contrasted MRI Approximately 60 percent of skull base ACC have been reported to exhibit perineural spread.4 Nevertheless, the role of perineural spread on survival is still debated, as some studies have demonstrated that perineural invasion is not associated with outcome.5,6 When possible, however, large nerves in the area should be biopsied and cleared by frozen section extradurally to theoretically prevent spread to the central nervous system In this patient, early diagnosis allowed for complete endoscopic resection with negative margins As in other sinonasal and nasopharyngeal tumors, symptoms may be vague and lead to advanced stage at the time of diagnosis In this case, the referring otolaryngologist performed nasopharyngoscopy due to the unilateral middle ear effusion, leading to diagnosis of the tumor The correct diagnosis of ACC by the pathologist, rather than nasopharyngeal carcinoma, was particularly important in selecting appropriate treatment for this patient Undifferentiated nasopharyngeal carcinoma, which may look similar to the solid pattern of ACC in a limited biopsy, is primarily treated with radiation or concurrent chemoradiation, which is not an effective treatment for adenoid cystic carcinoma BÀN LUẬN -ACC vòm hầu gặp.(