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SMARCB1 (INI-1)-deficient sinonasal carcinoma is a rare, poorly differentiated carcinoma defined by complete loss of tumor suppressor gene SMARCB1 (INI-1) within the neoplastic cell nuclei demonstrated by the immunohistochemical stain. SMARCB1 (INI-1) gene inactivation has been implicated in the pathogenesis of a diverse group of malignant neoplasms that tend to share “rhabdoid” morphology. SMARCB1 (INI-1)-deficient sinonasal carcinoma was first reported by Agaimy et al. in 2014. These tumors are often basaloid with focal rhabdoid differentiation, prominent necrosis, increased mitotic activity, and aggressive behavior. Other than being INI-1 and NUT negative, they are positive for pancytokeratin and express variable immunoreactivity for squamous markers like p63 and neuroendocrine markers like synaptophysin. Most patients present with locally advanced disease and hence a combination of chemotherapy, radiotherapy, and surgery is usually recommended.
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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.
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Humans , Carcinoma, Adenoid Cystic/pathology , Paranasal Sinus Neoplasms/pathology , Retrospective Studies , Neoplasm Recurrence, Local/pathology , PrognosisABSTRACT
Objective@#To analyze the method and effect of reconstruction of the orbital floor defect with preserved orbital contents after advanced maxillary sinus cancer resection.@*Methods@#Thirty-three patients of T3/T4 stage maxillary sinus cancer with orbital invasion who were treated in Shandong Provincial Hospital from January 2010 to October 2016 were retrospectively analyzed, including 20 males and 13 females, aged from 12 to 80 years old. Patients were treated with appropriate surgical methods according to their clinical manifestation, imaging finding, invasion range, preoperative and intraoperative pathology. Surgical approaches such as maxillectomy, expanded maxillectomy and superstructure maxillectomy were used for patients with Medpor orbit floor repairment. Patients were followed up regularly after surgery, and the surgical efficacy was discussed by descriptive statistical method.@*Results@#Of those 33 cases, 19 were squamous cell carcinoma, 8 were adenoid cystic carcinoma, 5 were inverted papilloma malignant transformation, and 1 was myoepithelial carcinoma in our study. After tumor resection and orbital floor repair, the orbital contents and eyeball function of all patients remained intact. During follow-up time ranged from 3 to 8 years, a total of 6 patients relapsed at 3 years and died of ineffective treatment. The survival rate was 81.8% (27/33).@*Conclusion@#Tumor resection and Medpor orbital floor repair in maxillary sinus cancer patients with orbital invasion can preserve the function of the eyeball well, greatly improve the quality of patient′s life.
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OBJECTIVES: Endoscopic prelacrimal medial maxillectomy (EPMM) was previously reported to treat maxillary inverted papilloma. This study aimed to compare prelacrimal recess approach with the conventional Caldwell-Luc approach (CLA) to remove benign maxillary sinus tumors and to evaluate the usefulness of this approach based on our experience. METHODS: Ten patients who underwent EPMM at our hospital from January 2013 to December 2017 were reviewed. We also reviewed 30 patients who underwent benign maxillary sinus tumor resection via CLA during the same period. From medical records, postoperative pathological results, complications due to surgery, and recurrence rate were evaluated. RESULTS: There were eight inverted papilloma, one ameloblastoma, and one ossifying fibroma in the EPMM group. In the CLA group, all 30 cases were inverted papilloma. There were no cases of failure at gross total removal during surgery, and no recurrences were observed during follow-up in either groups. Mean follow-up period was 13.0 months in CLA group and 10.8 months in EPMM group. Regarding postoperative complications, 11 patients of the CLA group (37%) and three patients of the EPMM group (30%) had numbness around the cheek and upper lip area after surgery (P=0.715). In the CLA group, there were eight patients who had numbness lasting more than 3 months after surgery, and two patients had numbness for more than 1 year. However, facial numbness disappeared within 3 months in all patients in the EPMM group, in which epiphora was not observed. CONCLUSION: EPMM is the effective surgical approach for resecting benign maxillary sinus tumor compared with CLA. Although facial numbness was reported in EPMM, the duration of numbness was shorter than CLA.
Subject(s)
Humans , Ameloblastoma , Cheek , Endoscopy , Fibroma, Ossifying , Follow-Up Studies , Hypesthesia , Lacrimal Apparatus Diseases , Lip , Maxillary Sinus Neoplasms , Maxillary Sinus , Medical Records , Papilloma, Inverted , Postoperative Complications , RecurrenceABSTRACT
OBJECTIVE To study the effect of the free anterolateral thigh myocutaneous flap on the appearance and functions after maxillectomy.METHODS The clinical data of 5 patients with malignant maxillary tumors from June,2016 to June 2017 were analyzed retrospectively.The 5 cases were T3-4N0M0 lesions.All of them received the maxillectomy and then were reconstructed with the free anterolateral thigh myocutaneous flap,and the flap was about 1.25 times of the defect in volume.RESULTS The needed volumes for the reconstruction were from 165 ml to 225 ml and the operation time of reconstruction were between 80-95 minutes.All the free flaps survived after operation.Although the reconstructed side looked swelling obviously in the first month after surgery,the appearance became good and symmetrical after the radiotherapy.No diplopia occurred and the functions of breathing,swallowing and speaking were normal.No movement disorders happened caused by the donor of thigh flap.CONCLUSION The free anterolateral thigh myocutaneous flap as the reconstructive tissue has many merits.The volume is adequate and can be regulated according to the defect.The forward appearance is better than the free anterolateral thigh flap without muscles.So the reconstruction of free anterolateral thigh myocutaneous flap is an ideal material for reconstruction because of the good functions and being easy to operate.
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OBJECTIVE To explore and evaluate the value of CT in diagnosis of malignant tumor of maxillary sinus and the accuracy of the involved bone wall by comparing the preoperative CT imaging with the pathologic examination.METHODS 11 patients without maxillary sinus squamous cell carcinoma and lymph node metastasis received pathological examination and enhanced CT scan before operation,partial or total maxillary resection were implemented according to the CT features and scope.The position and azimuth of the cut bone tissue samples were marked.The specimens were routinely fixed,decalcified,embedded,sliced and HE stained to observe the bone tissue pathological changes on the bone wall under light microscope.RESULTS Nasal sinus enhancement CT scan showed that the medial wall of maxillary sinus were all resorped and invaded(4 cases lack inner wall).Anterior wall was invaded in 6 cases,superior wall in 7 cases and bottom wall in 3 cases,posterior and exterior wall in 9 cases;After ruling out the cases without internal wall of maxillary sinus,the inner wall of the maxillary sinus was invaded by cancer cells,so was the front wall and the bottom wall.Those showed bone wall erosion on preoperative CT with continuous change but without interruption and accompanied by bone wall thickening and hardening were found without tumor invasion by postoperative pathological verification.CONCLUSION Bone wall damage on preoperative CT does not mean tumor invasion,and the probability of each maxillary sinus wall invasion is different;the comprehensive analysis found that for wormhole like change of bone wallon preoperative CT with continuous bone wall thickening and hardening of the 'reconstruction of bone destruction',there was no tumor invasion by postoperative pathological validation;Routine selection analysis of bone tissue pathology can supplement the extent diagnosis of malignant tumor of maxillary sinus on preoperative CT scan,so it can accurately estimate the T staging of tumor.It may provide a more effective basis for selection of minimally invasive surgery,postoperative evaluation of surgical effect and formulating more comprehensive treatment protocol.
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Introdução: A incidência do câncer de tireoide vem crescendo de maneira considerável, sendo o sexo feminino mais acometido. O carcinoma da tireoide pode ser classificado em bem diferenciado, pouco diferenciado, medular e anaplásico. Metástases em seio maxilar raramente são encontradas, com um número reduzido de casos na literatura. Relato do caso: Paciente do sexo feminino, 78 anos, sem queixas iniciais, trazida ao ambulatório de Cirurgia de Cabeça e Pescoço do Hospital Estadual Mario Covas por familiares, que notaram, há dois meses, protrusão de olho esquerdo associada à diminuição da acuidade visual do mesmo. Ao exame físico se notou uma tireoide cinco vezes aumentada, endurecida, heterogênea e aparentemente multinodular; assimetria de hemiface esquerda com parestesia; e proptose de órbita esquerda. O tratamento da paciente consistiu em tireoidectomia total e radioterapia adjuvante. Paciente foi a óbito no 29º pós-operatório por pneumonia nosocomial e edema agudo de pulmão. Conclusões: Paciente possuía carcinoma pouco diferenciado de tireoide, uma variante incomum de câncer tireoidiano, com metástase rara para seio maxilar. Trata-se de uma manifestação agressiva do carcinoma tireoidiano, resultando em prognóstico ruim e taxa de mortalidade alta.
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Introduction: Adenoid cystic carcinoma (ACC) is one of the most frequent malignant salivary gland tumors, which commonly affects the minor salivary glands of the mouth and is rare in the nose and paranasal sinuses. In the maxillary sinus, ACC can mimic inflammatory diseases and has a poor prognosis. Objective: To report a case of a 50-year-old man with ACC of the maxillary sinus whose clinical findings in the alveolar ridge mimicked an oroantral fistula. Case Report: An excisional biopsy was performed and histopathologic analysis revealed ACC. Lung metastases and residual tumor in the maxillary sinus were detected by imaging methods. In view of the poor general health of the patient, no new surgical intervention was performed and he was only treated by radiotherapy and follow-up. Conclusion: Although rare in the maxillary sinus, ACC should be included in the differential diagnosis of lesions affecting this site...
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Humans , Male , Middle Aged , Carcinoma, Adenoid Cystic , Maxillary Sinus , NeoplasmsABSTRACT
PURPOSE: The optimal treatment of advanced maxillary sinus cancer has been challenging for several decades. Intra-arterial chemotherapy (IAC) for head and neck cancer has been controversial. We have analyzed the long-term outcome of neoadjuvant IAC followed by radiation therapy (RT) and surgery. MATERIALS AND METHODS: Twenty-seven patients with advanced maxillary sinus cancer were treated between 1989 and 2002. Five-fluorouracil (5-FU, 500 mg/m2) was infused intra-arterially, and followed by RT (total 50.4 Gy/28 fractions). A planned surgery was performed 3 to 4 weeks after completion of IAC and RT. RESULTS: At a median follow-up of 77 months (range, 12 to 169 months), the 5-year rates of overall survival in all patients were 63%. The 5-year rates of overall survival of stage T3/T4 patients were 70.0% and 58.8%, respectively. Seven of fourteen patients with disease recurrence had a local recurrence alone. The 5-year actuarial local control rates in patients with stage T3/T4, and in all patients were 20.0%, 32.3%, and 27.4%, respectively. Overall response rate after the completion of IAC and RT was 70.3%. During the follow-up, seven patients (25.9%) showed mild to moderate late complications. The tumor extent (i.e., the involvement of either orbit and/or base of skull) appeared to be related with local recurrence. CONCLUSION: Neoadjuvant IAC with 5-FU followed by RT and surgery may be effective to improve local tumor control in the patients with advanced maxillary sinus cancer. However, local failure was still the major cause of death. Further investigations are required to determine the optimal treatment schedule, radiotherapy techniques and chemotherapy regimens.
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Humans , Appointments and Schedules , Cause of Death , Fluorouracil , Follow-Up Studies , Head and Neck Neoplasms , Infusions, Intra-Arterial , Maxillary Sinus , Maxillary Sinus Neoplasms , Orbit , RecurrenceABSTRACT
Oncogenic osteomalacia is a rare acquired paraneoplastic syndrome caused by excessive renal phosphate wasting that causes hypophosphatemia and osteomalacia. Fibroblast growth factor 23 (FGF23) has been identified as the causative factor of oncogenic osteomalacia and is also known as its diagnostic marker in immunohistochemistry studies. Recently, the authors of the present study experienced a female patient with a prolonged history of osteomalacia; a rare maxillary sinus tumor was confirmed by anti-FGF23 immunohistochemical staining as the causative tumor. The present case is the first reported case in Korea to have been diagnosed as a head and neck tumor associated with oncogenic osteomalacia according to anti-FGF23 immunohistochemical staining.
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Female , Humans , Fibroblast Growth Factors , Head , Hypophosphatemia , Immunohistochemistry , Korea , Maxillary Sinus , Maxillary Sinus Neoplasms , Neck , Neoplasms, Connective Tissue , Osteomalacia , Paraneoplastic SyndromesABSTRACT
Renal cell carcinoma is a rare metastatic malignant neoplasm of the paranasal sinus. The clinical picture and radiologic findings might sometimes be insufficient to suspect metastatic renal cell carcinoma. Therefore, the definite diagnosis should be made according to the result of intraoperative biopsy. Recently, tyrosine kinase inhibitor, sunitinib, is considered as an effective agent in the treatment of metastatic renal cell carcinoma. We report a case of 69-year-old patient with metastatic renal cell carcinoma of the right maxillary sinus with accompanying fungal sinusitis. After 6 times of chemotherapy with sunitinib malate, there was no sign of recurrence or local advancement of the tumor during the 1-year follow-up period.
Subject(s)
Aged , Humans , Biopsy , Carcinoma, Renal Cell , Follow-Up Studies , Indoles , Maxillary Sinus , Maxillary Sinus Neoplasms , Neoplasm Metastasis , Protein-Tyrosine Kinases , Pyrroles , Recurrence , Sinusitis , TyrosineABSTRACT
Objective To explore the features of inflammatory pseudotumor in the maxillary sinus on CT and MRI.Methods The CT and MRI data of 8 patients with histology-proven inflammatory pseudotumor in the maxillary sinus were retrospectively analyzed.All 8 patients underwent CT scans and 7 of them also underwent MRI examinations.In addition, the time-intensity curve (TIC) of dynamic contrast-enhanced (DCE)MR imaging were analyzed in 3 patients.Results Two cases had lesions in the left maxillary sinus, while the others showed lesions in the right maxillary sinus.All the lesions showed irregular shape with well-defined margins in 5 cases and hazy margins in 3 cases.The mean maximum diameter of the lesions was 51 mm (ranged from 29 mm to72 mm).On non-enhanced CT, the lesions were isodense to gray matter in 6 and slightly hypodense in 1 patients.Only 2 patients had post-contrast CT with moderate enhancement of their lesions.The lesions caused adjacent bony absorption and destruction with residual bony sclerosis.On MR T_1 WI, inflammatory pseudotumor showed hypointense in 2 patients and isointense in 5 cases in relative to gray matter.On T_2 WI, the lesions revealed inhomogeneous hypointense in 6 patients and isointense in 1 patients.Inflammatory pseudotumor showed heterogeneously slight contrast enhancement in 2 cases and moderate enhancement in 5 cases.The TIC showed a steady enhancement pattern in 3 patients.The lesions extended to nasal cavity in 6 cases, pterygopalatine fossa and infratemporal fossa in 4, orbit in 4, and cheek in 3 cases.Six patients underwent follow-up for 2-5 years after surgery and 2 of them were found to have recurrence.In comparison to CT, MRI was proved to show the extent of the lesions more clearly.Conclusions Bony destruction with sclerosis and hypointense signal on MR T_2 WI are typical manifestations of inflammatory pseudotumor in the maxillary sinus.Combined CT and MRI can provide clinicians with more comprehensive information for the diagnosis, therapy, and follow-up of these lesions.
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Rhabdomyosarcoma is an aggressive malignant tumor rarely developing in the head and neck in adults. In the sinonasal region, rhabdomyosarcoma constitutes a clinically important group because of the difficulty of surgical resection and its generally poor prognosis. Inverted papilloma is a relative rare and benign sinonasal lesionconstituting 0.5% to 4% of all nasal tumors that has a known propensity for recurrence, local aggressiveness, and association with transformation to malignancy. Squamous cell carcinoma is the major malignancy that is associated with inverted papilloma. To our knowledge, this is the first case report of pleomorphic rhabdomyosarcoma accompanied with inverted papilloma, and we represent the case with a review of the related literatures.
Subject(s)
Adult , Humans , Carcinoma, Squamous Cell , Head , Maxillary Sinus , Maxillary Sinus Neoplasms , Neck , Papilloma, Inverted , Prognosis , Recurrence , RhabdomyosarcomaABSTRACT
OBJECTIVE To study the methods of surgical and prostheses repair of the defects after resection of the malignant maxillary sinus tumors.METHODS In 60 cases with malignant maxillary sinus tumors,surgical defects of 18 cases were repaired by pedicle forehead flaps,sternocleidomastoid myocutaneous flap,temporalis myofascial flap,pectoralis major myocutaneous flaps or palate flaps,and the surgical defects of 50 cases were repaired with maxillary obturator prostheses.RESULTS The postoperative speaking,swallowing,respiratory function and facial appearance recovered well in 68.3% cases,not well in 20% cases,poorly in 11.7% cases.There were 3 cases with wound split,1 case with necrosis of the margin of pectoralis major myocutaneous flaps and 3 cases with cutaneous fistula in inner canthus,and 8 cases with difficulty of opening mouth after operation.CONCLUSION Surgical reconstruction and obturator prostheses are the simple and economic methods for repair the defects after resection of the malignant maxillary sinus tumors.The proper fixation of prostheses and close the defects completely are the keys to recover the functions and facial appearance.
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Hybrid carcinoma of the salivary gland is a very rare entity that has been described only in the parotid and palate. The occurrence of a hybrid carcinoma of maxillary sinus has not been reported. The diagnosis of hybrid carcinoma is important particularly when the components of tumor have different biologic behaviors. Diagnosis and treatment require a high index of suspicion, especially when the tumor is an epithelial-myoepithelial carcinoma, pathological effort to look for a more aggressive accompanying tumor, and proper oncologic treatment. We describe a case of 26-yrold woman with a hybrid carcinoma composed of epithelial-myoepithelial carcinoma with an adenoid cystic carcinoma component (cribriform pattern) in the right maxillary sinus with a brief review of the relevant literature.