ABSTRACT
BACKGROUND: High-grade non-intestinal-type sinonasal adenocarcinoma (non-ITAC) is a rare and aggressive form of adenocarcinoma with poor prognosis. The current standard treatment approach involves surgery combined with radiation therapy. However, there is a need for exploring additional treatment modalities to improve patient outcomes. CASE PRESENTATION: We present a case of a 65-year-old male patient who presented with pain in the right maxillary sinus and was diagnosed with high-grade non-ITAC following surgery. Postoperative pathology revealed tumor invasion into bone tissue and vascular invasion, necessitating further treatment. The patient underwent radiation therapy, followed by immunotherapy with carilizumab combined with chemotherapy. During the maintenance immunotherapy period, tumor progression was observed, and genetic testing identified EGFR and TP53 mutations. Consequently, the patient was treated with gefitinib, a targeted therapy drug. Notably, the patient's lung metastases showed a gradual reduction in size, indicating a favorable treatment response. The patient is currently undergoing oral treatment with gefitinib. CONCLUSIONS: This case report highlights the potential benefit of combining immunotherapy and targeted therapy in the treatment of high-grade non-ITAC. Despite the rarity of this cancer type, this approach may offer an alternative treatment strategy for patients with this aggressive disease. We hope that this case can contribute to a deeper understanding of high-grade non-ITAC and promote the application of immunotherapy and targeted therapy in improving survival rates for patients with this condition.
Subject(s)
Adenocarcinoma , Humans , Male , Aged , Adenocarcinoma/pathology , Adenocarcinoma/therapy , Adenocarcinoma/drug therapy , Maxillary Sinus Neoplasms/pathology , Maxillary Sinus Neoplasms/therapy , Maxillary Sinus Neoplasms/drug therapy , Molecular Targeted Therapy , Immunotherapy/methods , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Antibodies, Monoclonal, Humanized/therapeutic use , Antibodies, Monoclonal, Humanized/administration & dosage , Gefitinib/therapeutic use , Maxillary Sinus/pathology , Paranasal Sinus Neoplasms/pathology , Paranasal Sinus Neoplasms/therapy , Paranasal Sinus Neoplasms/drug therapy , Neoplasm GradingABSTRACT
Objective:To summarize and analyze the clinical manifestations, diagnosis and management and prognostic features of solitary fibrous tumorï¼SFTï¼ in nasal cavity, sinus and skull base. Methods:The clinical data of 12 patients with STF from nasal cavity, sinus and cranial base admitted to the Affiliated Hospital of Qingdao University from April 2014 to January 2022 were retrospectively analyzed, including 4 patients admitted to the department of Otolaryngology head and neck surgery and 8 patients admitted to the department of skull base surgery The clinical characteristics, diagnosis, management and prognosis were analyzed. Results:Twelve patients were included in this research, including 7 males and 5 females. All patients received surgical treatment, and 4 patients also received postoperative adjuvant chemoradiotherapy. After follow-up for 12-60 months, 4 patients with adjuvant radiotherapy and chemotherapy had a good prognosis, and among 8 patients who did not receive radiotherapy and chemotherapy, 6 patients had good prognosis and 2 patients showed relapse. Four patients with a history of recurrence of SFT after surgery were admitted to our hospital for surgical treatment, in which 1 patient had relapse after surgery, and none had metastasis. Nasal cavity and sinus to skull base SFT is rare. The most effective treatment for this disease is surgical resection, and postoperative adjuvant chemoradiation and long-term follow-up can achieve a better prognosis. En bloc resection is the key to treatment success.
Subject(s)
Nasal Cavity , Skull Base Neoplasms , Solitary Fibrous Tumors , Humans , Male , Female , Nasal Cavity/pathology , Solitary Fibrous Tumors/therapy , Solitary Fibrous Tumors/diagnosis , Retrospective Studies , Skull Base Neoplasms/therapy , Prognosis , Skull Base , Middle Aged , Neoplasm Recurrence, Local , Nose Neoplasms/therapy , Nose Neoplasms/diagnosis , Adult , Paranasal Sinus Neoplasms/therapyABSTRACT
AIMS: Sinonasal teratocarcinosarcomas (SNTCS) are rare sinonasal malignancies, the incidence of which is less than 1% of all tumors. There is limited data available on SNTCS's, often as case reports and small case series. The management of SNTCS is complicated because of its location, locally aggressive biology, difficulty in achieving complete resection, and limited data on chemotherapy in these malignancies. This audit was performed to understand the role of neoadjuvant chemotherapy (NACT) in SNTCS's, its ability to downstage the disease, achieve complete resection, and impact on long-term survival outcomes. METHODS: This was a retrospective analysis of a prospectively maintained database approved by the Institutional Ethics Committee (IEC). The baseline characteristics, the extent of tumor, Kadish stage, NACT regimen, and adverse events were extracted from the Electronic Medical Records and the patient's case file. Patients with baseline extensive/inoperable disease were referred for NACT from the multidisciplinary joint clinic followed by response assessment (RECIST v1.1). Patients underwent skull-base surgery if respectable post-completion of NACT, however, if deemed unresectable were treated with non-surgical modalities or palliative therapies. RESULTS: The data of 27 patients were evaluated from the year 2015-2022. The median age was 42 years (IQR:30-56) and 85.2% (n = 23) were males. The ECOG-PS was 0-1 in 88.8% (n = 24) patients. All 27 patients received NACT in view of extensive disease at presentation. 74.1% (n = 20) patients received Cisplatin-Etoposide and 25.9% (n = 7) received other chemotherapy regimens. The median number of chemotherapy cycles was 2(IQR:2-3). 96.3% patients (n = 26) completed the planned NACT cycles. 70.4% (n = 19) patients achieved a partial response in post-NACT imaging. 77.8% (n = 18) underwent surgery, 18.5% (n = 5) received CTRT, and 7.4% (n = 2) received definitive-RT alone. The median PFS and OS of the cohort was 19months (95%CI:12.0-25.6) and 23months (95%CI:5.94-40.06) respectively. CONCLUSION: NACT is safe, feasible, and effective with significant response rates, leading to effective downstaging, resectability and improved survival in patients with locally advanced SNTCS's.
Subject(s)
Carcinosarcoma , Neoadjuvant Therapy , Nose Neoplasms , Tertiary Care Centers , Humans , Male , Female , Retrospective Studies , India , Adult , Middle Aged , Neoadjuvant Therapy/methods , Carcinosarcoma/drug therapy , Carcinosarcoma/therapy , Carcinosarcoma/pathology , Paranasal Sinus Neoplasms/drug therapy , Paranasal Sinus Neoplasms/therapy , Paranasal Sinus Neoplasms/pathology , Teratoma/drug therapy , Teratoma/therapy , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Aged , Chemotherapy, Adjuvant/methodsABSTRACT
BACKGROUND: Sinonasal Mucosal Melanoma (SNMM) is a rare but aggressive disease. Surgery with free margins, when feasible, is the treatment of choice. In the last three decades, electrochemotherapy (ECT) has emerged as a local ablative procedure, performed with the Cliniporator, for cutaneous and mucosal tumours of different histology. We present a case report of an ECT treatment performed by means of a new endoscopic electrode, on an elderly patient affected by primary SNMM. METHODS: An 88-year-old man with a diagnosis of SNMM (cT4aN0M0)-Stage IV, of the left nasal fossa presented at our institution. Symptoms were epistaxis and complete left nasal obstruction. He refused sinonasal extended surgery and radiotherapy. He underwent a tumor debulking followed by ECT exclusively for symptom control, with palliative intent. RESULTS: The patient underwent SNMM debulking under general anaesthesia, followed by ECT on tumour margins. After the procedure, he had been free from symptoms for 5 months, with a good quality of life. Local recurrence was controlled with a new local debulking and ECT procedure on margins. The patient remained symptom free for the next 4 months. Seventeen months after diagnosis, the patient is mild symptomatic for sinonasal disease. Therefore, he developed a systemic disease progression. CONCLUSIONS: In our experience, ECT can be used as an adjuvant tool for symptom and local control in SNMM when extended surgery is out of curative intent or unfeasible. As expected, ECT does not appear to have any effect on systemic disease progression.
Subject(s)
Electrochemotherapy , Melanoma , Nasal Mucosa , Paranasal Sinus Neoplasms , Humans , Male , Aged, 80 and over , Electrochemotherapy/methods , Melanoma/therapy , Paranasal Sinus Neoplasms/therapy , Paranasal Sinus Neoplasms/surgery , Nasal Mucosa/pathologyABSTRACT
OBJECTIVE: The aim of this work is to comprehensively review and synthesize the literature related to sinonasal mucosal melanoma (SNMM) treatment with immunotherapy, including potentially targetable genetic mutations, survival outcomes, and adverse events. DATA SOURCES: Embase, Cochrane, Scopus, and Web of Science. REVIEW METHODS: The study protocol was designed according to Preferred Reporting Items for Systematic Reviews and Meta-analysis statement. Databases were searched from inception through May 23, 2023. RESULTS: A total of 42 studies met inclusion criteria. Twenty-four of the included studies reported genetic mutations for a combined 787 patients with SNMM. 8.1% (95% confidence interval, CI: 7.6-8.6), 18.9% (95% CI: 18.1-19.8), and 8.5% (95% CI: 8.1-9.0) of reported patients were positive for BRAF, NRAS, and KIT mutations, respectively. The presence of brisk tumor-infiltrating lymphocytes was associated with improved recurrence-free survival and overall survival (OS). Six studies reported a combined 5-year OS after adjuvant immunotherapy treatment of 42.6% (95% CI: 39.4-45.8). Thirteen studies encompassing 117 patients reported adjuvant or salvage immune checkpoint inhibitor (ICI) immunotherapy response rates: 40.2% (95% CI: 36.8-43.6) had a positive response (tumor volume reduction or resolution). Eleven studies reported direct comparisons between SNMM patients treated with or without immunotherapy; the majority (7/11) reported survival benefit for their entire cohort or select subgroups of SNMM patients. With the transition to modern ICIs, there is a stronger trend toward survival improvement with adjuvant ICI. Tumors with Ki67 <40% may respond better to ICI's. CONCLUSION: ICI therapy can be an effective in select SNMM patients, especially those with advanced/metastatic disease.
Subject(s)
Immunotherapy , Melanoma , Paranasal Sinus Neoplasms , Humans , Melanoma/therapy , Melanoma/drug therapy , Immunotherapy/methods , Paranasal Sinus Neoplasms/therapy , Nasal Mucosa , Mutation , Immune Checkpoint Inhibitors/therapeutic useABSTRACT
BACKGROUND: Intestinal adenocarcinoma accounts for less than 0.1-4% of all malignancies in the region. It is common among woodworkers and leather workers. Sinonasal adenocarcinoma usually arises from the ethmoid sinus (40%) or nasal cavity (25%). Extension to nearby structures is common, but intracranial spread is very rare. These tumors are usually treated with surgery, with a reported 5-year survival rate of 59% to 80%. CASE PRESENTATION: This is a 60-year-old Black African male patient who presented with globalized headache, nasal obstruction with snoring during sleep, anosmia, change in mentation, sometimes agitation and left-side visual loss of one-year duration with worsening his above symptoms over the last one month. He couldn't smell soap bilaterally; in his left eye he could see only hand movement at nearly 30 cm. On brain magnetic resonance imaging, there was a T1 hypo- and T2 hyper-intense anterior cranial fossa mass arising from the left ethmoid sinuses and sphenoid sinuses and compressing the left optic structures, and brain computed tomography demonstrated heterogeneous hypo- to isodense mass. Complete tumor excision achieved and discharged with significant improvement and linked to oncology unit for radiotherapy. CONCLUSION: The management of these patients is multidisciplinary, involving neurosurgeons, otolaryngologists, oncologists, and maxillofacial surgeons. Surgical resection is the main treatment strategy, followed by radiotherapy, particularly intensity-modulated therapy. Chemotherapy is used in highly advanced, metastatic, and unresectable tumors.
Subject(s)
Adenocarcinoma , Paranasal Sinus Neoplasms , Humans , Male , Middle Aged , Cranial Fossa, Anterior/diagnostic imaging , Adenocarcinoma/diagnostic imaging , Adenocarcinoma/therapy , Paranasal Sinus Neoplasms/diagnostic imaging , Paranasal Sinus Neoplasms/therapy , Anosmia , BrainABSTRACT
Tumors located in the nasal cavity, paranasal sinuses and the skull base comprise a wide range of histologic subtypes. Among them, neuroendocrine and undifferentiated tumors are rare but noteworthy, because of their distinctive features, aggressive nature, and diagnostic complexities. A literature search was conducted in the PubMed/MEDLINE and the Scopus databases from 2019 until inception. The keywords "neuroendocrine", "undifferentiated", "nose", "sinonasal", "paranasal", "skull base" were used. Thirty-eight articles referring to neuroendocrine and undifferentiated tumors of the nose, paranasal sinuses and the skull base were finally included and analyzed. Neuroendocrine and undifferentiated tumors of the nose, paranasal sinuses and the skull base are infrequent malignancies, most commonly affecting middle-aged men. They usually present with non-specific symptoms, even though ocular or neurologic manifestations may occur. Prognosis is generally poor; however, novel targeted and immunological therapies have shown promising results. Sinonasal Neuroendocrine Carcinomas (SNECs) carry distinct histological and immunohistochemical features. Management consists of surgical resection coupled with systematic therapy. Sinonasal Undifferentiated Carcinomas (SNUCs) lack specific squamous or glandular features. They typically stain positive for pancytokeratin and INI1 antibody. Treatment includes induction chemotherapy, followed by a combination of chemotherapy and radiotherapy. Olfactory neuroblastomas (ONBs) have neuroepithelial or neuroblastic features. They show diffuse positivity for various markers, including synaptophysin, chromogranin, and neuron-specific enolase (NSE). Surgical resection plus radiotherapy is considered the treatment of choice. In conclusion, neuroendocrine and undifferentiated tumors arising from the nose, paranasal sinuses and the skull base represent a unique group of malignancies. A thorough understanding of their clinical features, molecular changes, diagnostic approaches, treatment modalities, and prognostic factors is critical for providing optimal patient care. Still, continued research efforts and multidisciplinary collaboration are warranted, in order to improve outcomes for patients diagnosed with these rare and aggressive tumors.
Subject(s)
Paranasal Sinus Neoplasms , Skull Base Neoplasms , Humans , Skull Base Neoplasms/therapy , Skull Base Neoplasms/pathology , Paranasal Sinus Neoplasms/pathology , Paranasal Sinus Neoplasms/therapy , Carcinoma, Neuroendocrine/pathology , Carcinoma, Neuroendocrine/therapy , Nose Neoplasms/pathology , Nose Neoplasms/therapy , Nose Neoplasms/diagnosis , Prognosis , Carcinoma/pathology , Carcinoma/therapy , Esthesioneuroblastoma, Olfactory/therapy , Esthesioneuroblastoma, Olfactory/pathology , Esthesioneuroblastoma, Olfactory/diagnosis , Maxillary Sinus NeoplasmsABSTRACT
KEY POINT: Social determinants of health interactively influence sinonasal cancer care and prognosis. Housing-transportation and socioeconomic status showed the largest associations with disparities. The social vulnerability index can reveal the social determinants of sinonasal cancers.
Subject(s)
Paranasal Sinus Neoplasms , Humans , United States/epidemiology , Prognosis , Paranasal Sinus Neoplasms/epidemiology , Paranasal Sinus Neoplasms/therapy , Male , Female , Social Determinants of Health , Middle Aged , Aged , Social Class , Healthcare Disparities , Adult , Socioeconomic Factors , Vulnerable Populations/statistics & numerical dataABSTRACT
PURPOSE: Sinonasal nuclear protein in testis carcinoma (SNUTC) is a rare, aggressive malignancy caused by genetic rearrangements in the NUTM1 gene. The prognosis of SNUTC ranks among the most unfavorable within the naso-sinusal district, with an overall survival of 9.7 months. This systematic review aimed to determine the best therapeutic strategy for SNUTC. METHODS: We reviewed eligible articles for patient demographics, TNM and stage at presentation, best response after primary treatment, disease-free survival and overall survival (OS) times, other following therapy lines, and final outcomes. RESULTS: Among 472 unique citations, 17 studies were considered eligible, with reported treatment data for 25 patients. Most studies (n = 12) were case reports. The most frequently administered treatment regimen was surgery as primary treatment and combined radiochemotherapy as second-line or adjuvant treatment. Four patients were alive at follow-up. CONCLUSION: Basing on the existing literature, a standardized line in the treatment of SNUTC is not yet well delineated. A self-personalized strategy of therapy should be drawn on each patient affected by SNUTC.
Subject(s)
Nuclear Proteins , Humans , Carcinoma/therapy , Carcinoma/genetics , Carcinoma/pathology , Combined Modality Therapy , Neoplasm Proteins/genetics , Neoplasm Staging , Nuclear Proteins/genetics , Oncogene Proteins/genetics , Paranasal Sinus Neoplasms/therapy , Paranasal Sinus Neoplasms/genetics , Paranasal Sinus Neoplasms/pathologyABSTRACT
BACKGROUND: Sinonasal mucosal melanoma (SNMM) is a relatively rare malignant tumour with a poor prognosis. This study was designed to identify prognostic factors and establish a nomogram model to predict the overall survival (OS) of patients with SNMM. METHODS: A total of 459 patients with SNMM were selected from the Surveillance, Epidemiology, and End Results (SEER) database as the training cohort. Univariate and multivariate Cox regression analyses were used to screen for independent factors associated with patient prognosis and develop the nomogram model. In addition, external validation was performed to evaluate the effectiveness of the nomogram with a cohort of 34 patients with SNMM from Peking Union Medical College Hospital. RESULTS: The median OS in the cohort from the SEER database was 28 months. The 1-year, 3-year and 5-year OS rates were 69.8%, 40.4%, and 30.0%, respectively. Multivariate Cox regression analysis indicated that age, T stage, N stage, surgery and radiotherapy were independent variables associated with OS. The areas under the receiver operating characteristic curves (AUCs) of the nomograms for predicting 1-, 3- and 5-year OS were 0.78, 0.71 and 0.71, respectively, in the training cohort. In the validation cohort, the area under the curve (AUC) of the nomogram for predicting 1-, 3- and 5-year OS were 0.90, 0.75 and 0.78, respectively. Patients were classified into low- and high-risk groups based on the total score of the nomogram. Patients in the low-risk group had a significantly better survival prognosis than patients in the high-risk group in both the training cohort (P < 0.0001) and the validation cohort (P = 0.0016). CONCLUSION: We established and validated a novel nomogram model to predict the OS of SNMM patients stratified by age, T stage, N stage, surgery and radiotherapy. This predictive tool is of potential importance in the realms of patient counselling and clinical decision-making.
Subject(s)
Melanoma , Paranasal Sinus Neoplasms , Humans , Nomograms , Melanoma/therapy , Paranasal Sinus Neoplasms/therapy , Area Under Curve , Clinical Decision-Making , Prognosis , SEER ProgramABSTRACT
BACKGROUND: Patients with sinonasal malignancy (SNM) present with significant sinonasal quality of life (QOL) impairment. Global sinonasal QOL as measured by the 22-item Sinonasal Outcomes Test (SNOT-22) has been shown to improve with treatment. This study aims to characterize SNOT-22 subdomain outcomes in SNM. METHODS: Patients diagnosed with SNM were prospectively enrolled in a multi-center patient registry. SNOT-22 scores were collected at the time of diagnosis and through the post-treatment period for up to 5 years. Multivariable regression analysis was used to identify drivers of variation in SNOT-22 subdomains. RESULTS: Note that 234 patients were reviewed, with a mean follow-up of 22 months (3 months-64 months). Rhinologic, psychological, and sleep subdomains significantly improved versus baseline (all p < 0.05). Subanalysis of 40 patients with follow-up at all timepoints showed statistically significant improvement in rhinologic, extra-nasal, psychological, and sleep subdomains, with minimal clinically important difference met between 2 and 5 years in sleep and psychological subdomains. Adjuvant chemoradiation was associated with worse outcomes in rhinologic (adjusted odds ratio (5.22 [1.69-8.66])), extra-nasal (2.21 [0.22-4.17]) and ear/facial (5.53 [2.10-8.91]) subdomains. Pterygopalatine fossa involvement was associated with worse outcomes in rhinologic (3.22 [0.54-5.93]) and ear/facial (2.97 [0.32-5.65]) subdomains. Positive margins (5.74 [2.17-9.29]) and surgical approach-combined versus endoscopic (3.41 [0.78-6.05])-were associated with worse psychological outcomes. Adjuvant radiation (2.28 [0.18-4.40]) was associated with worse sleep outcomes. CONCLUSIONS: Sinonasal QOL improvements associated with treatment of SNM are driven by rhinologic, extra-nasal, psychological, and sleep subdomains.
Subject(s)
Paranasal Sinus Neoplasms , Quality of Life , Humans , Male , Female , Middle Aged , Prospective Studies , Aged , Paranasal Sinus Neoplasms/surgery , Paranasal Sinus Neoplasms/therapy , Sino-Nasal Outcome Test , Treatment Outcome , AdultABSTRACT
Sinonasal mucosal melanoma (SNMM) is a rare and aggressive disease representing only 4% of all sinonasal malignancies and 1.4% of all melanomas. With an incidence of approximately 0.2 to 2 cases per million, the disease represents a very rare cancer type. As a result, there is a lack of data and most of the evidence for this highly aggressive disease is based on retrospective observations and analyses. The standard of care is radical tumor resection followed by an adjuvant radiotherapy. Nevertheless, the rate of local recurrence is high, up to 50%. In addition, the majority of patients (up to 70%) develop distant metastases during the course of their disease. Both contribute to the extremely poor prognosis of the disease. Mucosal melanomas (SM) and cutaneous melanomas (CM) behave differently with respect to biology, clinic presentation and prognosis. Compared to CM, survival rates are significantly lower for SM. The 5-year survival rate is around 25% in SNMM but 39-97% in cutaneous melanoma. Similar to CM, immune checkpoint inhibitors achieve promising results in SM. However, response rates are lower in SM compared to CM. The goal of this CME article is to provide an overview on biology, diagnosis, therapy, and prognosis of SNMM.
Subject(s)
Melanoma , Paranasal Sinus Neoplasms , Skin Neoplasms , Humans , Melanoma/diagnosis , Melanoma/therapy , Retrospective Studies , Paranasal Sinus Neoplasms/diagnosis , Paranasal Sinus Neoplasms/therapy , Radiotherapy, AdjuvantABSTRACT
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.
Subject(s)
Humans , Carcinoma, Adenoid Cystic/pathology , Paranasal Sinus Neoplasms/therapy , Prognosis , Proportional Hazards Models , Retrospective StudiesABSTRACT
RESUMEN El melanoma mucoso maligno es un tumor neuroectodérmico de menos del 5% de las neoplasias nasosinusales. Es un tumor de mal pronóstico clásicamente su promedio de supervivencia a cinco años es cercano al 28% y 17,5% a diez años, siendo aún peor cuando existe metástasis en cuello. Se presentan a continuación dos casos de melanoma mucoso en cavidad nasal y senos paranasales, el primero mujer 58 años con melanoma septal llevada a septotomía abierta más quimioterapia y radioterapia adyuvante, con recurrencia en cuello submaxilar derecho tratada con vaciamiento supraomohioideo actualmente sin recurrencia; el segundo hombre de 40 años con antecedente de resección melanoma septal quien presenta nuevo melanoma región etmoidal izquierda luego de su primera cirugía y es llevado a resección endoscópica transnasal sin tratamiento adyuvante por decisión del paciente.
ABSTRACT Sinonasal mucosal melanoma is a neuroectodermic tumor account less 5% of nasosinusal tumors. It has a poor prognosis with a five-year average survival near 28% and 17.5% to 10 years; if neck metastasis is even worst. Here we present two clinical cases. First one a woman 58 years old with septal melanoma who receives an open septectomy with chemotherapy and radiotherapy with submaxillary recurrence treated with supraomohyoid neck dissection. The second case, a man 40 years old with previous septal melanoma surgery who has an ethmoid sinus recurrence and receives transnasal endoscopic surgery without adjuvant therapy by patient desire.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Melanoma/pathology , Nasal Mucosa/pathology , Paranasal Sinus Neoplasms/pathology , Paranasal Sinus Neoplasms/therapy , Nasal Obstruction/etiology , Nose Neoplasms/pathology , Nose Neoplasms/therapy , Radiotherapy, Adjuvant , Melanoma/therapy , Nasal Mucosa/surgeryABSTRACT
Los tumores nasosinusales son neoplasias poco frecuentes. Su epidemiología, histopatología y características clínicas son diferentes a las del resto de neoplasias malignas de cabeza y cuello. El diagnóstico y tratamiento de estos tumores plantea diversos desafíos debido a su escasa incidencia, su diversidad histológica, la producción de sintomatología inespecífica en los estadios precoces y por tener un pronóstico variable en función de su histología, lugar de origen y estadificación. Su localización centrofacial y la proximidad de estructuras como la órbita y la base del cráneo hacen que su tratamiento sea difícil y complejo, conllevando una elevada morbimortalidad. La cirugía seguida de radioterapia es el tratamiento de elección en la mayor parte de los casos. Para conseguir unos buenos resultados se requiere de equipos multidisciplinares altamente especializados. En este artículo se expone un protocolo de consenso para el tratamiento de los tumores nasosinusales realizado por la Sociedad Española de Otorrinolaringología en colaboración con la Sociedad Española de Oncología Médica y la Sociedad Española de Oncología Radioterápica (AU)
Sinonasal tumors are rare neoplasms with distinctive clinical, aetiological and pathological features. The diagnosis and treatment of these tumours is challenging because of their low incidence, histological diversity and production of non-specific symptoms in the early stages. They have a variable prognosis depending on their histology, origin and staging. Their location, close to neurocritical structures, which are of special relevance to surgery and postoperative treatment, makes their treatment difficult and complex, leading to high morbidity and mortality. Surgery followed by radiotherapy is the mainstay of treatment. To provide the best possible care, patients with sinonasal cancer should be treated in clinical referral centres specializing in skull-base pathologies. Such centres should include a multidisciplinary team led by otolaryngologist surgeons. This article outlines a consensus protocol for the management of these tumours devised by the Spanish Society of Otolaryngology in collaboration with the Spanish Society of Medical Oncology and the Spanish Society for Radiation Oncology (AU)
Subject(s)
Humans , Paranasal Sinus Neoplasms/therapy , Chemoradiotherapy , Nasal Surgical Procedures , Antineoplastic Agents/therapeutic use , Practice Patterns, Physicians' , Paranasal Sinus Neoplasms/pathology , Esthesioneuroblastoma, Olfactory/epidemiology , Disease-Free Survival , Neoplasm StagingABSTRACT
El aumento de volumen en la región frontal puede deberse a múltiples etiologías, dentro de las cuales deben considerarse: mucocele, tumor de Pott-Puffy, lesiones fibro-óseas, tumores de nariz y senos paranasales, lesiones intracraneales y metástasis. El objetivo del estudio fue describir el protocolo clínico empleado en los pacientes que se presentaron con aumento de volumen frontal y una propuesta de estadificación de las lesiones inflamatorias. Se realizó un estudio retrospectivo observacional. Se encontraron 7 casos con aumento de volumen en la región frontal: 4 casos secundarios a enfermedad inflamatoria (3 casos tumor de Pott-Puffy, un mucocele frontal) y 3 por neoplasia (un caso benigno y 2 malignos). Es muy importante considerar, entre los diagnósticos diferenciales de aumento de volumen en la región frontal, enfermedades inflamatorias que pueden representar una complicación grave de infecciones nasosinusales o neoplasias malignas avanzadas. Se propone un sistema de estadificación de las lesiones inflamatorias frontales (AU)
Frontal swelling can be due to multiple etiologies, including: mucocele, Pott's puffy tumor, fibro osseous lesions, benign and malignant neoplasms of the nose and paranasal sinuses, intracranial lesions, and metastasis. The objective of this study was to describe the clinical protocol used for the diagnosis of patients presented with frontal swelling and the proposal for staging of inflammatory lesions. We performed an observational retrospective analysis. We found 7 cases of patients with frontal swelling: 4 cases secondary to inflammatory pathology (3 Potts puffy tumors and one frontal mucocele), and 3 cases secondary to neoplasms (one benign and 2 malignant neoplasms). It's very important to consider the wide differential diagnosis that can present as frontal swelling, from inflammatory pathologies secondary to possible advanced infections of the paranasal sinuses to invasive malignant neoplasms. We propose a system of staging of frontal inflammatory lesions (AU)
Subject(s)
Humans , Male , Female , Frontal Sinusitis/diagnosis , Frontal Sinusitis/etiology , Frontal Sinusitis/therapy , Frontal Sinus/injuries , Pott Puffy Tumor/surgery , Pott Puffy Tumor/etiology , Osteoma/diagnosis , Diagnosis, Differential , Osteoma/therapy , Mucocele/diagnosis , Mucocele/therapy , Paranasal Sinus Neoplasms/diagnosis , Paranasal Sinus Neoplasms/therapy , Neoplasm Metastasis/pathology , Neoplasm Metastasis/therapy , Retrospective Studies , Observational Study , Magnetic Resonance Spectroscopy/methodsABSTRACT
BACKGROUND: Inadequate epidemiologic investigations of the paranasal sinuses malignancies prompted this retrospective study with special emphasis on a major group of 111 tumors. MATERIAL AND METHODS: Clinical records of 111 patients with histologically confirmed malignant tumors of the paranasal sinuses were investigated retrospectively from April 2000 to January 2012. Collection of data included demographic information, clinical manifestations, treatment plans, and histopathology of the tumor. RESULTS: There were 69 (62.16%) male and 42 (37.83%) female patients (male-to-female ratio of 1.6:1), with a median age of 49±12.2 years (range 21 to 88 years). A high level of occurrence was noticed in the fifth (26.3%) decade of life. The most frequent histological types were squamous cell carcinoma (43.5%) and adenoid cystic carcinoma (19%). Among clinical manifestations, nasal obstruction was the most frequent followed by diplopia, and facial swelling. Fifty three patients (47.74%) were treated with combined approach of surgery and radiation therapy. CONCLUSIONS: Paranasal sinuses malignancies are rare conditions with nonspecific symptoms which make early diagnosis of the lesions more challenging. The optimal therapeutic protocol for patients suffering from these tumors is still a somewhat controversial entity and requires further studies
Subject(s)
Humans , Paranasal Sinus Neoplasms/therapy , Histological Techniques , Retrospective Studies , Radiotherapy/methods , Antineoplastic Agents/therapeutic use , Head and Neck Neoplasms/surgeryABSTRACT
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Subject(s)
Humans , Male , Adult , Carcinoma, Merkel Cell/diagnosis , Carcinoma, Merkel Cell/therapy , Ethmoid Sinus/pathology , Ethmoid Sinus , Paranasal Sinus Neoplasms/diagnosis , Paranasal Sinus Neoplasms/therapy , Immunohistochemistry/methods , ImmunohistochemistryABSTRACT
We report on a patient who presented at our clinic with a growth of the right palate of twenty years duration. A biopsy of the area was performed and the lesion was diagnosed as low-grade mucoepidermoid carcinoma (MEC). Due to the size of the tumor, it was decided to begin treatment with radiotherapy followed by chemotherapy. Once the initial treatment was completed, the lesion was reevaluated and surgery followed by reconstruction was recommended. The Patient rejected the recommended treatment and opted to enrolled in a pain management, palliative care program.
Reportamos sobre un paciente que se presentó con un crecimiento del paladar derecho de veinte años de evolución. Se realizó una biopsia del área y se diagnosticó la lesión como carcinoma mucoepidermoide (MEC) bajo grado. Debido al tamaño del tumor, se decidió comenzar el tratamiento con radioterapia seguida de quimioterapia. Una vez que el tratamiento inicial se completó, la lesión fue reevaluada y se recomendó la cirugía seguida de reconstrucción. El paciente rechazó el tratamiento recomendado y optó por seguir un tratamiento de manejo del dolor, programa de cuidados paliativos.