[Clinical analysis of 12 cases of solitary fibrous tumors in nasal cavity, sinuses and skull base].

Objective:To summarize and analyze the clinical manifestations, diagnosis and management and prognostic features of solitary fibrous tumor（SFT） 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.

Electrochemotherapy as adjuvant treatment in a sinonasal mucosal melanoma in elderly patient: a case report.

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.

Neoadjuvant Chemotherapy in Locally Advanced Sinonasal Teratocarcinosarcoma a Rare Malignancy: An Audit From an Academic Tertiary Care Centre in India.

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.

Sinonasal adenocarcinoma presented as a giant anterior cranial fossa mass: a case report and review of the literature.

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.

Assessment of social vulnerability impact in care and prognosis of sinonasal cancers in the United States.

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.

Therapeutic approaches to sinonasal NUT carcinoma: a systematic review.

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.

Development and validation of a nomogram for predicting overall survival in patients with sinonasal mucosal melanoma.

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.

[Diagnosis and therapy of sinonasal mucosal melanoma]. / Diagnostik und Therapie von Schleimhautmelanomen der Nase/Nasennebenhöhlen.

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.

International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors.

BACKGROUND: Sinonasal neoplasms, whether benign and malignant, pose a significant challenge to clinicians and represent a model area for multidisciplinary collaboration in order to optimize patient care. The International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors (ICSNT) aims to summarize the best available evidence and presents 48 thematic and histopathology-based topics spanning the field. METHODS: In accordance with prior International Consensus Statement on Allergy and Rhinology documents, ICSNT assigned each topic as an Evidence-Based Review with Recommendations, Evidence-Based Review, and Literature Review based on the level of evidence. An international group of multidisciplinary author teams were assembled for the topic reviews using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses format, and completed sections underwent a thorough and iterative consensus-building process. The final document underwent rigorous synthesis and review prior to publication. RESULTS: The ICSNT document consists of four major sections: general principles, benign neoplasms and lesions, malignant neoplasms, and quality of life and surveillance. It covers 48 conceptual and/or histopathology-based topics relevant to sinonasal neoplasms and masses. Topics with a high level of evidence provided specific recommendations, while other areas summarized the current state of evidence. A final section highlights research opportunities and future directions, contributing to advancing knowledge and community intervention. CONCLUSION: As an embodiment of the multidisciplinary and collaborative model of care in sinonasal neoplasms and masses, ICSNT was designed as a comprehensive, international, and multidisciplinary collaborative endeavor. Its primary objective is to summarize the existing evidence in the field of sinonasal neoplasms and masses.

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

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

Clinicopathological Characteristics, Survival Outcomes, and Prognostic Factors in Adult Patients With Sinonasal Rhabdomyosarcoma: Insights from a Retrospective Cohort Study.

BACKGROUND/AIM: Rhabdomyosarcoma (RMS) is a rare tumor with distinct morphological types and challenging diagnosis. This study aimed to investigate clinicopathological characteristics, survival outcomes, and factors influencing prognosis in adult patients with sinonasal RMS, addressing a critical gap in knowledge. PATIENTS AND METHODS: This retrospective cohort study employed various statistical analyses to investigate patients with RMS. Descriptive statistics summarized demographic and clinical characteristics, while survival analysis using the Kaplan-Meier method and Cox proportional hazards model explored the relationship between covariates and survival outcomes. RESULTS: We analyzed 13 cases (7 males, 6 females) of sinonasal RMS. The average age at onset was 42.5 years (standard deviation 18.9). Tumors were observed in multiple locations, predominantly in the maxillary sinus (n=7), followed by the ethmoid sinus (n=5), and the sphenoid sinus (n=1). The study revealed a low survival rate, with 12 patients succumbing to the disease and only one patient surviving. Over time, survival probabilities declined from 92.31% (at 0.5 months) to 7.69% (at 45 months). The analysis indicated a borderline statistically significant positive association between age at diagnosis below 40 years and survival (p=0.05). Sex was found to be significantly associated with survival (p=0.03), with male patients exhibiting a higher survival rate (hazard ratio=0.08, 95%CI=0.01-0.81). CONCLUSION: This study highlights the complex nature of sinonasal RMS in adults. The low survival rate and distinct tumor locations emphasize the need for further research to improve diagnosis and treatment outcomes.

EUSICA/COST IMMUNO-model workshop fostering collaboration to advance sinonasal cancer research: A meeting report.

Sinonasal cancer is a clinically and histologically heterogeneous group of rare tumors with generally poor clinical outcomes. Their low incidence hampers the advancement of clinical management as well as translational research, and calls for multicenter and multinational collaboration between physicians and researchers. This report describes the proceedings of a two-day conference organized by the European Network for Sinonasal Cancer Research (EUSICA) and COST Action 'IMMUNO-model', fostering such collaboration and focusing on preclinical tumor and immuno models, surgical and radio-oncological treatments, core facilities for genetic characterization and molecular tumor classification, and cancer registry.

Clinical characteristics analysis of sinonasal diffuse large B-cell lymphoma and extranodal NK/T-cell lymphoma.

KEYPOINTS: Extranodal NK/T-cell lymphoma (ENKL) was the most common sinonasal lymphoma at our hospital. ENKL occurs at a younger age, and is more prevalent in the nasal cavity. ENKL had a lower Ann Arbor stage, and a better prognosis than diffuse large B-cell lymphoma (DLBLC).

Impact of Clinical Factors and Treatments on SMARCB1 (INI-1)-Deficient Sinonasal Carcinoma.

The objective of this study was to report outcomes for 19 consecutive patients with SMARCB1 (INI-1)-deficient sinonasal carcinoma. Patients were treated from 2014 to 2021 and followed for a median of 22.3 months. The median overall survival (OS) and disease-free survival (DFS) were 31.8 and 9.9 months, respectively. Patients with nasal cavity or maxillary sinus tumors had 84% better disease-specific survival (DSS) (hazard ratio [HR], 0.136; 95% confidence interval [CI], 0.028-0.66; p = .005) and 71% better DFS (HR, 0.29; 95% CI, 0.097-0.84; p = .041) than patients with other sinonasal sites. Patients who received induction chemotherapy were 76% less likely to die of disease (DSS HR, 0.241; 95% CI, 0.058-1.00; p = .047). In the largest single-institution study of SMARCB1-deficient sinonasal carcinoma to date, OS and DFS approached 3 years and 1 year, respectively, but were better for nasal cavity and maxillary sinus tumors. Patients may benefit from induction chemotherapy.

Comparison of Primary B/NKT Non-Hodgkin Lymphomas in Nasopharynx, Nasal Cavity, and Paranasal Sinuses.

OBJECTIVE: We aimed to compare clinical and survival differences between B-cell (B-NHL) and NKT-cell non-Hodgkin lymphomas (NKT-NHL) located in the nasal cavity (NC), nasopharynx, and paranasal sinuses, which are always categorized as one sinonasal type. STUDY DESIGN: Patients diagnosed with primary B-NHL and NKT-NHL in the nasal cavity, nasopharynx, and paranasal sinuses from Surveillance, Epidemiology, and End Results (SEER) database were included (1975-2017). SETTING: Population-based cohort study. METHODS: We conducted univariate and multivariate Cox regressions and Kaplan-Meier analysis to examine survival outcomes of B/NKT-NHL in the nasal cavity, nasopharynx, and paranasal sinuses, respectively. RESULTS: Overall, most B-NHL cases originated from the nasopharynx, while the majority of NKT-NHL cases occurred in the nasal cavity. Notably, the cancer-special survival (CSS) outcomes improved significantly in all sinonasal B-NHL cases over time, whereas no such improvement trend was observed in each sinonasal NKT-NHL type. Additionally, increasing age was linked with an elevated risk of death in B-NHL, particularly in the nasal cavity (Hazard ratio [HR]: 3.37), rather than in NKT-NHL. Compared with B-NHL, the adverse effect of a higher stage on CSS was more evident in NKT-NHL, particularly in its nasopharynx site (HR: 5.12). Furthermore, radiotherapy was beneficial for survival in patients with sinonasal B-NHL and NKT-NHL, except in the nasopharynx NKT-NHL. However, chemotherapy has only been beneficial for CSS in patients with paranasal sinuses B-NHL (HR: 0.42) since 2010, rather than in other types of B/NKT-NHL. CONCLUSION: Although B-NHL and NKT-NHL in the nasal cavity, nasopharynx and paranasal sinuses have similar anatomical locations, their clinicodemographics and prognoses are largely different and should be treated and studied as distinct diseases.

Biphenotypic Sinonasal Sarcoma: Case Reports of Diagnostic Findings and Clinical Management of 3 Cases.

BACKGROUND: Biphenotypic sinonasal sarcoma (BSNS) is a rare spindle cell sarcoma distinctly arising in the sinonasal area, with dual myogenic and neural differentiation, and characterised by the presence of PAX3 gene fusion, typically with MAML3. Although the majority may be indolent, up to 25% of cases reported in the literature are locally aggressive, with invasion of adjacent critical structures in the head and neck region. CASE REPORT: We report 3 cases of BSNS reviewed at our institution between 2016-2020 in addition to the current literature. Patient 1 underwent surgery followed by adjuvant radiotherapy but relapsed 24 months later and was not fit for systemic anticancer therapy and managed with palliative care. Due to comorbidities, patient 2 was recommended for active surveillance, with a view to intervening with radiotherapy should there be evidence of clinical progression. At 60 months, the nasal cavity mass remained stable on serial imaging. Patient 3 underwent primary surgical R0 resection and was offered adjuvant post operative radiotherapy 60 Gy/30 fractions/6 weeks but opted for active surveillance and has no clinical or radiological evidence of recurrence 22 months after surgery. CONCLUSION: The primary management for BSNS is surgical resection. We recommend discussing the role of postoperative adjuvant radiotherapy 60 Gy/30 fractions/6 weeks in patients who are fit for treatment. In clinical practice, dose levels will be constrained by surrounding normal tissues. At present, the role of systemic anticancer therapy is undefined. A prospective registry of ultra-rare cases may provide an evidence base with which to select optimal treatment strategies for BSNS in the future.

Tumors of the Nose and Paranasal Sinuses: Promoting Factors and Molecular Mechanisms-A Systematic Review.

Sinonasal neoplasms are uncommon diseases, characterized by heterogeneous biological behavior, which frequently results in challenges in differential diagnosis and treatment choice. The aim of this review was to examine the pathogenesis and molecular mechanisms underlying the regulation of tumor initiation and growth, in order to better define diagnostic and therapeutic strategies as well as the prognostic impact of these rare neoplasms. A systematic review according to Preferred Reporting Items for Systematic Review and Meta-Analysis criteria was conducted between September and November 2022. The authors considered the three main histological patterns of sinonasal tumors, namely Squamous Cell Carcinoma, Intestinal-Type Adenocarcinoma, and Olfactory Neuroblastoma. In total, 246 articles were eventually included in the analysis. The genetic and epigenetic changes underlying the oncogenic process were discussed, through a qualitative synthesis of the included studies. The identification of a comprehensive model of carcinogenesis for each sinonasal cancer subtype is needed, in order to pave the way toward tailored treatment approaches and improve survival for this rare and challenging group of cancers.