Biphenotypic Sinonasal Sarcoma- A case Series with a Review of Literature.

Biphenotypic sinonasal sarcoma (BSNS) is a rare malignant tumor that affects the upper nasal cavity and ethmoid sinuses. It is more commonly found in middle-aged women and is characterized by the infiltration and hypercellular proliferation of spindle cells. These cells exhibit specific immunoreactivity. We add seven cases diagnosed as BSNS, to the handful of cases already available in the literature.BSNS is a malignant disease of the sinonasal tract that requires prompt and accurate diagnosis, followed by surgical resection and consideration of radiotherapy. Our analysis of seven cases supports previous research that confirms the aggressive nature of the disease but also shows that it is treatable with the right approach.

Radiologic overview of sinonasal lesions.

Sinonasal tumors are often malignant and comprise approximately 3% of all head and neck malignancies. Half of these tumors arise in the nasal cavity, and other common locations of origin include the ethmoid and maxillary sinuses. Some unique clinical features are anosmia and altered phonation but the most common general features include headache, epistaxis, and diplopia. CT and MRI may be used to assess tumor location, invasion of adjacent tissue, presence of metastasis, internal tumor heterogeneity, and contrast enhancement. Local invasion of the tumor beyond the sinonasal tract can impact adjacent structures such as the cranial nerves, skull base, branches of the internal carotid artery, and orbit leading to neurologic signs, facial pain, and diplopia. Imaging is used in the diagnosis, staging, and treatment planning of sinonasal tumors. This collection of benign and malignant sinonasal tumors will include some rare and unique cases with an emphasis on imaging features demonstrating a wide variety of pathologies.

Metastatic sinonasal malignancies of colorectal origin: Case report and comprehensive review of the literature.

Primary adenocarcinomas represent a small percentage of sinonasal malignancies. Metastasis of colorectal malignancies to the paranasal sinuses is rare, poorly understood, and typically fatal. This case documents an unusual source of metastatic sinonasal malignancy and offers comparison to a cohort of similar patients found in the literature.

SMARCB1 (INI-1) deficient sinonasal carcinoma of the right maxillary sinus - A rare entity.

INTRODUCTION AND IMPORTANCE: SMARCB1 (INI-1) is a vital tumour suppressor gene on chromosome 22q11.2, preventing tumour development in the SWI/SNF complex. Mutations cause SMARCB1-deficient tumours with distinct features. Loss of INI-1 expression is seen in malignancies, including sinonasal carcinoma and atypical teratoid/rhabdoid tumours. Recently recognized as a separate entity, SMARCB1-deficient sinonasal carcinomas (SDSC) are rare, clinically aggressive, and mimic other malignancies, emphasizing their significant diagnosis due to poorer prognosis, particularly in the elderly. CASE PRESENTATION: A 66-year-old male presented with a 4-month-old right cheek swelling, diagnosed initially as a sinonasal neoplastic mass. The biopsy revealed sinonasal mucosal fragments infiltrated by a tumour with plasmacytoid morphology. Immunohistochemistry (IHC) of the tumour cells was positive for p63 and pan-cytokeratin and showed INI-1 loss. Subsequent subtotal maxillectomy was performed, and the patient received adjuvant chemotherapy and radiotherapy. At a thirteen-month follow-up, the patient achieved his daily activities with no signs of recurrence. CLINICAL DISCUSSION: The loss of protein expression in sinonasal cancer is predominantly attributed to the homozygous deletion of SMARCB1. SDSC, a profoundly invasive malignant carcinoma, tends to infiltrate sinuses and extend into the intracranial regions. The IHC findings of our case were in coherence with previous studies in SMARCB1. The prognosis is particularly unfavourable in males and advanced tumours. CONCLUSION: The tumour's microscopic and immunohistochemical characteristics indicated the SDSC. Due to its aggressive nature and high mortality rates, dealing with a paranasal mass, one should be suspicious of this tumour.

Sinonasal Malignancy Following Cranial Irradiation: A Scoping Review and Case Report of Sinonasal Teratocarcinosarcoma.

Background Radiation therapy is a mainstay of treatment for brain tumors, but delayed complications include secondary malignancy which may occur months to years after treatment completion. Methods We reviewed the medical records of a 41-year-old female treated with 60 Gy of radiation for a recurrent astrocytoma, who 6 years later developed a locally advanced sinonasal teratocarcinosarcoma. We searched MEDLINE, Embase, and Web of Science to conduct a scoping review of biopsy-proven sinonasal malignancy in patients who previously received cranial irradiation for a brain tumor. Results To our knowledge, this is the first report of a patient to present with a sinonasal teratocarcinosarcoma after receiving irradiation for a brain tumor. Our scoping review of 1,907 studies produced 14 similar cases of secondary sinonasal malignancy. Median age of primary cancer diagnosis was 39.5 years old (standard deviation [SD]: 21.9), and median radiation dose was 54 Gy (SD: 20.3). Median latency time between the primary cancer and secondary sinonasal cancer was 9.5 years (SD: 5.8). Olfactory neuroblastoma was the most common sinonasal cancer ( n = 4). Fifty percent of patients died from their sinonasal cancer within 1.5 years. Conclusion Patients who receive radiation exposure to the sinonasal region for treatment of a primary brain tumor, including low doses or scatter radiation, may be at risk of a secondary sinonasal malignancy later in life. Physicians who monitor at-risk patients must be vigilant of symptoms which may suggest sinonasal malignancy, and surveillance should include radiographic review with careful monitoring for a secondary malignancy throughout the entire irradiated field.

A Case Report of Sinonasal Primary Squamous Cell Carcinoma.

This case report presents a 64-year-old male diagnosed with sinonasal primary squamous cell carcinoma (SNSCC), a rare and aggressive upper aerodigestive tract malignancy. Initially, he presented with unilateral recurrent epistaxis. Imaging and histopathology confirmed the diagnosis. The patient's non-compliance with clinic appointments led to significant disease progression, culminating in his unfortunate demise. This case underscores the importance of early detection and continuous monitoring in SNSCC, given its nonspecific early symptoms and poor prognosis. It emphasizes the necessity for heightened suspicion in patients with recurrent or unresolved sinonasal complaints, as timely intervention is crucial for better outcomes.

Preservation of orbit in tumor invasion through the periorbita in sinonasal malignancy.

OBJECTIVE: One of the possible risks of sinonasal malignancy is its possible spread in the orbit. However, there is no clear consensus among the different departments as to whether it is necessary to exenterate the orbit in limited tumorous infiltration of periorbital fat. The purpose of the study was to demonstrate that periorbital infiltration and periorbital fat invasion without involvement of deeper orbital tissues are not the indication of orbital exenteration. MATERIALS AND METHODS: Retrospective analysis was performed over a 17-year period of patients undergoing surgical treatment for sinonasal malignancy with histologically verified periorbital infiltration or deeper invasion into the orbit. A total of 32 patients were included in the study. For each group, the following data were analysed: sex, age, preoperative imaging studies, histological findings, site of origin, stage, surgical reconstruction, oncological treatment, survival, cause of death, number of recurrences in the orbit and functional status of preserved eyes. RESULTS: Based on our criteria for orbital exenteration, orbital preservation was feasible in 18 patients. Orbital exenteration was performed in 14 patients with deeper tumor infiltration. There was a statistically insignificant difference in survival between the two groups. The 5-year overall survival (OS) was 44% for the orbital preservation group (only 2 patients died from local tumor recurrence) and 34% for the orbital exenteration group. The groups did not differ in other observed factors other than the extent of orbital infiltration. In 11 (61.1%) patients, vision was without significant change after radiation therapy. In 2 (11.1%) patients, visual function was impaired due to diplopia. 5 (27.8%) patients had severely impaired vision due to optic nerve atrophy after radiation therapy. CONCLUSIONS: Our results show a relatively high survival rate in the group of patients with orbital preservation with a high chance of vision preservation, which justifies our approach to orbital preservation even in some tumors with periorbital infiltration.

The Prognostic Implications of Neutrophil-to-Lymphocyte Ratio in Olfactory Neuroblastoma.

OBJECTIVE: Olfactory neuroblastoma is a rare sinonasal malignancy with comparatively positive prognosis and survival, but with a range of biological behaviors that can be difficult to prognosticate with current means of risk stratification. Neutrophil-to-lymphocyte ratio (NLR) has been found across a diverse range of malignancies to be associated with poorer outcomes. This paper aims to elucidate the relationship of NLR with olfactory neuroblastoma to assess its prognostic value in this setting. STUDY DESIGN: Retrospective chart review. SETTING: A single tertiary care academic hospital. METHODS: The study cohort included all patients treated for initial presentation of olfactory neuroblastoma from 2004 to 2020. NLR was calculated from preoperative labs, and each patient was evaluated for Kadish staging, Hyams grade, intraoperative positive margin, use of adjuvant therapy, posttreatment recurrence, and death. All statistical analysis was conducted using R and relationship between NLR and variables was assessed via binomial logistic regression. RESULTS: Forty-four patients were included, 24 were male. Average age 52.8, average length of follow-up was 9.6 years. Patients were grouped by low (Kadish A/B) and advanced (Kadish C/D) stage, n = 23 and n = 21, respectively, and low (Hyams I/II) and high (Hyams III/IV) risk, n = 15 and n = 11, respectively. Advanced Kadish stage was associated with elevated NLR, odds ratio 5.69 [2.30, 20.7], P = .001. No other variables were associated with elevated NLR including Hyams grade, margin status, recurrence, and mortality. CONCLUSION: Higher Kadish grade is associated with elevated NLR which may provide novel prognostic value to current risk-stratifying systems.

Long-Term Follow-Up of Post Radiation Complete Sinus Opacification in Sinonasal Cancer Patients.

OBJECTIVE: Treatment of sinonasal malignancies most often requires primary or postoperative radiation treatment. Post radiation sinonasal morbidity has been previously described; however, none addressed post-radiation sinus obstruction. Our objective was to investigate the long-term outcomes of post radiation complete isolated sinus opacification (CISO). METHODS: A retrospective analysis of sinonasal cancer patients treated with radiation therapy during the years 2002 to 2022. Clinical, imaging and treatment data were collected from patients' medical records. Only patients with at least 12 months of follow-up and available imaging for review were included. RESULTS: Out of 109 patients, 37 patients were identified to meet the inclusion criteria. Mean follow-up was 58 months. 35% of patients were diagnosed with persistent post radiation CISO with a mean onset of 4 months. All these patients remained asymptomatic, and their imaging remained stable during follow-up with none developing an expanding mucocele. Ethmoid sinus tumor involvement was found to be more prevalent in the CISO group (62% vs. 25%, p-value = 0.048) as well as chemotherapy/immunotherapy (54% vs. 38%, p-value = 0.046). Multivariant analysis revealed that ethmoid sinus involvement (OR = 9.516, p-value = 0.047) and adjuvant therapy, either chemotherapy/immunotherapy (OR = 10.75, p-value = 0.036) were found to be a predictive factor for complete opacification. CONCLUSION: Our study revealed that a substantial number of post-radiation patients develop a stable and persistent CISO, often in the frontal and sphenoid sinuses. These patients remained asymptomatic, and none required surgical intervention during nearly 5 years of follow-up. LEVEL OF EVIDENCE: 4 Laryngoscope, 2024.

Recurrence morbidity of olfactory neuroblastoma.

BACKGROUND: With modern treatment paradigms, olfactory neuroblastoma (ONB) has favorable overall survival (OS); however, the incidence of recurrence remains high. The primary aims of this study were to delineate the prognosis of recurrence of ONB and explore how recurrence subsites are associated with OS, disease-specific survival (DSS), and further recurrence. METHODS: A retrospective chart review of ONB cases from nine academic centers between 2005 and 2021 was completed. Tumor characteristics, recurrence subsites, timelines to recurrence, additional recurrences, and survival estimates were determined using descriptive and time-to-event analyses. RESULTS: A final cohort of 233 patients was identified, with 70 (30.0%) patients recurring within 50.4 (standard deviation ±40.9) months of diagnosis on average, consisting of local (50%), neck (36%), intracranial (9%), and distant (6%) recurrence. Compared with subjects without recurrence, patients with recurrence had significantly different primary American Joint Committee on Cancer T stage (p < 0.001), overall stage (p < 0.001), and modified Kadish scores (p < 0.001). Histopathology identified that dural involvement and positive margins were significantly greater in recurrent cases. First recurrence was significantly associated with worse 5-year DSS (hazard ratio = 5.62; p = 0.003), and subjects with neck or local recurrence had a significantly better DSS compared to intracranial or distant recurrence. CONCLUSIONS: Recurrent cases of ONB have significantly different stages and preoperative imaging factors. Patients with local or neck recurrence, however, have better DSS than those with intracranial or distant recurrence, independent of initial tumor stage or Hyams grade. Identifying specific factors that confer an increased risk of recurrence and DSS is important for patient counseling in addition to surveillance planning.

Lymphatic drainage of sinonasal malignancies and the role of sentinel node biopsies.

BACKGROUND: Locoregional recurrence is a critical factor in the prognosis of sinonasal malignancies. Due to the rarity of these tumours, as well as the heterogeneity of histologies and anatomical subsites, there is little evidence regarding the rate and location of regional metastases in sinonasal malignancies. Elective regional lymph node dissection in the therapy of sinonasal malignancies has become controversial. On the one hand, elective regional lymph node dissection is considered to be an overtreatment in the cN0 cases. On the other hand, undetected occult lymphatic metastases are associated with a poor prognosis. In this study, we discuss the role of sentinel lymph node biopsy as a minimally invasive procedure in the treatment of sinonasal malignancies based on our two years of practical experience and the currently available data. RESULTS: This is a descriptive, monocentric, retrospective study, including 20 cases of cN0 malignant sinonasal neoplasm, that underwent a surgical therapy between 2020 and 2022. The following aspects were investigated: tumour entity, localisation of the primary tumour, tumoral stage, localisation of the sentinel lymph nodes, and postoperative complications. Squamous cell carcinoma was the most frequently diagnosed tumour entity (50%), followed by adenocarcinoma (20%) and malignant melanoma (15%), adenoid cystic carcinoma and mucoepidermoid carcinoma. Sentinel lymph nodes were most frequently found in the ipsilateral neck region I (45%), followed by the ipsilateral neck region II (40%). In all cases, the removed lymph nodes were free of malignancy. There were no postoperative complications due to lymph node biopsy. There were no recurrences during the study period. CONCLUSION: Sentinel node biopsy could add more safety to the management of cN0 sinonasal malignancies due to its low morbidity. Whether SNB could provide an alternative to elective neck dissection in the management of SNM should be investigated in further studies.

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.

Skull Base Reconstruction by Subsite after Sinonasal Malignancy Resection.

Reconstruction after the resection of sinonasal malignancies is complex and primarily dependent on the defect size and location. While the reconstructive paradigm for sellar mass resection is well delineated, the challenges associated with reconstruction after sinonasal malignancy resection are less well described. This narrative review will address the goals of reconstruction after both endonasal endoscopic and open sinonasal malignancy resection and reconstructive options specific to these subsites. The goals of reconstruction include repairing cerebrospinal fluid leaks, restoring sinonasal function, providing a nasal airway, and optimizing the patient's quality of life. These goals are often complicated by the anatomic nuances of each involved sinus. In this review, we will discuss the methods of reconstruction specific to each sinonasal subsite and describe the factors that guide choosing the optimal reconstructive technique.

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.

The medial transorbital approach in cranioendoscopic skull base tumor resections for locally advanced tumors.

BACKGROUND: Orbital structure preservation and avoidance of facial incisions without compromising oncological outcome are key to maintaining function and quality of life in locally advanced sinonasal tumor surgery. A transorbital approach at our institution has proven invaluable during cranioendoscopic skull base tumor resections and there are few descriptions of this in the literature. METHODS: An IRB-approved retrospective chart review was conducted at a tertiary cancer center for patients between 2020 and 2022 undergoing cranioendoscopic tumor resections utilizing a transorbital approach. Data collected included histopathology, sinus origin, disease extent, stage, operative details, length of stay, neo-adjuvant treatment and adjuvant treatment. Recurrence, survival, and complication rates were assessed. RESULTS: Four patients were identified for inclusion, including a SMARCB1-deficient carcinoma, esthesioneuroblastoma, squamous cell carcinoma and meningioma. All patients had resection of gross and microscopic disease with preservation of orbital contents. Post-operatively, one patient had mild diplopia on inferior gaze, all other patients had normal vision. Median follow-up was 9.5 months. One patient had recurrence of disease intracranially. CONCLUSIONS: The cranioendoscopic approach with a medial transorbital incision has multiple benefits. It avoids the need for a Weber-Ferguson incision with associated facial scar, allows for early intra-operative assessment for orbital invasion using tactile feedback and safe dissection of disease while protecting the globe and rectus muscles. This leads to preservation of eye function while ensuring an oncological resection. Other advantages include ligation of the anterior ethmoid artery and access for reconstruction of the medial orbital wall.

Biphenotypic Sinonasal Sarcoma with Orbital and Skull Base Involvement Report of 3 Cases and Systematic Review of the Literature.

Biphenotypic sinonasal sarcoma (BSNS) is a rare malignant tumour of the upper nasal cavity and ethmoid sinuses that presents predominantly in middle aged female patients and show a characteristic infiltrative and hypercellular proliferation of spindle cells that demonstrate a specific immunoreactivity. We present three cases with BSNS that had different presenting complaints, either sinonasal or orbital problems, underwent endoscopic surgical treatment and/or radiotherapy and have been disease free on long follow up. A systematic review of all published cases was performed to identify all BSNS cases known at present. BSNS requires prompt and correct diagnosis with accurate surgical resection as well as consideration of radiotherapy. Our three cases confirm the findings of the literature and support that BSNS is an aggressive but treatable malignant disease of the sinonasal tract.

A destructive sinonasal organizing hematoma mimicking malignancy.

Sinonasal organizing hematomas (SOH) are rare, benign lesions that can be mistaken for malignancies due to their unfamiliarity among clinicians and aggressive appearance on imaging, which can lead to aggressive and unnecessary therapeutic interventions. Herein, we report an unusual case of SOH in an 87-year-old female patient who sought care at a maxillofacial surgery service due to persistent right nasal obstruction and imaging findings that suggested the possibility of sinonasal malignancy. We highlight the importance of recognizing these lesions to ensure adequate treatment through a conservative approach.

Esthesioneuroblastoma: Management of the N0 neck.

Esthesioneuroblastoma is a rare sinonasal malignancy that arises from the olfactory epithelium. The overall incidence of lymph node metastases is 25%. However, neck disease can present in a delayed fashion. As such, management of the clinically negative neck is controversial, with some advocating for elective neck treatment and others recommending observation with salvage treatment if necessary. At this time, no prospective head-to-head comparisons of elective versus salvage treatment have been performed.

Understanding frozen section histopathology in Sinonasal and anterior skull base malignancy and proposed reporting guidelines.

BACKGROUND: Intraoperative frozen section histopathology (IFSH) in sinonasal and skull base surgery although widely used is not well studied. METHODS: We reviewed a database of sinonasal and anterior skull base tumors, between 1973 and 2019, and identified 312 suitable operative cases. Clinicopathologic data was collected and analyzed, in addition to descriptive data for histopathological reports classified as "ambiguous," or "limited/insufficient-quality/quantity." RESULTS: Overall, the sensitivity, specificity, positive predictive value, negative predictive value, and accuracy for IFSH were 90.2%, 97.5%, 94.2%, 95.6%, and 95.2%, respectively. IFSH for adenocarcinoma, salivary carcinoma, and SCC all demonstrated a better clinical utility with a sensitivity of 90% or greater, while it was less than 90% for esthesioneuroblastoma, melanoma, and sarcoma. Other factors such as unclear reporting, poor quality specimens, or limited quality specimens were shown to lower diagnostic performance. Based on limitations identified, we proposed a novel IFSH reporting algorithm to improve IFSH in sinonasal and skull base surgery. CONCLUSIONS: IFSH is an accurate and clinically useful technique in sinonasal and skull base surgery patients; however, limitations exist.

Multilayer Anterior Skull Base Reconstruction with Cortical Rib Bone Graft: Preliminary Experience.

OBJECTIVE: During the past decades, different methods have been described for anterior skull base reconstruction. Regarding larger skull base defects, few investigators have described the use of bone grafts to foster support and prevent frontal lobe sagging, herniation, or falling. The aim of this study is to describe the use of a rib bone graft, which could be an option in these cases due to its rigidity and dimensions. METHODS: We retrospectively collected preoperative, intraoperative, and postoperative data at the last follow-up of 10 patients who underwent multilayer anterior skull base reconstruction, including rib bone graft, for large anterior cranial base defects at 2 tertiary care academic hospitals. RESULTS: Eight patients underwent endoscopic craniectomy for sinonasal malignancies, and the other two underwent transnasal endoscopic surgery for congenital meningoencephalocele. Anterior skull base defects measured on average 3.8 cm ± 0.9 SD antero-posteriorly (range 2.5-5 cm) and 2.3 ± 0.9 SD latero-laterally (range 0.9-4 cm). Multilayer reconstruction was performed in all cases, including a rib bone graft positioned as intracranial extradural layer. No patient experienced thoracic complications during the postoperative period. No side effects related to the bone graft or meningoencephalocele occurrence were reported after a mean follow-up of 8.0 ± 6.3 months. CONCLUSIONS: The use of a cortical rib bone graft could be a safe and effective option in skull base reconstruction when managing large defects after cancer removal.