Leiomyosarcoma of sphenoid sinus- A rare tumor at an unusual site.

Leimyosarcoma (lms) is a malignant soft tissue tumor of smooth muscles. The tumor arises intramuscularly and in subcutaneous locations. It is unusual to encounter lms in head and neck region, even more infrequent to discover lms in nasal and paranasal sinuses. A case of 28 years old male with leiomyosarcoma originating from sphenoid sinus with intracranial extension is being presented with aim to highlight its rarity and to highlight the differential diagnosis and the need for prudent diagnosis in the work-up of the patient.

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.

A Minimally Invasive Endoscopic Transnasal and Transorbital Approach to Sinonasal Tumor Resection.

A 35-year-old female with an extensive biphenotypic sinonasal sarcoma (BSNS) eroding through the left frontal sinus into the orbit required gross total resection of the tumor. We wanted to forgo an open approach and provide a less invasive alternative via combined endonasal and transorbital endoscopic techniques to allow for tumor removal through small, well-disguised incisions. We utilized three portals-nasal, transorbital, and anterior table window-to create interconnected orbit-sinonasal corridors, thus generating alternate pathways for visualization and manipulation of this extensive tumor. Laryngoscope, 134:1308-1312, 2024.

SMARCB1 (INI1)-deficient sinonasal carcinoma manifesting as oral lesions: A report of two cases.

BACKGROUND: Sinonasal carcinomas represent a rare group of malignancies, accounting for less than 5% of all head and neck cancers and a worldwide incidence of less than 1 case per 100 000 inhabitants annually. Despite the restricted anatomical location, sinonasal carcinomas harbor some of the most histologically and molecularly diverse groups of tumors. SMARCB1 (INI1)-deficient sinonasal carcinomas are locally aggressive tumors commonly detected late, leading to devastating morbidity and mortality. CASE REPORT: We present two cases of SMARCB1-deficient sinonasal carcinoma involving the oral cavity and presenting as progressive radiolucent lesions with local swelling associated with maxillary dentition and alveolar bone. Both cases were initially considered odontogenic in origin and involved the destruction of the left anterior maxilla. CONCLUSION: Given the rarity and the variable presentation of these tumors, they pose a challenge for head and neck surgeons, dentists, and pathologists due to the potential overlapping features with odontogenic and non-odontogenic inflammatory and neoplastic lesions. These cases highlight the importance of a multidisciplinary team and include SMARCB1-deficient sinonasal carcinomas in the differential diagnosis of destructive lesions of the maxilla.

Computed Tomography Imaging Patterns of Sinonasal Inverted Papillomas: Comparison of Primary and Recurrent Disease.

OBJECTIVE: To analyze clinical and radiographic features that may impact the rate of focal hyperostosis (FH) on computed tomography (CT) for primary and recurrent sinonasal inverted papillomas (IPs) as well as highlight factors that may affect concordance between FH and IP true attachment point (TAP). METHODS: All IPs resected between 2006 and 2022 were retrospectively reviewed. CTs were read by a neuroradiologist blinded to operative details. IP with malignancy was excluded. Operative reports and long-term follow-up data were evaluated. RESULTS: Of 92 IPs, 60.1% had FH, 25% had no CT bony changes, and 20.7% were revision cases. The recurrence rate for rhinologists was 10.5% overall and 7.3% for primary IPs. Primary and revision IPs had a similar rate of FH (63% vs. 52.6%; p = 0.646) and FH-TAP agreement (71.7% vs. 90%; p = 0.664). Nasal cavity IPs, especially with septal attachment, were more likely to lack bony changes on CT (57.1%) compared to other subsites (p = 0.018). Recurrent tumors were 16 mm larger on average (55 mm vs. 39 mm; p = 0.008). FH (75.0% vs. 60.9%; p = 0.295), FH-TAP concordance (91.7% vs. 74.4%; p = 0.094), and secondary IP (18.8% vs. 20.3%; p = 0.889) rates were similar between recurrent and nonrecurrent tumors. CONCLUSION: Primary and revision IPs have a similar rate of FH and FH-TAP agreement. Nasal cavity IPs are less likely to exhibit bony CT changes. Lower recurrence was associated with smaller size and fellowship training but not multiple TAPs, revision, FH absence, or FH-TAP discordance. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:1591-1596, 2024.

Evaluation of narrow-band imaging in the diagnosis of sinonasal inverted papilloma.

KEY POINTS: Narrow-band imaging (NBI) can be used to differentiate benign sinonasal lesions NBI can be used in the preoperative identification of sinonasal inverted papilloma Future studies can focus on NBI for recurrent inverted papilloma and surgical margin guidance.

Preoperative prediction of sinonasal papilloma by artificial intelligence using nasal video endoscopy: a retrospective study.

Sinonasal inverted papilloma (IP) is at risk of recurrence and malignancy, and early diagnosis using nasal endoscopy is essential. We thus developed a diagnostic system using artificial intelligence (AI) to identify nasal sinus papilloma. Endoscopic surgery videos of 53 patients undergoing endoscopic sinus surgery were edited to train and evaluate deep neural network models and then a diagnostic system was developed. The correct diagnosis rate based on visual examination by otolaryngologists was also evaluated using the same videos and compared with that of the AI diagnostic system patients. Main outcomes evaluated included the percentage of correct diagnoses compared to AI diagnosis and the correct diagnosis rate for otolaryngologists based on years of practice experience. The diagnostic system had an area under the curve of 0.874, accuracy of 0.843, false positive rate of 0.124, and false negative rate of 0.191. The average correct diagnosis rate among otolaryngologists was 69.4%, indicating that the AI was highly accurate. Evidently, although the number of cases was small, a highly accurate diagnostic system was created. Future studies with larger samples to improve the accuracy of the system and expand the range of diseases that can be detected for more clinical applications are warranted.

Computed tomographic characteristics of frontal sinus ossifying fibroma in a dog.

A 10-year-old female spayed Kelpie cross was presented to The Austin Vet Specialists for further investigation of a mineralized, lobulated frontal sinus mass that had previously been detected radiographically. Computed tomography (CT) revealed a large, expansile, well-defined, heterogeneously mineral attenuating mass invading both frontal sinuses. The mass was surgically debulked via a frontal sinusotomy approach. Histopathology was consistent with ossifying fibroma. This  is the first published report to describe frontal sinus ossifying fibroma in a dog, and the second to describe CT features of ossifying fibroma involving the cranium in a dog.

Extended sphenoidotomy combined with transpterygoid approach for sphenoidal sinus inverted papilloma.

PURPOSE: Inverted papilloma of the sphenoid sinus (IPSS) is a rare tumor with debated surgical management due to its proximity to vital structures. The aim of this manuscript is to highlight the role of a transpterygoid approach (TPA) and pedicle-orientated strategy in case of involvement of critical structures in IPSS and compare it with data from the literature. METHODS: Patients with primary IPSS between January 2000 and June 2021 were included. Pre-operative CT/MRI were analyzed to classify the pneumatization of the SS and predict the insertion point of the inverted papilloma. All patients were treated with a trans-sphenoidal approach which was combined with a TPA in case of lateral insertion point. A systematic search was also performed to summarize the available literature. RESULTS: Twenty-two patients were treated for IPSS. By CT, the SS was categorized with type III pneumatization in 72.8% of cases. Eleven patients (50%) were treated with a TPA with a statistical association with the insertion point on the SS lateral wall (p = 0.01), rather than a SS pneumatization (p = 0.63). The overall success was 95.5% after a mean follow-up of 35.9 months. For the literature, 26 publications were included on 97 patients and described a trans-sphenoidal approach with a success rate of 84.6% after a mean follow-up of 24.5 months. CONCLUSION: IPSS is generally treated with a sphenoidotomy approach, although in selected cases, a TPA should be preferred to expose the whole SS lateral wall though allowing a complete pedicled oriented resection of the tumor.

Novel PAX3::INO80D Fusion in Biphenotypic Sinonasal Sarcoma in an Adult.

This case report describes a novel gene fusion of PAX3::INO80D in a biphenotypic sinonasal carcinoma in a woman in her 40s.

Malignant and Nonmalignant Sinonasal Tumors.

Sinonasal tumors are rare, diverse, complex lesions with overlapping demographic and clinical features. Malignant tumors are more common, with a grave prognosis, and require biopsy for accurate diagnosis. This article briefly reviews the classification of sinonasal tumors and provides imaging examples and imaging characteristics of each clinically important nasal and paranasal mass lesions. Although there are no true pathognomonic imaging features, it is important for the radiologist to have a broad knowledge of the various CT and MR imaging findings that can help narrow the differential diagnosis and aid in early diagnosis and mapping of tumor for treatment planning.

Whole-tumor histogram analysis of apparent diffusion coefficient maps with machine learning algorithms for predicting histologic grade of sinonasal squamous cell carcinoma: a preliminary study.

PURPOSE: Accurate histologic grade assessment is helpful for clinical decision making and prognostic assessment of sinonasal squamous cell carcinoma (SNSCC). This research aimed to explore whether whole-tumor histogram analysis of apparent diffusion coefficient (ADC) maps with machine learning algorithms can predict histologic grade of SNSCC. METHODS: One hundred and forty-seven patients with pathologically diagnosed SNSCC formed this retrospective study. Sixty-six patients were low-grade (grade I/II) and eighty-one patients were high-grade (grade III). Eighteen histogram features were obtained from quantitative ADC maps. Additionally, the mean ADC value and clinical features were analyzed for comparison with histogram features. Machine learning algorithms were applied to build the best diagnostic model for predicting histological grade. The receiver operating characteristic (ROC) curve was used to evaluate the performance of each model prediction, and the area under the ROC curve (AUC) were analyzed. RESULTS: The histogram model based on three features (10th Percentile, Mean, and 90th Percentile) with support vector machine (SVM) classifier demonstrated excellent diagnostic performance, with an AUC of 0.947 on the testing dataset. The AUC of the histogram model was similar to that of the mean ADC value model (0.947 vs 0.957; P = 0.7029). The poor diagnostic performance of the clinical model (AUC = 0.692) was improved by the combined model incorporating histogram features or mean ADC value (P < 0.05). CONCLUSION: ADC histogram analysis improved the projection of SNSCC histologic grade, compared with clinical model. The complex histogram model had comparable but not better performance than mean ADC value model.

SMARCB1-Deficient Sinonasal Carcinoma: Case Report and Review of the Literature.

BACKGROUND SMARCB1-deficient sinonasal carcinoma is a rare neoplasm with inactivation of the SWI/SNF complex, with an aggressive clinical course as most of the lesions present as advanced in pT3/T4 stages with frequent recurrence, and many patients succumb to the disease. Reported initially in 2014, the lesion has male predominance, with an age range of 19 to 89 years and predilection for the ethmoid sinus and nasal cavity. Histopathological findings show a proliferation of small- to medium-sized monomorphic basaloid cells with indistinctive cytoplasmic borders and round variably prominent nuclei with scattered cells that show rhabdoid morphology. Cytoplasmic vacuoles are common. It has similar morphological findings to a wide array of neoplasms in the sinonasal area. CASE REPORT We report a case of SMARCB1-deficient sinonasal carcinoma in a 30-year-old man referred to our hospital with a preliminary diagnosis of sinonasal adenocarcinoma, intestinal type. Computed tomography showed a huge destructive soft tissue mass in the left maxillary sinus, extended to involve the left nasal cavity with extension to the skull base and perineural spread along the foramen rotundum. Histological examination revealed a malignant basaloid neoplasm embedded in a myxoid stroma that showed loss of SMARCB1 stain. The patient was treated with induction chemotherapy using etoposide and cisplatin for disease control. CONCLUSIONS SMARCB1-deficient sinonasal carcinoma is a rare neoplasm with an aggressive clinical course and high-grade behavior despite having uniform cytological features. This poses complex diagnoses, especially in small biopsies. Incorporating morphological findings with ancillary tests is required to identify this high-grade malignancy.

The Role of Positron Emission Tomography for the Management of Sinonasal Malignancies: A Systematic Review.

BACKGROUND: Positron emission tomography (PET) scan is a valuable imaging modality widely used in the management of cancers. Its usage is well defined for most head and neck malignancies. However, there is a lack of consensus regarding the utility of PET scan for sinonasal malignancies. This is highlighted by the latest international consensus statement on endoscopic skull base surgery. OBJECTIVE: This systematic review aims to clarify the role of PET scan in the management of sinonasal malignancies. METHODS: We conducted a comprehensive literature search using PubMed, MEDLINE, EMBASE, Web of Science, CINAHL, and Cochrane databases for research studies of interest. The Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) updated statement was used to guide the review. RESULTS: In total, 1807 articles were assessed for eligibility. Thirty-nine original papers, published between 2004 and 2021, met inclusion criteria. Seven articles focused on the role of PET scan for inverted papilloma, 23 for sinonasal carcinoma, 4 for melanoma, and 3 for lymphoma, and finally, 3 articles focused on the use of specific PET scan tracers for sinonasal malignancies. Qualitative summaries for each potential role of PET scans were provided. In general, included studies were retrospective in nature with low level of evidence. CONCLUSIONS: In general, and across all types of sinonasal malignancies, PET scan yielded positive results regarding detection and initial staging. It was also considered as the modality of choice for detection of distant metastases, except in the case of sinonasal lymphoma. PET scan's main limit resides in its inability to detect lesions in or close to the metabolic activity of the brain.

Treatment and Management of a Sinonasal Hemangiopericytoma.

Sinonasal Hemangiopericytoma (HPC) is a rare vascular tumor originated from Zimmermann's pericyte with not well valuable course. The diagnosis needs a careful ENT endoscopic examination, radiological study and histopathological analysis with immunohistochemistry for confirmation of diagnosis. We describe a case of a male patient, 67 years old, with history of repeated monolateral right epistaxis. Endoscopic and radiological examination showed an ethmoid-sphenoidal lesion which occupied the entire nasal fossa extending towards the choana, with blood supply coming from the posterior ethmoidal artery. The patient performed an extemporaneous biopsy with subsequent en-bloc removal in operating room, without prior embolization, with the Centripetal Endoscopic Sinus Surgery technique (CESS). The histopathologic analysis led to the diagnosis of sinus HPC. The patient performed close endoscopic follow-ups every 2 months, without performing radio or chemotherapy, and with no evidence of recurrence after 3 years of follow-up. Analyzing the recent literature, a more indolent course with low recurrence rates after total endoscopic surgery remotion was described. Preoperative embolization can be useful in determinate cases, but cause to different complications, it should not be ordinarily performed.

Differentiating sinonasal malignant melanoma from squamous cell carcinoma using DWI combined with conventional MRI.

PURPOSE: This study aimed to investigate the feasibility of diffusion-weighted imaging (DWI) in combination with conventional MRI features to differentiate sinonasal malignant melanoma (SNMM) from sinonasal squamous cell carcinoma (SNSCC). METHODS: A total of 37 patients with SNMM and 44 patients with SNSCC were retrospectively reviewed. Conventional MRI features and apparent diffusion coefficients (ADCs) were evaluated independently by two experienced head and neck radiologists. ADCs were obtained from two different regions of interest (ROIs) including maximum slice (MS) and small solid sample (SSS). Multivariate logistic regression analysis was performed to identify significant MR imaging features in discriminating between SNMM and SNSCC. Receiver operating characteristic (ROC) curves were used to assess the diagnostic performance. RESULTS: SNMMs were more frequently located in the nasal cavity, with well-defined border, T1 Septate Pattern (T1-SP) and heterogeneous T1 hyperintensity, whereas SNSCCs were more frequently located in the paranasal sinus, with homogenous T1 isointensity, ill-defined border, reticular or linear T2 hyperintensity, and pterygopalatine fossa or orbital involvement (all p < 0.05). The mean ADCs of SNMM (MS ADC, 0.85 × 10-3mm2/s; SSS ADC, 0.69 × 10-3mm2/s) were significantly lower than those of SNSCC (MS ADC, 1.05 × 10-3mm2/s; SSS ADC, 0.82 × 10-3mm2/s) (p < 0.05). With a combination of location, T1 signal intensity, reticular or linear T2 hyperintensity, and a cut-off MS ADC of 0.87 × 10-3mm2/s, the sensitivity, specificity, and AUC were 97.3%, 68.2%, and 0.89, respectively. CONCLUSION: DWI combined with conventional MRI can effectively improve the diagnostic performance in differentiating SNMM from SNSCC.

Osteoma of the ethmoid sinus in a pediatric patient - a case report.

BACKGROUND: Osteomas of the paranasal sinuses occur rarely in the pediatric population, we find only a few reference of symptomatic osteomas in the literature. Opinions on the indication for surgical treatment are controversial. CASE: The authors present a case of symptomatic osteoma of the right ethoimoidal sinus in a 12-year-old boy, who was treated surgically, with endoscopic endonasal approach. The symptomatology, diagnosis and therapy of these tumors in the pediatric patient are discussed. CONCLUSION: Osteomas of the paranasal sinuses are slow-growing benign lesions. Symptomatic osteomas can grow expansively and cause serious complications. The treatment of osteoma is surgical and the endoscopic approach offers the possibility of removal with cosmetic benefits.

Preoperative Prediction of Sinonasal Inverted Papilloma-associated Squamous Cell Carcinoma (IP-SCC).

INTRODUCTION: Sinonasal inverted papillomas (IP) can undergo transformation into IP-squamous cell carcinomas (IP-SCC). More aggressive treatment plan should be established when IP-SCC is suspected. Nevertheless, inaccuracy of the preoperative punch biopsy results to detect IP-SCC from IP raises the need for an additional strategy. The present study aimed to investigate significant clinicoradiological remarks associated with IP-SCC than IP. MATERIAL AND METHODS: Postoperative surgical specimens obtained from patients with confirmed IP or IP-SCC at a single tertiary medical center from 1997 to 2018 were retrospectively evaluated. Patients' demographic and clinical characteristics, preoperative in-office punch biopsy results, and preoperative computed tomography (CT) or magnetic resonance images were reviewed. Univariate and multivariate analyses were performed to assess the odds ratio (OR) associated with IP-SCC. The area under the curve (AUC) in the receiver Operating Characteristic (ROC) curve was calculated in the prediction model to discriminate IP-SCC from IP. RESULTS: The study included 44 IP-SCC and 301 patients with IP. The diagnostic sensitivity of in-office punch biopsy to detect IP-SCC was 70.7%. Multivariate analysis showed that factors significantly associated with IP-SCC included tobacco smoking >10PY (adjusted-OR [aOR]: 4.1), epistaxis (aOR: 3.4), facial pain (aOR: 4.2), bony destruction (aOR: 37.6), bony remodeling (aOR: 36.3), and invasion of adjacent structures (aOR: 31.6) (all p < 0.05). Combining all significantly related clinicoradiological features, the ability to discriminate IP-SCC from IP reached an AUC of 0.974. CONCLUSION: IP patients with a history of tobacco smoking, facial pain, epistaxis, and bony destruction, remodeling, or invasion of an adjacent structure on preoperative images may be at higher risk for IP-SCC. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:2502-2510, 2023.