[Analysis of imaging features of rare tumors in nasal cavity and paranasal sinus].

Objective:To explore the imaging features of rare tumors of nasal cavity and sinuses, and to improve the understanding of these diseases, thereby aiding clinical diagnosis and treatment. Methods:The CT and MRI findings of 79 cases of rare neoplasm of nasal cavity and sinuses confirmed by pathology were retrospectively analyzed, and the imaging features were summarized. Results:Among the 79 cases, there were 16 cases of neuroendocrine carcinoma, most showing expansive and infiltrative bone destruction without hyperosteogeny and sclerosis. The sphenoid sinus exhibited a "pigeon" shape. In 28 cases of malignant melanoma, MRI signals were diverse, typical signals were rare, but mixed signals were more common. In 12 cases of rhabdomyosarcoma, MRI enhancement mostly showed "grape-like" enhancement and partial ring enhancement; There were 10 cases of olfactory neuroblastoma, the lesions were consistent with the distribution area of olfactory mucosa, most of them were lobulated, marginal nodules, and "flower ring" enhancement, and 2 cases grew across intracranial and external, with multiple cystic lesions and surrounding flaky edema bands. In 5 cases of solitary fibrous tumor, Benign tumors had regular shape and uniform density, while malignant tumors had irregular shape and uneven density, The enhancement was obviously uneven and showed a "pattern" change. There were 2 cases of sarcomatoid carcinoma, both with lobed appearance, uneven density, lamellar low-density shadow, and osteolytic bone destruction. In 4 cases of schwannoma, the enhancement showed obvious inhomogeneous enhancement. One case showed cystic necrosis, one case showed calcification, and the surrounding structure was compressed without damage. There was 1 case of neurofibroma, with many cystic components, low signal separation and compartmentalized enhancement. One case of paraganglioma showed moderate enhancement in the arterial phase and progressive enhancement in the venous phase, accompanied by significant swelling bone destruction. Conclusion:Rare tumors of nasal cavity and paranasal sinuses have distinctive imaging features. CT and MRI can effectively show the extent of the lesions and the degree of infiltration into adjacent tissues and organs, which is helpful for early clinical diagnosis and staging. However, definitive diagnosis still depends on pathology and immunohistochemistry.

Endoscopy-assisted medial canthus incision for olfactory neuroblastoma: a case report.

Sinonasal malignant tumors are a group of uncommon malignancies that account for less than 1% of all tumors. These tumors often involve the maxillary sinus and nasal cavity, with less cumulative incidence in the ethmoidal sinus, sphenoidal sinus, and frontal sinus. The lack of consensus on the management of sinonasal malignancies is due to their rarity, diagnostic challenges, and the heterogeneity of treatments. In this paper, we present a case of endoscopic-assisted medial canthus incision combined with radiotherapy in the treatment of sinonasal malignant tumors, with the aim of providing valuable insights to clinicians on the management of these tumors.

A Case of Olfactory Neuroblastoma Developing Bilateral Retropharyngeal Lymph Node Metastasis 14-years After Skull Base Surgery.

Olfactory neuroblastoma (ONB) is an uncommon malignant tumor and is usually treated by a multidisciplinary approach includes surgery, radiotherapy, and chemotherapy. A 62 years-old male had a tumor in the nasal cavity and diagnosed as ONB with Kadish A stage. Anterior skull base surgery was performed as radical treatment. Since the surgical margin was negative, no postoperative radiotherapy was administered. 14 years after the surgery, bilateral otitis media with effusion (OME) was occurred, we found the recurrence tumor at bilateral retropharyngeal lymph node (RPLN) which surrounded the internal carotid arteries. Since these were unresectable, we planned chemoradiotherapy which was 70Gy of intensity modulated radiotherapy combined with two courses of carboplatin and etoposide. The tumor volume was reduced and bilateral OME were improved. He has been alive for 3 years after salvage treatment. Although ONB has a relatively good prognosis, it is known to often cause cervical lymph node metastasis. Grades III and IV of Hyams classification are considered high risk. This case, initial tumor was limited in the nasal cavity and its clinical classification was early stage, but Hyams classification was grade III. In reference to this case, considering that RPLN metastasis are difficult to radically resect at the salvage surgery, including this area in postoperative radiotherapy was considered an option.

Augmentation of tumor expression of HLA-DR, CXCL9, and CXCL10 may improve olfactory neuroblastoma immunotherapeutic responses.

BACKGROUND: Olfactory neuroblastoma is a rare malignancy of the anterior skull base typically treated with surgery and adjuvant radiation. Although outcomes are fair for low-grade disease, patients with high-grade, recurrent, or metastatic disease oftentimes respond poorly to standard treatment methods. We hypothesized that an in-depth evaluation of the olfactory neuroblastoma tumor immune microenvironment would identify mechanisms of immune evasion in high-grade olfactory neuroblastoma as well as rational targetable mechanisms for future translational immunotherapeutic approaches. METHODS: Multispectral immunofluorescence and RNAScope evaluation of the tumor immune microenvironment was performed on forty-seven clinically annotated olfactory neuroblastoma samples. A retrospective chart review was performed and clinical correlations assessed. RESULTS: A significant T cell infiltration was noted in olfactory neuroblastoma samples with a stromal predilection, presence of myeloid-derived suppressor cells, and sparse natural killer cells. A striking decrease was observed in MHC-I expression in high-grade olfactory neuroblastoma compared to low-grade disease, representing a mechanism of immune evasion in high-grade disease. Mechanistically, the immune effector stromal predilection appears driven by low tumor cell MHC class II (HLA-DR), CXCL9, and CXCL10 expression as those tumors with increased tumor cell expression of each of these mediators correlated with significant increases in T cell infiltration. CONCLUSION: These data suggest that immunotherapeutic strategies that augment tumor cell expression of MHC class II, CXCL9, and CXCL10 may improve parenchymal trafficking of immune effector cells in olfactory neuroblastoma and augment immunotherapeutic responses.

Olfactory neuroblastoma mimics molecular heterogeneity and lineage trajectories of small-cell lung cancer.

The olfactory epithelium undergoes neuronal regeneration from basal stem cells and is susceptible to olfactory neuroblastoma (ONB), a rare tumor of unclear origins. Employing alterations in Rb1/Trp53/Myc (RPM), we establish a genetically engineered mouse model of high-grade metastatic ONB exhibiting a NEUROD1+ immature neuronal phenotype. We demonstrate that globose basal cells (GBCs) are a permissive cell of origin for ONB and that ONBs exhibit cell fate heterogeneity that mimics normal GBC developmental trajectories. ASCL1 loss in RPM ONB leads to emergence of non-neuronal histopathologies, including a POU2F3+ microvillar-like state. Similar to small-cell lung cancer (SCLC), mouse and human ONBs exhibit mutually exclusive NEUROD1 and POU2F3-like states, an immune-cold tumor microenvironment, intratumoral cell fate heterogeneity comprising neuronal and non-neuronal lineages, and cell fate plasticity-evidenced by barcode-based lineage tracing and single-cell transcriptomics. Collectively, our findings highlight conserved similarities between ONB and neuroendocrine tumors with significant implications for ONB classification and treatment.

Deep learning model for differentiating nasal cavity masses based on nasal endoscopy images.

BACKGROUND: Nasal polyps and inverted papillomas often look similar. Clinically, it is difficult to distinguish the masses by endoscopic examination. Therefore, in this study, we aimed to develop a deep learning algorithm for computer-aided diagnosis of nasal endoscopic images, which may provide a more accurate clinical diagnosis before pathologic confirmation of the nasal masses. METHODS: By performing deep learning of nasal endoscope images, we evaluated our computer-aided diagnosis system's assessment ability for nasal polyps and inverted papilloma and the feasibility of their clinical application. We used curriculum learning pre-trained with patches of nasal endoscopic images and full-sized images. The proposed model's performance for classifying nasal polyps, inverted papilloma, and normal tissue was analyzed using five-fold cross-validation. RESULTS: The normal scores for our best-performing network were 0.9520 for recall, 0.7900 for precision, 0.8648 for F1-score, 0.97 for the area under the curve, and 0.8273 for accuracy. For nasal polyps, the best performance was 0.8162, 0.8496, 0.8409, 0.89, and 0.8273, respectively, for recall, precision, F1-score, area under the curve, and accuracy. Finally, for inverted papilloma, the best performance was obtained for recall, precision, F1-score, area under the curve, and accuracy values of 0.5172, 0.8125, 0.6122, 0.83, and 0.8273, respectively. CONCLUSION: Although there were some misclassifications, the results of gradient-weighted class activation mapping were generally consistent with the areas under the curve determined by otolaryngologists. These results suggest that the convolutional neural network is highly reliable in resolving lesion locations in nasal endoscopic images.

The Two-Stage Folded Paramedian Forehead Flap Without Cartilage Grafts for Full Thickness Distal Nasal Defects: A Review of 35 Patients.

BACKGROUND: Full thickness defects of the ala, soft triangle, and nasal tip involving the nasal lining have traditionally been repaired with the three-stage folded paramedian forehead flap (FPFF), with a cartilage graft for support. For similar defects, the authors utilize the two-stage FPFF without cartilaginous support which provides reproducible functional and aesthetic results.  Objective: To describe the authors’ experience with the two-stage FPFF, including outcomes, complications, and design modifications to enhance functional and aesthetic success.  Methods: An IRB-approved retrospective database review of FPFF was performed at two sites. Using postoperative photographs, outcomes were assessed by blinded non-investigator dermatologist raters using a modified observer scar assessment scale. RESULTS: Thirty-five patients were reconstructed using the two-stage FPFF without cartilage grafts. Subjective assessment of scar vascularity, pigment, relief, and thickness by 3 independent reviewers yielded an overall cosmesis score of 8.4±1.9 (out of 40). CONCLUSION: The two-stage FPFF without cartilage grafts is a reliable, cosmetically elegant repair that can provide optimal functional and aesthetic results for complex unilateral distal nose defects.J Drugs Dermatol. 2024;23(4): doi:10.36849/JDD.7358.

Diagnostic accuracy of clinical visual assessment using endoscopic images for nasal cavity mass lesions.

Objectives: Discrimination of nasal cavity lesions using nasal endoscopy is challenging because of the differences in clinical manifestations and treatment strategies. We aimed to investigate the diagnostic accuracy of clinical visual assessment (CVA) of nasal cavity masses using endoscopic images and determine whether there is a difference according to pathologic class and the examiners' experience. Methods: We collected pathologically confirmed endoscopic images of normal findings, nasal polyp (NP), benign tumor, and malignant tumor (each class contained 100 images) randomly selected. Eighteen otolaryngologists, including six junior residents, six senior residents, and six board-certified rhinologists classified the test set images into four classes of lesions by CVA. Diagnostic performance according to the pathologic class and the examiner's experience level was evaluated based on overall accuracy, F1-score, confusion matrix, and area under the receiver operating characteristic curve (AUC). Results: Diagnostic performance was significantly different according to the pathological class of nasal cavity mass lesions with the overall accuracy reported high in the order of normal, NP, benign tumor, and malignant tumor (0.926 ± 0.100; 0.819 ± 0.135; 0.580 ± 0.112; 0.478 ± 0.187, respectively), F1 score (0.937 ± 0.076; 0.730 ± 0.093; 0.549 ± 0.080; 0.554 ± 0.146, respectively) and AUC value (0.96 ± 0.06; 0.84 ± 0.07; 0.70 ± 0.05; 0.71 ± 0.08, respectively). The expert rhinologist group achieved higher overall accuracy than the resident group (0.756 ± 0.157 vs. 0.680 ± 0.239, p < .05). Conclusion: CVA for nasal cavity mass was highly dependent on the pathologic class and examiner's experience. The overall accuracy was reliably high for normal findings, but low in classifying benign and malignant tumors. Differential diagnosis of lesions solely based on nasal endoscopic evaluation is challenging. Therefore, clinicians should consider further clinical evaluation for suspicious cases.

Advantages of the co2 laser use in the rare condition of nasal mucosa squamous cell carcinoma surgery in dogs-a clinical prospective study.

Nasal mucosa tumors are an uncommon process and very dificult to work on with surgery. Radiotherapy associated or not with chemotherapy is the standard method to treat the disease. However, its access it is in the majority of the case not possible, making the surgery the best choice to try to achieve the patient's control. The anatomy of the region makes the complete surgical resection very difficult to achieve using the common and conventional blade scalpel surgery. The study features the advantages of using a CO2 laser to perform nasal mucosa carcinoma surgery in 6 dogs (N = 6). For the work we used an Aesculigth CO2 surgical laser model -Vetscalpel®, with the settings of 12Watts in a Superpulse mode, and a 0.25-0.4 mm focus to dissect the nasal mucosa, and a 1.5 mm focus for vaporization of the area. All the masses were histopathologically characterized as squamous cells carcinoma. The CO2 surgical laser allow us to work in a bloodless region promoting a more accurate dissection of the nasal mucosa sparing therefore the underlying and adjacent tissues and being less invasive. Also, it was possible to do the vaporization of the entire surgical area interviened. None of the patients presented relapse of clinical signs. Only 2 individuals were alive at the end of the study, presenting a survival rate of 420 and 514 days, which is in the same line of literature results of the treatment with radiotherapy combined with chemotherapy wich shows a median of 474-580 days. The study demonstrates successful outcomes with CO2 laser surgery in treating nasal mucosa SCC in dogs, with patients experiencing improved survival rates compared to traditional treatment methods. This highlights the efficacy and potential of CO2 laser surgery as a valuable tool in managing aggressive nasal tumors in veterinary oncology.

Nasal angioleiomyoma in 2 dogs.

This case report describes 2 dogs, an Appenzeller Mountain dog and an Irish Wolfhound, with angioleiomyoma within the nasal cavity. Endoscopic surgical resection resulted in cure in both dogs. Macroscopically and on diagnostic imaging, tumor masses may appear malignant because of local turbinate destruction. This highlights the importance of histological examination before any recommendations are made to owners because tumors of the nasal cavity may be benign and surgery curative.

Squamous cell carcinoma of the nasal vestibule in the Netherlands: A clinical and epidemiological review of 763 cases (2008-2021).

BACKGROUND: Squamous cell carcinoma of the nasal vestibule (SCCNV) is a rare disease, distinctly different in presentation, treatment, and outcome from squamous cell carcinoma (SCC) of the nasal cavity and paranasal sinuses. However, these are often not analyzed separately. METHODS: The Netherlands Cancer Registry (NCR) and pathology reports from the Dutch Nationwide Pathology Databank (PALGA) were used to identify all newly diagnosed SCCNV cases in the Netherlands between 2008 and 2021. RESULTS: A total of 763 patients were included. The yearly incidence rate displayed a significant downward trend with an annual percentage change (APC) of -3.9%. The 5-year overall survival (OS) and disease-free survival were 69.0% and 77.2%, respectively. The 5-year relative survival was 77.9% and improved slightly over the inclusion period. OS for patients who were staged cT3 appeared to be worse than those staged cT4a, calling the applicability of the TNM-classification into question. CONCLUSION: SCC of the nasal vestibule is rare, with declining incidence rates. Introducing a specific topography code for SCCNV is recommended to enhance registration accuracy. The TNM classification seems poorly applicable to SCCNV, suggesting the need to explore alternative staging methods.

Treatment of unilateral olfactory neuroblastoma: Appropriate extent of surgical resection and potential for olfactory preservation.

Historically, comprehensive surgical resection for olfactory neuroblastoma has included the bilateral olfactory epithelium, cribriform plate, overlying dura, olfactory bulbs and tracts. This results in postoperative anosmia that may significantly impact a patient's quality of life without definitive added benefit in survival. The prevalence of occult intracranial disease is low, especially for Hyams grade I and II tumors. A unilateral approach sparing the contralateral cribriform plate and olfactory system can be considered for select cases of early stage, low-grade tumors when the disease does not cross midline to involve the contralateral olfactory cleft or septal mucosa and when midline dural margins can be cleared with frozen pathology. Approximately half of patients who undergo unilateral resection may have residual olfaction even with adjuvant unilateral radiation. Early data suggest favorable disease-free survival and overall survival for patients who underwent the unilateral approach; however, larger sample studies are needed to confirm comparability to bilateral resections regarding oncologic outcomes.

The impact of multidisciplinary approaches on the outcomes of olfactory neuroblastoma treated with postoperative radiotherapy.

BACKGROUND: We investigated the outcomes of postoperative radiation therapy for olfactory neuroblastoma (ONB) and our cross-departmental collaboration to enhance the effectiveness of cancer treatment. METHODS: We retrospectively evaluated 22 patients with ONB who underwent postoperative radiotherapy after tumor resection. En bloc resection was performed; pathology specimens were prepared in coronal sections; and irradiation fields were determined after discussion with radiation oncologists, head and neck surgeons, and pathologists. RESULTS: The overall survival and local control rates were 95.5% and 100%, respectively, at a median 37-month follow-up. The 3- and 5-year disease-free survival (DFS) rates were 64.4% and 56.3%, respectively. Of the 22 patients, 9 (8 Kadish C and 1 Kadish B) had disease recurrence. Of the nine patients, five had positive margins and two had closed margins; cervical lymph node recurrence occurred in six, and distant metastasis with or without cervical lymph node recurrence occurred in three. DFS analysis of risk factors showed no statistically significant differences, but positive margins were a significant recurrence factor in multivariate analysis. CONCLUSIONS: The local control rate of ONB treated with postoperative radiation therapy was 100%. This may be attributed to cross-departmental cooperation between head and neck surgeons, pathologists, and radiation oncologists, which resulted in accurate matching of CT images for treatment planning with the location of the tumor and positive margins. Longer follow-up periods are required to evaluate the effectiveness of our strategy.

Sinonasal teratocarcinosarcoma in an adolescent male: A case report of an unusual neoplasm.

ABSTRACT: Sinonasal teratocarcinosarcoma (SNTCS) is an extremely rare and aggressive malignant tumor arising in the sinonasal tract, having a combined clinicopathological feature of teratoma and carcinosarcoma. It shows a male predominance and affects adults with an age range of 18-79 years and a mean age of 60 years. Here, we report a case of SNTCS in a 14-year-old male patient who presented with swelling over the upper right alveolus and pain in the right jaw for 2 months. The tumor was completely removed by right total maxillectomy with orbital mess reconstruction, and postoperative radiotherapy with chemotherapy was given. The follow-up of the patient for 2 years has shown evidence of recurrence and is now on palliative care.

A case report on a nasal and oral cavity involving large solitary fibrous tumor and comprehensive review of case literature.

Solitary fibrous tumor (SFT) represents an uncommon spindle cell sarcoma predominantly situated within soft tissue, with a notably infrequent occurrence in the nasal cavity and paranasal sinuses. In this report, we present a case involving a middle-aged male with a sizable solitary fibrous tumor affecting both the nasal and oral cavities.