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.

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.

Intravascular papillary endothelial hyperplasia of the maxillary sinus extending into the contralateral nasal cavity.

INTRODUCTION: Intravascular papillary endothelial hyperplasia (IPEH) predominantly occurs in the subcutaneous and dermal regions and rarely originates from the sinonasal mucosa. CASE PRESENTATION: We report on the case of a 58-year-old male patient who presented with progressive bilateral nasal obstruction, left-sided epiphora, and intermittent epistaxis. Computed tomography revealed a soft tissue opacity in the left maxillary sinus with intersinusoidal nasal wall demineralization, extending into the surrounding ethmoid cells and the right nasal cavity through a contralateral deviation of the nasal septum. Contrast-enhanced T1-weighted magnetic resonance imaging further confirmed these findings. The IPEH originating from the maxillary sinus extended into the contralateral nasal cavity, and it was successfully removed using an endoscopic endonasal approach, avoiding overly aggressive treatment. CONCLUSION: This case report highlights the diagnostic challenges of IPEH in the sinonasal region and the importance of considering IPEH as a differential diagnosis in patients presenting with nasal obstruction, epiphora, and intermittent epistaxis.

Pathogenic Somatic Mutation of DICER1 and Clinicopathological Features in Nasal Chondromesenchymal Hamartomas: A Series of Nine Cases.

Nasal chondromesenchymal hamartoma (NCMH) is a rare benign polypoid mesenchymal tumor arising in the nasal cavity and/or paranasal sinuses. Recognizing these sporadic, rare lesions is crucial, as surgical complete removal of the mass is the common treatment approach. This retrospective study analyzed the demographics, symptoms, and imaging data of 9 patients diagnosed with NCMH between January 2017 and June 2023, possibly representing the largest single-center adult case cohort to date. Diagnostic techniques included nasal endoscopy, CT/MRI scan, immunohistological studies, and morphologic comparisons. Pathologic specimens were subjected to Sanger sequencing of exons 24 and 25 of DICER1. The average age of 9 cases was 24.4 years, and the oldest was 55 years. Four of the patients were children, ranging from 1 year old to 11 years old, with an average of 4.5 years. Nasal congestion is the most common registered symptom. Endoscopic findings showed that most patients had smooth pink neoplasms or polypoid masses in the nasal meatus. Radiologic scanning revealed soft-tissue density masses that occupied the nasal cavity. Histologically, the characteristic structure of NCMHs is immature cellular cartilage nodules and mature cartilage nodules distributed in a loose mucoid matrix. Five of the 9 patients had somatic DICER1 missense mutations. Four of the patients with DICER1-mutated NCMH exhibited a p.E1813 missense hotspot mutation. We also report a case of a rare p.P1836H missense mutation. The detected DICER1 somatic mutations provide compelling evidence of an association with the DICER1 tumor family. We emphasize the importance of pathologic consultation and the need for pathologists to accumulate experience in NCMH diagnosis to avoid misdiagnosis.

Diagnostic benefits of platelet-to-lymphocyte, neutrophil-to-lymphocyte, and albumin-to-globulin ratios in dogs with nasal cavity diseases.

BACKGROUND: A multimodal approach for diagnostic tests under anesthesia is required to diagnose nasal cavity pathology (NP) reliably in dogs. Blood test results may provide clues to the suspected NP. METHODS: This prospective blinded study assessed 72 dogs with chronic nasal discharge due to NPs, and 10 healthy dogs as the control group (CG). NPs were diagnosed using whole-body computed tomography (CT), upper airway endoscopy, examination of nasal mucosal swabs by bacterial and fungal culture, and histopathological examination of nasal mucosa biopsies. The exclusion criteria were the presence of any additional diseases or corticosteroid pre-treatment. In consideration of these exclusion criteria, 55 dogs entered the study. Dogs were classified into benign (benign tumors, idiopathic rhinitis (IR), and others) and malignant (carcinomas and sarcomas) NP groups. Blood count and blood chemistry tests were performed. The neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and albumin-to-globulin ratio (AGR) were calculated and compared. RESULTS: 25 dogs with malignant NP (13 and 12 with carcinomas and sarcomas, respectively) and 30 dogs with benign NP (seven with benign tumors,13 with IR, and 10 others) were included. In general, in dogs with NP there were only slight abnormalities in complete blood count. However, PLR was significantly higher in dogs with malignant NP (carcinoma and sarcoma) than in those with benign NP and in the CG. Compared with the CG, the NLR was significantly increased in all dogs with NP, and the AGR was mild but significantly lower, except in dogs with sarcomas and benign tumors. CONCLUSIONS: In dogs with nasal disease alone, there are usually no marked abnormalities in blood count. However, while mildly increased NLR and decreased AGR can be observed in almost all NPs, an increased PLR may indicate a malignant NP and can be used as an additional screening tool in dogs with nasal discharge due to nasal cavity pathology.

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.

Nasal spindle cell tumor: A case report and literature review.

BACKGROUND: Spindle cell tumors are rare and can occur in any organ or tissue. Due to their rarity the clinicopathological features and diagnostic protocols have not been adequately studied. However, it has become necessary to develop differential diagnosis of spindle cell tumors. Here, we report a case of a nasal spindle cell tumor diagnosed at our hospital in attempt to contribute to this gap in literature. KEY POINTS FROM THE CASE: A male in his 30s was admitted to our hospital with nasal obstruction that had persisted for several years. Electronic fibrolaryngoscopy revealed a smooth neoplasm within the nasal cavity. MAIN LESSONS TO BE LEARNED FROM THIS CASE REPORT: The results of this case emphasize that spindle cell tumors have large morphological variations, and it is difficult to determine the origin of tumor cells using hematoxylin and eosin staining alone. Therefore, it is necessary to improve the immunohistochemistry and combine it with clinical symptoms to diagnose the disease.

The role of insulinoma-associated protein 1 in predicting the progression and prognosis of human olfactory neuroblastoma in China.

OBJECTIVE: Recent studies have suggested that insulinoma-associated protein 1 (INSM1) is a useful marker for pathological diagnosis of neuroendocrine tumors. In the present study, we investigated the association between INSM1 expression and prognosis in patients with olfactory neuroblastoma (ONB) and assessed the usefulness of INSM1 as a prognostic biomarker in these patients. METHOD: Immunohistochemistry was performed on 109 ONB patients who underwent endoscopic surgery at Beijing Tong Ren Hospital (Beijing, China) between June 2006 and November 2021 Patient age at the time of surgery ranged from 10 months to 72 years (mean age, 43.55 ± 13.47 years). In total, 63 (57.8%) and 46 (42.2%) tumors occurred in male and female patients, respectively. The percentages of grade I-IV cases were 13.8% (15/109), 36.7% (40/109), 29.4% (32/109) and 20.2% (22/109), respectively. RESULTS: The expression rate (moderately/strongly positive) of INSM1 was significantly higher in high-grade (Ⅲ/Ⅳ; 83%; 45/54) than low-grade (Ⅰ/Ⅱ; 27%; 15/55) ONB cases. High expression levels of INSM1 were significantly positively associated with high pathological stage (p < 0.001), local recurrence, and death. Kaplan­Meier analysis revealed that patients with high INSM1 expression had significantly shorter disease­free survival (DFS) and mean survival (75.01 ± 10.71 vs. 158.56 ± 10.32) times, and shorter overall survival (OS). Multivariate Cox regression analysis revealed that INSM1 was an independent prognostic factor for DFS (HR: 4.963, 95%CI [2.11-10.84] p < 0.001) and OS (HR: 4.791, 95%CI [2.117-10.485], p < 0.001) after adjusting for sex, age, and tumor grade. In addition, INSM1 was an independent prognostic factor for DFS in patients treated with surgery (HR: 3.714, 95%CI [1.267-10.889], p = 0.017) and chemotherapy (HR: 5.574, 95%CI [1.584-19.612], p = 0.007). CONCLUSION: INSM1 expression had a positive association with the prognosis of patients with ONB and could serve as a prognostic biomarker in these patients.

Dorsal rhinotomy in a dog with a chondro-osseous respiratory epithelial adenomatoid hamartoma: a case report.

OBJECTIVE: To describe the clinical presentation, novel surgical approach, and outcome of a dog diagnosed with chondro-osseous respiratory epithelial adenomatoid hamartoma (COREAH). ANIMAL: 5-year-old castrated male Yorkshire Terrier. CLINICAL PRESENTATION, PROGRESSION, AND PROCEDURES: The dog was presented with chronic upper respiratory noise, congestion, facial swelling, ocular discharge, and an abscess on the nasal bridge. Two CT scans were performed 4 months apart. The CT scans yielded similar results: cyst-like nasal masses with severely destructive bilateral rhinitis with extensive polyostotic bony lysis. A dorsal rhinotomy with a turbinectomy and debridement of the nasal cavity were performed. A poorly defined but extensive lesion was found occupying the entirety of the left frontal sinus as well as the nasal cavity. TREATMENT AND OUTCOME: Histopathology revealed a mass consistent with COREAH. The dog recovered well from surgery, except for self-limiting subcutaneous emphysema, and 3 weeks postoperatively was reportedly doing well, with mild nasal discharge. Stridor, nasal discharge, and sneezing episodes were reported postoperatively; however, these were improved. At 18 months postoperatively, the dog died from uncontrolled seizures while hospitalized for suspected acute hemorrhagic diarrhea syndrome at a different hospital. CLINICAL RELEVANCE: COREAH should be considered a potential cause of destructive bilateral rhinitis and bony lysis in dogs. Dorsal rhinotomy can be a surgical treatment for dogs with possible COREAH with acceptable outcome, though complete remission of clinical signs may not be achieved. This is the first clinical description of COREAH in a dog.

Characterizing Immune Infiltration in Esthesioneuroblastoma Subtypes Through Gene Expression Deconvolution.

BACKGROUND: Esthesioneuroblastoma (ENB) is a rare cancer deriving from the olfactory mucosa. Among the basal or neural genomic subtypes, the basal subtype is associated with poorer survival, poor differentiation, and higher levels of tumor-infiltrating immune cells (TIICs). The immune microenvironment of these ENB subtypes remains unclear. We used an established machine learning algorithm on ENB transcriptomic profiles. METHODS: The authors characterized 22 immune cell populations using the CIBERSORTx deconvolutional machine learning pipeline on RNA sequencing data from 18 ENB cases. The characterization aimed to elucidate differences in relative proportions and populations of TIICs between basal and neural ENB. RESULTS: No differences in age, Hyams, Dulguerov, IDH2 mutation, or PD-L1 expression were seen between basal and neural subtypes of ENB (P > 0.05). Also, no difference in median overall survival was appreciated (52.0 ± 13.1 months vs. 50.0 ± 43.2 months, P = 0.5). As a cohort, M2 macrophages were the most abundant subpopulation (14%) followed by naïve B cells (13%) and CD4 memory resting T cells (12%). No gross differences in CD20, CD4, or CD8 cells/mm2 were apparent on gross histology (P > 0.05). However, further analysis showed that activated CD4 memory T cells were significantly increased in the basal ENBs, whereas resting dendritic cells were increased in the neural ENB subtype. The TIIC profiles alone could not differentiate between basal and neural ENB, but did suggest immunoprofile differences. CONCLUSIONS: Basal and neural subtypes display distinct TIIC involvement, which may impact their difference in outcome. These findings provide the framework for further investigation in novel immunomodulation strategies for ENB.

The Effect of Rosmarinic Acid on Wound Healing of Nasal Mucosa in the Rats.

BACKGROUND: The nose can be damaged by environmental pollutants and foreign bodies, as well as a result of trauma, infection or surgical interventions. Proper healing of the damaged nasal mucosa is important for health. OBJECTIVE: There is no study in the literature investigating the effects of rosmarinic acid on mucosal healing. The aim of this study was to investigate the effect of rosmarinic acid on nasal mucosal healing. METHODS: 21 male, adult Spraque Dawley albino rats were divided into three groups as the control group, the local treatment group in which rosmarinic acid was applied locally to the nasal mucosa, and the systemic treatment group in which rosmarinic acid was injected intraperitoneally. The wound area was obtained by creating a trauma area by inserting a 10 mm interdental brush through the right nasal nostril into the right nasal cavities of all animals. For the following 15 days, the treatment agent was applied as indicated once a day and on the 15th day the animals were decapitated and tissue samples taken from the nasal mucosa were prepared for histopathological examination. The preparations were examined in terms of cellular hyperplasia, goblet cell hypertrophy and degeneration, leukocyte infiltration, cilia loss and degeneration, edema and vascular dilatation, and they have been classified into four categories as mild (+), moderate (++), severe (+++) and very severe (++++). RESULTS: There was a significant difference between the groups in terms of all parameters evaluated, and there is a decrease in the intensity of the parameters with transition from the control group to the local group and from there to the systemic group. CONCLUSION: Systemic rosmarinic acid administration showed an enhancing effect on the healing of experimentally induced nasal mucosal injury due to its possible anti-inflammatory effect.

A Large Pleomorphic Adenoma in Nasal Cavity Misdiagnosed as Inverted Papilloma.

Pleomorphic adenoma (PA) is a benign tumor characterized by slow-growing mixed tumors in the craniofacial area. It is relatively common in salivary glands; however, PA of the nasal cavity, which arises in the minor salivary glands, is rare. We present the case of a large PA in the nasal cavity of an adult immunocompetent woman with nasal obstruction and intermittent epistaxis. Based on preoperative radiologic examinations, she was misdiagnosed with an inverted papilloma. Endoscopic resection was performed under general anesthesia. Pathologically, the patient was confirmed to have PA, which has great cellularity and few stromal components. No complications or recurrences during the 1-year follow-up period were observed.

Paranasal sinus angiosarcoma with facial paralysis as a novel manifestation: a case report and literature review.

BACKGROUND: Paranasal sinus angiosarcoma is an uncommon malignancy, with only a few reported cases worldwide. Although it exhibits multiple symptoms, facial paralysis has not been previously documented as a noticeable presentation. CASE PRESENTATION: In this case, we report a 40-year-old male who presented with facial numbness and pain for one month, weakness of his facial muscles for 15 days, and recurrent right epistaxis for 1 year. He had a history of nasal inflammatory polyps with chronic sinusitis. Computed tomography and magnetic resonance imaging showed space-occupying lesions in the right nasal cavity and maxillary sinus, with bone destruction occurring in the sinus wall and turbinate. This patient then underwent endoscopic surgery. According to the histopathological and immunohistochemical results, he was eventually diagnosed with paranasal sinus angiosarcoma in April 2021. To date, this patient has not initiated any radiotherapy or chemotherapy and has survived with lymphatic metastasis for at least 3 years. CONCLUSIONS: This manuscript suggests that paranasal sinus angiosarcoma can present with facial paralysis. Moreover, pathological and immunohistochemical tests are still vital for diagnosing paranasal sinus angiosarcoma and differential diagnosis. Additionally, regular follow-up is crucial for patients with paranasal sinus angiosarcoma, enabling monitoring of recurrence, metastasis, and recovery while contributing valuable clinical data to understanding this rare disease and associated research endeavours.

Transnasal Endoscopic Pituitary Surgery-The Role of a CT Scan in Individual Tailoring of Posterior Septum Size Resection.

OBJECTIVE: This study was designed to evaluate the possibility of predicting the minimum size of septal resection for safe tumor extraction in transnasal paraseptal pituitary adenoma resection from preoperative computed tomography scans. METHODS: A retrospective CT scan analysis was performed on 20 patients who underwent endoscopic pituitary surgery at the University Hospital in Ostrava. Virtual insertion of the straight instrument into the sphenoid cavity was simulated using a CT scan. The minimum septal resection size was predicted and compared to various diameters in the nasal cavity. The results were then compared with cadaveric dissections, in which septal resections were performed at 1 cm and 2 cm distances from the anterior sphenoid wall. The association between cadaver dissections and CT scan results was studied. RESULTS: A total of 20 patients who underwent endoscopic transnasal surgery for pituitary adenoma between the years 2020 and 2021 were enrolled in the study. The mean virtual posterior septal size resection needed to reach the medial edge of the ICA with the straight instrument, without infracturing the nasal septum, was 13.2 mm. In cadavers with a 1 cm posterior septal resection, the medial edge of the ICA was reached with the straight instrument. In 2 cm resections, it was possible to reach beyond the lateral edge of the ICA. CONCLUSION: There is no significant correlation between the minimum septal size resection and measured diameters in the nasal cavity. According to our study, a 1 cm resection is sufficient for a non-extended pituitary tumor extraction. More extensive septal resections allow for better maneuverability and overview in the surgical field.

Incidental seromucinous hamartoma of the anterior nasal cavity presenting after episode of vestibular neuritis.

A woman in her 70s presented to primary care clinic complaining of acute onset dizziness for 1 day that was initially diagnosed as vestibular neuritis and treated with steroids. The next day, she presented to the emergency department with worsening symptoms. Imaging revealed no intracranial process; however, non-contrast CT imaging revealed a soft-tissue mass in the posterior ethmoid sinus. The vertigo completely resolved before an otolaryngologist surgically removed the nasal mass, which actually originated from the right cribriform plate and extended to the anterior middle turbinate head. The final pathology was consistent with seromucinous hamartoma.

Nasal chondromesenchymal hamartoma (NCMH): a rare DICER1-associated tumour in an adult male.

An adult male presented to the ENT clinic with a 1-year history of unilateral nasal blockage. He had presented to another institution 5 years previously with the same issue, undergoing resection of what was reported to be a benign inflammatory polyp with osseous metaplasia. Detailed examination revealed a large mass filling the left nasal cavity. Excisional biopsy and secondary specialist review of pathology revealed nasal chondromesenchymal hamartoma (NCMH) with associated DICER1 mutations. NCMH is a rare, benign tumour of the sinonasal tract, presenting more often in the early childhood, with symptoms related to the site and extent of the tumour. As highlighted in this case, complete excision is mandatory for definitive diagnosis and treatment of NCMH, and an awareness of the association with DICER1 mutation, which can predispose individuals to a range of neoplasia, is key to providing appropriate genetic counselling.

Sinonasal schwannoma masquerading as an IgG4-related nasal mass.

An elderly (8th decade) diabetic patient presented with insidious, painless protrusion of the right eye for 1 month, associated with nasal congestion. Past history included healed serous chorioretinopathy in the right eye (>30 years back) and recently diagnosed (1 year prior) autoimmune IgG4-related pancreatitis for which he was on long-term corticosteroids. On nasal endoscopic examination, a well circumscribed mass was found in the right nasal cavity.Keeping in mind the systemic diagnosis, the sinonasal mass was suspected to be a IgG4-related disease. An endoscopic biopsy was performed and revealed a surprise diagnosis of grade 1 nasal schwannoma.

Peptide Receptor Radionuclide Therapy for Recurrent Olfactory Neuroblastoma After Cranioplasty for Surgical Infection: A Case Report.

BACKGROUND: Peputide receptor radionuclide therapy with 177Lu for midgut neuroendocrine metastasis has been clinically approved as a safe treatment. Unresectable metastases of olfactory neuroblastoma have shorter survival due to insufficient effective systemic treatment. CASE REPORT: Herein, we report a patient treated with peputide receptor radionuclide therapy for unresectable recurrent olfactory neusroblastoma following a rare cranial metastasectomy infection. A 50-year-old female patient with olfactory neuroblastoma of Kadish C was initially treated by skull base surgery plus postoperative radiotherapy following chemotherapy. Recurrent disease with neck and intracranial metastases was treated by four salvage surgeries. Surgical site infection following intracranial metastasectomy was treated with debridement and delayed cranioplasty. Peputide receptor radionuclide therapy was performed for unresectable multiple metastases after cranioplasty. Successful therapy using four cycles of peputide receptor radionuclide had neither grade 3 nor grade 4 adverse events. The patient was followed at an outpatient clinic. CONCLUSION: Further case accrual of peputide receptor radionuclide therapy is required to develop a treatment for unresectable olfactory neuroblastoma.

Radiologic-Pathologic correlation of Pindborg tumor of maxilla with extension to maxillary sinus, nasal cavity, and infraorbital margin: A case report.

Calcifying epithelial odontogenic tumor is a benign epithelial odontogenic tumor thought to originate from the stratum intermedium. Clear cell type, Langerhans cell/non-calcified type, and cystic/microcystic are the three recently recognized histological subtypes of CEOT in the 5th edition of the World Health Organization Classification of Head and Neck Tumors. Almost 350-400 cases of CEOT have been reported in literature, accounting for less than 1% of the reported cases of odontogenic tumors. We are reporting a case of Pindborg tumor of the maxilla with extension to the maxillary sinus, nasal cavity, and infraorbital margin, with an emphasis on radiographic and histopathologic presentation.