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Zhonghua Bing Li Xue Za Zhi = Chinese... Nov 2023To investigate the clinicopathological features and differential diagnosis of olfactory carcinoma (OC). Twenty-one cases of sinonasal tumors, including those initially...
To investigate the clinicopathological features and differential diagnosis of olfactory carcinoma (OC). Twenty-one cases of sinonasal tumors, including those initially diagnosed as olfactory neuroblastoma (ONB) and those with uncertain diagnosis, were collected from the Department of Pathology, the First Affiliated Hospital of University of Science and Technology of China (Anhui Provincial Hospital) from January 2016 to August 2022, among which 3 cases were reclassified as OC. The clinicopathological features were investigated, and the remaining 18 cases were used as control. Of the three OC patients, 2 were male and 1 was female, with an average age of 57 years ranging from 35 to 74 years. Microscopically, the tumor cells were arranged in solid, nested or lobulated patterns with occasional palisading around the solid nests. The stroma was highly vascular with focal neurofibrillary areas. There were prominent rosettes or pseudorosettes formation. The tumor cells were mainly ovoid to spindly with scant to moderate amount of cytoplasm, one or several small nucleoli, and fine chromatin content. Brisk mitotic figures were seen. In all 3 cases of OC, there were scanty atypical glands and some were ciliated. Immunohistochemically, at least one epithelial marker and neuroendocrine marker were diffusely expressed in the tumor. Some of the tumor cells were positive for p40 and p63, and the sustentacular cells showed the expression of S-100 protein. All cases tested were negative for NUT, CD99 and desmin, with intact expression of SMARCA4 (BRG1) and SMARCB1 (INI-1). Ki-67 proliferation index varied from 20% to 80%. Follow-up after 16-18 months showed no mortality with tumor recurrence from 1 patient after 16 months. OC is a rare sinonasal tumor with neuroepithelial differentiation, its histomorphology is diverse, and the combination of immunohistochemical markers is essential for appropriate diagnosis.
Topics: Humans; Male; Female; Middle Aged; Paranasal Sinus Neoplasms; Biomarkers, Tumor; Carcinoma; Diagnosis, Differential; S100 Proteins; DNA Helicases; Nuclear Proteins; Transcription Factors
PubMed: 37899320
DOI: 10.3760/cma.j.cn112151-20230208-00101 -
Zhonghua Bing Li Xue Za Zhi = Chinese... Nov 2023
Topics: Humans; Consensus; East Asian People; Esthesioneuroblastoma, Olfactory; Nasal Cavity; Nose Neoplasms
PubMed: 37899315
DOI: 10.3760/cma.j.cn112151-20230411-00249 -
Journal of Personalized Medicine Oct 2023Poorly differentiated sinonasal carcinomas (PDCs) are tumors that have a poor prognosis despite advances in classical treatment. Predictive and prognostic markers and...
Poorly differentiated sinonasal carcinomas (PDCs) are tumors that have a poor prognosis despite advances in classical treatment. Predictive and prognostic markers and new personalized treatments could improve the oncological outcomes of patients. In this study, we analyzed SOX2 and βIII-tubulin as biomarkers that could have prognostic and therapeutic impacts on these tumors. The cohort included 57 cases of PDCs: 36 sinonasal undifferentiated carcinoma (SNUC) cases, 13 olfactory neuroblastoma (ONB) cases, and 8 sinonasal neuroendocrine carcinoma (SNEC) cases. Clinical follow-up data were available for 26 of these cases. Sox2 expression was detected using immunohistochemistry in 6 (75%) SNEC cases, 19 (53%) SNUC cases, and 6 (46%) ONB cases. The absence of Sox2 staining correlated with a higher rate of recurrence ( = 0.015), especially distant recurrence. The majority of cases showed βIII-tubulin expression, with strong positivity in 85%, 75%, and 64% of SNEC, ONB, and SNUC cases, respectively. Tumors with stronger βIII-tubulin expression demonstrated longer disease-free survival than those with no expression or low expression ( = 0.049). Sox2 and βIII-tubulin expression is common in poorly differentiated sinonasal tumors and has prognostic and therapeutic utility.
PubMed: 37888115
DOI: 10.3390/jpm13101504 -
The Lancet. Oncology Nov 2023We previously reported rates of pathological complete responses (51% [95% CI 39-62] per independent central review, the primary endpoint) and major pathological...
BACKGROUND
We previously reported rates of pathological complete responses (51% [95% CI 39-62] per independent central review, the primary endpoint) and major pathological responses (13% per independent central review, a secondary endpoint) to neoadjuvant cemiplimab (an anti-PD-1 inhibitor) among 79 patients with locoregionally advanced, resectable cutaneous squamous cell carcinoma. Here, we present follow-up data, including event-free, disease-free, and overall survival.
METHODS
This single-arm, multicentre, phase 2 study included patients aged 18 years or older with resectable stage II-IV (M0) cutaneous squamous cell carcinoma and Eastern Cooperative Oncology Group performance status of 0 or 1. Patients received up to four planned doses of neoadjuvant cemiplimab 350 mg intravenously every 3 weeks followed by curative-intent surgery. After surgery, per investigator discretion, patients received either adjuvant cemiplimab for up to 48 weeks, radiotherapy, or observation alone. Secondary endpoints included in this follow-up analysis are event-free survival, disease-free survival, and overall survival, all summarised using the Kaplan-Meier method. Activity and safety endpoints were analysed for all enrolled patients who received at least one dose of neoadjuvant cemiplimab. In this report, safety data are reported for all patients who received at least one dose of adjuvant cemiplimab. This trial is registered with ClinicalTrials.gov, NCT04154943, has completed enrolment and follow-up is ongoing.
FINDINGS
Between March 20, 2020, and July 8, 2021, 79 patients were enrolled. Median age was 73 years (IQR 66-81), 67 (85%) patients were male, 12 (15%) were female, 69 (87%) were White, one was Asian (1%), one was other race (1%), and race was not reported for eight (10%). As of data cutoff (Dec 1, 2022), median follow-up was 18·7 months (IQR 15·6-22·1) for all 79 patients. Among 70 patients who had surgery, 65 (93%) had post-surgical management data: 32 (49%) of 65 were observed postoperatively, 16 (25%) received adjuvant cemiplimab, and 17 (26%) received adjuvant radiotherapy. 11 (14%) of 79 patients had event-free survival events, with an estimated 12-month event-free survival of 89% (95% CI 79-94) for all patients. None of 40 patients who had a pathological complete response and one (10%) of ten patients with major pathological response had recurrence. Six (9%) of 70 patients who completed surgery had a disease-free survival event, with an estimated 12-month disease-free survival of 92% (95% CI 82-97). Nine (11%) of 79 patients died, with an estimated 12-month overall survival for all patients of 92% (95% CI 83-96). Four (25%) of 16 patients who received adjuvant cemiplimab treatment had grade 3 adverse events, including one (6%) who had increased blood potassium, one (6%) who had traumatic limb amputation, and two who had serious adverse events (one [6%] cardiomyopathy and one [6%] hypophysitis). There were no grade 4 adverse events or treatment-related deaths.
INTERPRETATION
For patients with resectable stage II-IV cutaneous squamous cell carcinoma, neoadjuvant cemiplimab followed by surgery might be a potential treatment option, addressing a substantial unmet need.
FUNDING
Regeneron Pharmaceuticals and Sanofi.
Topics: Humans; Male; Female; Aged; Carcinoma, Squamous Cell; Neoadjuvant Therapy; Follow-Up Studies; Skin Neoplasms; Neoplasm Staging; Antineoplastic Combined Chemotherapy Protocols
PubMed: 37875144
DOI: 10.1016/S1470-2045(23)00459-X -
BMJ Case Reports Oct 2023
Topics: Humans; Esthesioneuroblastoma, Olfactory; Nose Neoplasms; Nasal Cavity; Neurilemmoma
PubMed: 37852667
DOI: 10.1136/bcr-2023-257847 -
Cureus Sep 2023Background The aim of this study was to determine the histological diagnosis of unilateral nasal polyps and to determine the prevalence of neoplastic pathologies. This...
Background The aim of this study was to determine the histological diagnosis of unilateral nasal polyps and to determine the prevalence of neoplastic pathologies. This study also assessed difference in pathologies whether patients presented symptomatically or were asymptomatic (if they had a mass found incidentally for unrelated throat symptoms). Method This was a 10-year retrospective study of patients undergoing unilateral nasal mass surgery between 2004 and 2014 at a UK district general hospital. We recorded patient demographics, laterality, histology, symptoms, clinical suspicion, and imaging findings. Results 123 patients were included who underwent unilateral surgery between 2004 and 2014 (male n=83, female n=40; mean age 56 years ± 19.5). The majority were of inflammatory origin (n=92; 74.8%). The most common benign neoplastic cause was inverted papilloma (n=19; 15.4%). A number of malignant neoplastic causes were also found, including: melanoma (n=3; 2.44%), olfactory neuroblastoma (n=2; 1.63%), and other non-inflammatory masses (n=7; 5.69%). 15 of these masses were found incidentally, with 14 being inflammatory, and one an olfactory neuroblastoma; therefore, 6.67% of our incidental unilateral nasal masses were found to be of neoplastic pathology. Conclusion This study's findings support the continued practice of routine biopsy of unilateral nasal masses for histological diagnosis, irrespective of whether they are symptomatic or found incidentally. The accuracy of both clinical suspicion and radiological suspicion on CT scans is not adequate to alter this practice.
PubMed: 37790007
DOI: 10.7759/cureus.44526 -
Ear, Nose, & Throat Journal Sep 2023Ectopic olfactory neuroblastoma (ONB) is a rare neuroendocrine sinonasal malignancy which arises from sinonasal regions where olfactory neuroepithelium does not exist....
Ectopic olfactory neuroblastoma (ONB) is a rare neuroendocrine sinonasal malignancy which arises from sinonasal regions where olfactory neuroepithelium does not exist. Presentation of syndrome of inappropriate antidiuretic hormone release (SIADH) in patients with ectopic ONB is extremely rare. We report a case of a 22-year-old patient with ectopic ONB and paraneoplastic SIADH that was managed at our center. The ONB was arising from the left lamina papyracea and extending into the maxillary and ethmoid sinuses and filling the nasal cavity. Correction of sodium levels occurred within 24 hours of endoscopic resection via a combined trans-nasal transorbital approach. To avoid negative sequela secondary to rapid sodium correction, the patient was managed by desmopressin to obtain gradual sodium correction, which was achieved on the fourth postoperative day. Ectopic presentation of ONB with paraneoplastic SIADH is extremely rare with only 7 cases reported in the literature to date. Management via surgical resection and/or radiotherapy for these patients requires the managing clinician to be vigilant of serum sodium levels. A multidisciplinary approach is essential for optimal outcomes.
PubMed: 37776288
DOI: 10.1177/01455613231199696 -
Exploring the Impact of Using Patient-Specific 3D Prints during Consent for Skull Base Neurosurgery.Journal of Neurological Surgery. Part... Oct 2023Informed consent is fundamental to good practice. We hypothesized that a personalized three-dimensional (3D)-printed model of skull base pathology would enhance...
Informed consent is fundamental to good practice. We hypothesized that a personalized three-dimensional (3D)-printed model of skull base pathology would enhance informed consent and reduce patient anxiety. Digital images and communication in medicine (DICOM) files were 3D printed. After a standard pre-surgery consent clinic, patients completed part one of a two-part structured questionnaire. They then interacted with their personalized 3D printed model and completed part two. This explored their perceived involvement in decision-making, anxiety, concerns and also their understanding of lesion location and surgical risks. Descriptive statistics were used to report responses and text classification tools were used to analyze free text responses. In total,14 patients undergoing elective skull base surgery (with pathologies including skull base meningioma, craniopharyngioma, pituitary adenoma, Rathke cleft cyst, and olfactory neuroblastoma) were prospectively identified at a single unit. After 3D model exposure, there was a net trend toward reduced patient-reported anxiety and enhanced patient-perceived involvement in treatment. Thirteen of 14 patients (93%) felt better about their operation and 13/14 patients (93%) thought all patients should have access to personalized 3D models. After exposure, there was a net trend toward improved patient-reported understanding of surgical risks, lesion location, and extent of feeling informed. Thirteen of 14 patients (93%) felt the model helped them understand the surgical anatomy better. Analysis of free text responses to the model found mixed sentiment: 47% positive, 35% neutral, and 18% negative. In the context of skull base neurosurgery, personalized 3D-printed models of skull base pathology can inform the surgical consent process, impacting the levels of patient understanding and anxiety.
PubMed: 37671293
DOI: 10.1055/a-1885-1111 -
International Forum of Allergy &... Feb 2024Sinonasal neoplasms, whether benign and malignant, pose a significant challenge to clinicians and represent a model area for multidisciplinary collaboration in order to... (Review)
Review
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.
Topics: Humans; Quality of Life; Hypersensitivity; Head and Neck Neoplasms; Paranasal Sinus Neoplasms
PubMed: 37658764
DOI: 10.1002/alr.23262 -
Indian Journal of Otolaryngology and... Sep 2023Malignancies in children are different from those found in adults and are a significant cause of childhood mortality.They have varied clinical presentation depending on...
INTRODUCTION
Malignancies in children are different from those found in adults and are a significant cause of childhood mortality.They have varied clinical presentation depending on site and type of disease.It is essential to recognize the early signs and symptoms of malignancies in childhood, especially those involving head and neck region, so as to reduce childhood mortality and morbidity.
MATERIALS
A total of 2384 children were admitted over a period of 7 years. Out of these, 1004 fulfilled the inclusion criteria and were chosen for further evaluation.They were thoroughly evaluated by undertaking a detailed history and clinical examination.Whenever required, additional investigations were performed.After carrying out the necessary investigations, the cases were accordingly managed. Data was evaluated using proper statistical tools.
RESULTS
Out of 1004 cases fulfilling the inclusion criteria, 42 turned out to be malignant, with a male-to-female ratio of 1:1.2. Malignancies in children were more common in the age group of 11-18 years, followed by 1-5 years,6-10 years and 0-1 years,with rates of 59.5%, 21.4%, 16.7% and 2.4% respectively. A wide variety of tumour types were recorded,e.g.,Hodgkin's lymphoma,non-Hodgkin's lymphoma,acute leukemia,papillary carcinoma thyroid, nasopharyngeal carcinoma,Langerhans cell histiocytosis,rhabdomyosarcoma, olfactory neuroblastoma and salivary gland neoplasm.
CONCLUSION
Incidence of head and neck tumors in pediatric age group was found to be 1.76% with lymphoma being the most frequent.Commonest age of presentation was above 10 years. There was an overall female predominance with a male:female ratio of 1:1.2. Awareness of a potential malignancy and careful follow-up of children with suspicious head and neck cancers is mandatory for early diagnosis and prompt treatment.
PubMed: 37636808
DOI: 10.1007/s12070-023-03779-1