Jane Y Tong, Charlyn Gomez, Nicholas Randolph, Kevin D Pereira, Amal Isaiah
{"title":"Airway endoscopy findings in children with recurrent croup.","authors":"Jane Y Tong, Charlyn Gomez, Nicholas Randolph, Kevin D Pereira, Amal Isaiah","doi":"10.1159/000547196","DOIUrl":"https://doi.org/10.1159/000547196","url":null,"abstract":"<p><strong>Introduction: </strong>Microlaryngoscopy and bronchoscopy (MLB) is frequently performed to identify subglottic pathology in recurrent croup. However, the reported incidence of positive findings that could alter management or require intervention varies widely. We sought to determine the utility of endoscopic airway evaluation in this setting.</p><p><strong>Methods: </strong>All MLBs performed by three pediatric otolaryngologists at a tertiary care academic children's hospital from January 1, 2019, to December 31, 2023, were retrospectively reviewed.</p><p><strong>Results: </strong>62 MLBs were performed for recurrent croup. Forty-four (71.0%) patients were male. The mean age was 4.3 years (95% confidence interval, 3.7-4.9). Ten (16.1%) patients were born preterm. Despite serving a predominantly Medicaid-insured minority population, 59.7% of the referred children were privately insured and 85.5% were White. Twenty-one (33.9%) had abnormal findings on MLB, though most were relatively minor. Only three (4.8%) patients demonstrated subglottic stenosis, two classified as Cotton-Myer grade I and one as grade II, with the latter undergoing dilation. Preterm birth, younger age, history of intubation, or inpatient otolaryngology consultation were significantly associated with abnormal findings on MLB.</p><p><strong>Conclusion: </strong>MLB performed in children with recurrent croup has a low incidence of subglottic pathology. Those who could benefit from surgical intervention have characteristics that could identify them preoperatively. Healthcare disparities may significantly influence patterns of referral for MLBs. These results could help the discussion around procedure appropriateness and shared decision-making with caregivers.</p>","PeriodicalId":520736,"journal":{"name":"ORL; journal for oto-rhino-laryngology and its related specialties","volume":" ","pages":"1-10"},"PeriodicalIF":0.0,"publicationDate":"2025-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144556557","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Younghac Kim, Hyojun Kim, Nayeon Choi, Eun-Hye Kim, Junhun Cho, Han-Sin Jeong
{"title":"Surgery alone without adjuvant radiation therapy for high or intermediate-grade salivary gland cancer.","authors":"Younghac Kim, Hyojun Kim, Nayeon Choi, Eun-Hye Kim, Junhun Cho, Han-Sin Jeong","doi":"10.1159/000547198","DOIUrl":"https://doi.org/10.1159/000547198","url":null,"abstract":"<p><strong>Introduction: </strong>Postoperative radiation therapy (RT) is recommended for completely resected T1-2 salivary gland cancer (SGC), if the tumor pathology is adenoid cystic carcinoma, intermediate, or high-grade tumors. In this case series, we reviewed cases of intermediate or high-grade SGC, managed with curative surgery alone without adjuvant RT, and compared those outcomes with pathology- and tumor stage-matched SGC, treated with surgery and postoperative RT.</p><p><strong>Methods: </strong>We retrieved cases of intermediate or high-grade tumors arising from the major salivary glands treated with surgery alone without adjuvant RT (N = 29) from the institutional SGC database. The main reasons for no adjuvant RT and treatment outcomes were reviewed in detail. In addition, we established the tumor pathology (grade)- and tumor stage (T1-2N0M0)-matched cases treated with surgery and postoperative RT as comparison groups (N = 59), to investigate relative treatment outcomes.</p><p><strong>Results: </strong>The overall treatment outcome was 82.7% disease-specific survival (DSS) at a median follow-up of 41 months, with a 10.3% incidence of systemic metastasis in intermediate or high-grade SGCs treated with surgery alone. The primary reasons for not receiving adjuvant RT were no recommendation by the surgeon (37.9%) and patient refusal of RT (27.6%). Comparisons of the surgery alone and surgery with postoperative RT in tumor pathology (grade)- and tumor stage-matched groups revealed similar treatment outcomes; 100% DSS at 35-50 months in the surgery alone group and 90-100% DSS at 37-60 months in the surgery and postoperative RT group. Meanwhile, patients with advanced SGCs (T3-4 or N+) treated with surgery alone had a DSS of 50.0% at a median follow-up of 63 months, and systemic metastasis was the main cause of treatment failure (37.5%).</p><p><strong>Conclusion: </strong>Our findings suggest that surgery alone may yield favorable outcomes for a specific subset of early-stage, intermediate- to high-grade salivary gland cancers (SGC) without any adverse pathological features. Further studies are warranted to validate this approach.</p>","PeriodicalId":520736,"journal":{"name":"ORL; journal for oto-rhino-laryngology and its related specialties","volume":" ","pages":"1-15"},"PeriodicalIF":0.0,"publicationDate":"2025-06-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144532863","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nadhirah Mohd Shakri, Jason Yun Bing Lo, Marina Mat Baki, Zahirrudin Zakaria, Nik Fariza Husna Nik Hassan, Syarifah Nafisah Syed Hamzah, Bao Ling Wong, Puvanaeswaran Dakshinamurthi, Mawaddah Azman
{"title":"Trends in Etiology and Outcomes of Acquired Unilateral Vocal Fold Paralysis: A Multicenter Retrospective Study in Peninsular Malaysia.","authors":"Nadhirah Mohd Shakri, Jason Yun Bing Lo, Marina Mat Baki, Zahirrudin Zakaria, Nik Fariza Husna Nik Hassan, Syarifah Nafisah Syed Hamzah, Bao Ling Wong, Puvanaeswaran Dakshinamurthi, Mawaddah Azman","doi":"10.1159/000546457","DOIUrl":"https://doi.org/10.1159/000546457","url":null,"abstract":"<p><strong>Introduction: </strong>Unilateral vocal fold paralysis (UVFP) results from either mechanical or neurological causes, significantly impacting voice, breathing, and swallowing functions. Thyroid and parathyroid surgeries are commonly implicated as surgical causes; however, non-surgical etiologies remain diverse. Some studies have suggested a change in the trend of UVFP etiologies over time, likely due to advancements in surgical techniques, improved diagnostics, and evolving healthcare practices. This study examines trends in etiology and outcomes of UVFP with early interventions in a multicenter setting in Malaysia over two decades.</p><p><strong>Methods: </strong>A retrospective, multicenter review of UVFP cases was conducted over two periods: 2004-2012 and 2013-2021. Data on patient demographics, UVFP etiology, laterality, and intervention outcomes were analyzed. Interventions included voice therapy alone or in combination with early surgical interventions. Statistical analysis was performed to compare trends and outcomes across periods.</p><p><strong>Results: </strong>Thyroid and parathyroid surgeries remained the predominant surgical cause of UVFP. Iatrogenic UVFP was more commonly right-sided, while non-surgical cases predominantly involved the left side. No significant differences were observed in the distribution of surgical and non-surgical etiologies between the two study periods. Early interventions demonstrated significantly better voice outcomes compared to voice therapy alone (p < 0.05).</p><p><strong>Conclusion: </strong>This is the first long-term, multicenter study in Malaysia addressing UVFP trends and outcomes. Early surgical interventions provide superior outcomes compared to conservative approaches, highlighting the need for timely diagnosis and management to optimize patient care.</p>","PeriodicalId":520736,"journal":{"name":"ORL; journal for oto-rhino-laryngology and its related specialties","volume":" ","pages":"1-17"},"PeriodicalIF":0.0,"publicationDate":"2025-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144487745","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Daria Rometsch, Frank Simon, Philipp Göttker, Felix Simon Heitkötter, Armands Riders, Claudia Rudack, Maximilian Oberste
{"title":"Prognostic Role of PD-L1 and IDO-1 Expression in Sinonasal Carcinoma.","authors":"Daria Rometsch, Frank Simon, Philipp Göttker, Felix Simon Heitkötter, Armands Riders, Claudia Rudack, Maximilian Oberste","doi":"10.1159/000546118","DOIUrl":"10.1159/000546118","url":null,"abstract":"<p><strong>Introduction: </strong>Sinonasal carcinomas (SNC) are rare, aggressive tumours with complex immunological aspects. Still, prognostic markers are missing. The study aimed to investigate the relationship between immune checkpoints - specifically programmed death-ligand 1 (PD-L1) and Indolamin-2,3-dioxygenase 1 (IDO-1) - and clinical parameters in SNC.</p><p><strong>Methods: </strong>In this monocentric study immunohistochemical analysis of the checkpoints PD-L1 and IDO-1 was carried out retrospectively in 85 SNC patients from 2004 to 2018. Tumour proportion score (TPS), combined positive score (CPS) and combined IDO-1 score classified the PD-L1 and IDO-1 expression. The study was conducted in a specialized tertiary care centre, indicating comprehensive multidisciplinary care with surgical, radiotherapeutic, and systemic treatment intervention.</p><p><strong>Results: </strong>Over a median observation period of 41 months, 52 squamous cell carcinomas (SCC, 61%) and 33 adenocarcinomas (AC, 39%) were included. A total of 31 patients (37%) were classified in UICC IV, 89.4% (n = 76) received a primary resection. Three-year progression-free survival (PFS)/overall survival (OS) rates were 63.6%/81.4% in SCC and 73.1%/82.5% in AC, respectively. Sinonasal AC showed significantly more frequently negative PD-L1 expression and CPS <1 compared to sinonasal SCC (p = 0.035, p = 0.002). Sinonasal SCC with TPS score >50%, combined low IDO-1 score and high UICC stage showed significantly worse 3-year PFS and OS values (p < 0.05).</p><p><strong>Conclusion: </strong>The study identifies PD-L1 and IDO-1 as possible prognostic factors for sinonasal SCC, but less so for sinonasal AC. A TPS score >50% and combined low IDO-1 score correlate with shortened 3-year PFS and OS in sinonasal SCC. Immune checkpoints could, therefore, represent important target molecules for the prognosis assessment of sinonasal SCC.</p>","PeriodicalId":520736,"journal":{"name":"ORL; journal for oto-rhino-laryngology and its related specialties","volume":" ","pages":"1-10"},"PeriodicalIF":0.0,"publicationDate":"2025-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144145400","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Learning Curve of Upper Aerodigestive Tract Foreign Body Management for Otorhinolaryngology Residents.","authors":"Ching-Ching Chiu, Chien-Yu Huang, Keng-Ming Chang, Sheng-Chiao Lin, Yu-Hsi Liu, Pei-Hsun Liao, Chuan-Jen Hung, Chin-Fang Cho, Jyun-Yi Liao, Yun-Ying She, Bor-Hwang Kang","doi":"10.1159/000517307","DOIUrl":"https://doi.org/10.1159/000517307","url":null,"abstract":"<p><strong>Introduction: </strong>Foreign body ingestion is the most common reason for otolaryngology specialist consultations in emergency departments. Among the different types of foreign bodies, fish bones are the most common, particularly in Asian populations. In Taiwan, upper aerodigestive tract foreign bodies (UADT-FBs) are mostly managed by residents in the otorhinolaryngology (ORL) department. Considering the learning curve required for all procedures, different management types between residents, and possible resulting safety issues, this study explored the outcomes of UADT-FB management by residents in different years of ORL training.</p><p><strong>Materials and methods: </strong>The medical records of 2,283 patients who visited Kaohsiung Veterans General Hospital's Emergency Department for UADT-FB during June 2013-August 2019 were retrospectively reviewed. The reviewed data included the demographic data of enrolled patients, outcomes of foreign body management, and follow-up chart records of the patients.</p><p><strong>Results: </strong>Among the 2,283 patients, 1,324 (58%) were found to be negative for foreign bodies, and foreign bodies in 951 (41.7%) were removed immediately. In the negative finding (NF) group, 2 (4.9%) patients were later found to be positive for foreign bodies during follow-up in the outpatient department. One (2.4%) patient developed a deep neck infection and esophageal perforation. The percentage of NFs decreased from 62.58% in residents in the first half of their first year (R1a) to 54% for third-year residents (R3). Comparing R1a with R3, the number needed to harm for retained UADT-FBs after patients visited the emergency department was 12.2.</p><p><strong>Discussion/conclusion: </strong>This study provides data from 1 referral center regarding the management of UADT-FBs. With increasing resident training, the percentage of NFs declined from 62.58 to 54%. Young residents, especially those in the first 6 months of their training, should have senior residents perform a second examination if UADT-FBs are not found in suspected cases.</p>","PeriodicalId":520736,"journal":{"name":"ORL; journal for oto-rhino-laryngology and its related specialties","volume":" ","pages":"193-199"},"PeriodicalIF":1.3,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000517307","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39263322","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"High Mobility Group Box-1 Protein and Interleukin 33 Expression in Allergic Rhinitis.","authors":"Nongping Zhong, Qing Luo, Xiaoyan Huang, Jieqing Yu, Jing Ye, Jian Zhang","doi":"10.1159/000519575","DOIUrl":"https://doi.org/10.1159/000519575","url":null,"abstract":"<p><strong>Background: </strong>Allergic rhinitis (AR) is characterized by an inflammatory reaction. High mobility group box 1 (HMGB1) protein and interleukin (IL)-33 are damage-associated molecular pattern molecules and have many characteristics similar to pro-inflammatory cytokines. However, the role of IL-33 and HMGB1 in AR remains unclear. The aim of this study is to explore the role of HMGB1 and IL-33 in AR.</p><p><strong>Methods: </strong>Twenty patients with AR (AR group) and 10 normal controls (normal group) were enrolled in this study. HMGB1 and IL-33 expression were analyzed by immunohistochemistry in epithelial cells of the inferior turbinate mucosa samples. Then, the human nasal mucosa epithelial cells (HNECs) were cultured in vitro, and the house dust mite allergen (Derp1) was used to stimulate the cells. Quantitative real-time PCR and ELISA assay were performed to detect HMGB1 and IL-33 expression in HNECs.</p><p><strong>Results: </strong>The expression of HMGB1 and IL-33 in the nasal mucosa was higher in the AR group than in the normal group, with a statistically significant difference (p < 0.05). In HNECs of AR, the expression of both HMGB1 and IL-33 in stimulated groups was higher than that in non-stimulated groups. The differences were statistically significant (p < 0.05). In addition, they increased gradually with the prolonging time and the concentration of the added Derp1.</p><p><strong>Conclusions: </strong>The expression of HMGB1 and IL-33 were both increased in AR. HMGB1 and IL-33 may have a close relationship in AR.</p>","PeriodicalId":520736,"journal":{"name":"ORL; journal for oto-rhino-laryngology and its related specialties","volume":" ","pages":"315-323"},"PeriodicalIF":1.3,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39642808","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Carlos M Chiesa-Estomba, Manuel Graña, Alfonso Medela, Jon A Sistiaga-Suarez, Jerome R Lechien, Christian Calvo-Henriquez, Miguel Mayo-Yanez, Luigi Angelo Vaira, Alberto Grammatica, Giovanni Cammaroto, Tareck Ayad, Johannes J Fagan
{"title":"Machine Learning Algorithms as a Computer-Assisted Decision Tool for Oral Cancer Prognosis and Management Decisions: A Systematic Review.","authors":"Carlos M Chiesa-Estomba, Manuel Graña, Alfonso Medela, Jon A Sistiaga-Suarez, Jerome R Lechien, Christian Calvo-Henriquez, Miguel Mayo-Yanez, Luigi Angelo Vaira, Alberto Grammatica, Giovanni Cammaroto, Tareck Ayad, Johannes J Fagan","doi":"10.1159/000520672","DOIUrl":"https://doi.org/10.1159/000520672","url":null,"abstract":"<p><strong>Introduction: </strong>Despite multiple prognostic indicators described for oral cavity squamous cell carcinoma (OCSCC), its management still continues to be a matter of debate. Machine learning is a subset of artificial intelligence that enables computers to learn from historical data, gather insights, and make predictions about new data using the model learned. Therefore, it can be a potential tool in the field of head and neck cancer.</p><p><strong>Methods: </strong>We conducted a systematic review.</p><p><strong>Results: </strong>A total of 81 manuscripts were revised, and 46 studies met the inclusion criteria. Of these, 38 were excluded for the following reasons: use of a classical statistical method (N = 16), nonspecific for OCSCC (N = 15), and not being related to OCSCC survival (N = 7). In total, 8 studies were included in the final analysis.</p><p><strong>Conclusions: </strong>ML has the potential to significantly advance research in the field of OCSCC. Advantages are related to the use and training of ML models because of their capability to continue training continuously when more data become available. Future ML research will allow us to improve and democratize the application of algorithms to improve the prediction of cancer prognosis and its management worldwide.</p>","PeriodicalId":520736,"journal":{"name":"ORL; journal for oto-rhino-laryngology and its related specialties","volume":" ","pages":"278-288"},"PeriodicalIF":1.3,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39928422","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
JinYu Wang, Juan Ding, Jing Song, Li Hu, Ning Cong, Zhao Han
{"title":"A Prospective Study of the Effect of Tinnitus Sound Matching Degree on the Efficacy of Customized Sound Therapy in Patients with Chronic Tinnitus.","authors":"JinYu Wang, Juan Ding, Jing Song, Li Hu, Ning Cong, Zhao Han","doi":"10.1159/000517631","DOIUrl":"https://doi.org/10.1159/000517631","url":null,"abstract":"<p><strong>Objectives: </strong>The aim of this study was to explore and compare the customized sound therapy effect between tinnitus sound matching and nonmatching patients in tinnitus customized sound therapy and therapy-related influencing factors.</p><p><strong>Methods: </strong>This prospective study investigated a total of 100 patients with unilateral chronic tinnitus who received customized sound therapy. The participants were dichotomously divided into matching (group A) and nonmatching (group B) groups after 4 stages of tinnitus matching via the tinnitus assistant app (provided by Sound Ocean Company, SuZhou, China). Each group consists of 50 participants. Before and 6 months after the treatment, Hospital Anxiety and Depression Scale (HADS), tinnitus handicap inventory (THI), and tinnitus loudness Visual Analog Scale (VAS) were used to evaluate the customized sound therapy effect and explore other related influencing factors.</p><p><strong>Results: </strong>(1) The HADS-A, HADS-D, THI, and VAS scores of 2 groups were both significantly decreased after treatment. (2) The HADS-A and THI scores improved markedly in group A than that in group B, which could be related to the hearing loss of the tinnitus side ear before treatment; the lighter the degree of hearing loss, the better the improvement. No statistically significant differences were detected in HADS-D and VAS scores between the 2 groups, and also, these were not related to the degree of hearing loss. The differences in age, gender, and tinnitus duration did not show any statistically significant effect on the improvement of the 2 groups.</p><p><strong>Conclusions: </strong>Both tinnitus sound matching and nonmatching of the customized sound therapy brought a significant effect to tinnitus participants. Our study also suggests that THI and HADS-A scores of those with tinnitus matching participants improved markedly as compared to those of nonmatching participants, and the customized sound therapy effect is negatively correlated with the severity of hearing loss.</p>","PeriodicalId":520736,"journal":{"name":"ORL; journal for oto-rhino-laryngology and its related specialties","volume":" ","pages":"229-237"},"PeriodicalIF":1.3,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9227664/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39384064","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hannan A Qureshi, Anshu Bandhlish, Robert P DeConde, Ian M Humphreys, Waleed M Abuzeid, Aria Jafari
{"title":"Initial Presentation of Granulomatosis with Polyangiitis as Progressive Skull Base Osteomyelitis.","authors":"Hannan A Qureshi, Anshu Bandhlish, Robert P DeConde, Ian M Humphreys, Waleed M Abuzeid, Aria Jafari","doi":"10.1159/000518971","DOIUrl":"https://doi.org/10.1159/000518971","url":null,"abstract":"<p><p>A healthy man in his 30s presented with a 2-week history of severe bitemporal pain and pressure. He was initially treated for presumed acute rhinosinusitis, but his symptoms continued to worsen and underwent endoscopic sinus surgery at an outside community facility. He developed left abducens nerve palsy postoperatively, and magnetic resonance imaging (MRI) demonstrated evidence of extensive skull base osteomyelitis. He was initiated on intravenous (IV) broad-spectrum antibiotics but was subsequently found to have prostatic and submandibular sterile fluid collections. The patient subsequently developed new right abducens and left vagal nerve palsies and underwent revision endoscopic sinus surgery. Pathology revealed extensive inflammation, necrotizing granulomas, and evidence of small and medium vessel vasculitis. Extensive laboratory workup was negative, except for anti-PR-3 antibody positivity. Given the characteristic findings on pathology and laboratory findings, the patient was diagnosed with granulomatosis with polyangiitis (GPA). High-dose glucocorticoid therapy as well as rituximab infusion were promptly initiated. He had marked improvement in his symptoms and resolution of his right CN VI palsy but left-sided CN VI and CN X palsies persisted. This patient presented without the typical rhinologic manifestations of GPA, and rather presented with progressive sinusitis, skull base osteomyelitis with associated cranial neuropathies, and aseptic systemic abscesses. Prompt diagnosis of GPA is particularly important in those with otorhinolaryngological manifestations, as early initial immunosuppressive therapy has been linked to lower relapse and mortality rates. Vigilance and early differentiation between GPA and other forms of sinusitis is of critical importance, particularly when symptoms are refractory to standard rhinosinusitis therapies.</p>","PeriodicalId":520736,"journal":{"name":"ORL; journal for oto-rhino-laryngology and its related specialties","volume":" ","pages":"342-346"},"PeriodicalIF":1.3,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39474150","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Desmoplastic Small Round Cell Tumor of the Submandibular Gland: A Case Report and Literature Review.","authors":"Qingjiao Li, Xiaolu Yuan","doi":"10.1159/000517563","DOIUrl":"https://doi.org/10.1159/000517563","url":null,"abstract":"<p><p>Desmoplastic small round cell tumor (DSRCT) is a rare and aggressively malignant tumor mostly occurring in the abdominal and pelvic cavity of young patients. However, few cases had been reported concerning DSRCT occurring in the head and neck region. We presented a rare case of DSRCT of the right submandibular in a 25-year-old man. MRI revealed a 3 × 2-cm solid nodule located in the right submandibular, and physical examination showed no other occupying lesion elsewhere. Histologically, the tumor was composed of various-sized small round cell nests, embedded in an abundant desmoplastic stroma. Immunohistochemically, the tumor cells were typically positive for epithelial (CK and EMA), mesenchymal (vimentin and desmin), and neuroendocrine (CD56, NSE, Syn, and CgA) markers, but negative for WT1. Fluorescence in situ hybridization revealed the presence of a break apart involving the Ewing sarcoma (EWS) gene. The patient received chemotherapy and radiotherapy and relapsed after 19 months of follow-up. DSRCT of the submandibular gland is rare, and the diagnosis of this tumor in an uncommon location relies on the histomorphology, immunophenotype, and EWS gene translocation detection. Differential diagnosis including primary salivary gland tumors and the other small round cell tumors needs to be excluded.</p>","PeriodicalId":520736,"journal":{"name":"ORL; journal for oto-rhino-laryngology and its related specialties","volume":" ","pages":"262-268"},"PeriodicalIF":1.3,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000517563","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39252853","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}