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European Journal of Pediatrics Jun 2024Achondroplasia (ACH; MIM #100,800), caused by a heterozygous gain of function pathogenic variant in the fibroblast growth factor receptor 3 gene (FGFR3; MIM*134,934), is...
Achondroplasia (ACH; MIM #100,800), caused by a heterozygous gain of function pathogenic variant in the fibroblast growth factor receptor 3 gene (FGFR3; MIM*134,934), is the most prevalent and most readily identifiable cause of disproportionate short stature that is compatible with life. In addition, individuals with achondroplasia face significant medical, functional, and psychosocial challenges throughout their lives. This study assessed associated morbidities in patients with achondroplasia at a single center in Turkey. In this study, the clinical findings and associated morbidities of a group of patients with achondroplasia (n = 68) with clinical multidisciplinary follow-up at a single center between the years 2005-2023 are evaluated retrospectively. A total of 68 patients, 30 male (44.1%) and 38 female (55.9%), were evaluated. In the majority (84.2%) of patients, shortness of extremities was detected in the prenatal period at an average of 28.7 gestational weeks (± 3.6 SDS) with the aid of ultrasonography. More than half (n = 34/63, 54%) of the patients had a father of advanced paternal age (≥ 35 years). Among the complications, respiratory system manifestations, including obstructive sleep apnea (70%), ear-nose-throat manifestations including adenoid hypertrophy (56.6%) and otitis media (54.7%), neurological manifestations due to foramen magnum stenosis (53.2%), and skeletal manifestations including scoliosis (28.8%), are represented among the most common. The mortality rate was 7.3% (n = 5/68).Conclusion: This study not only represents the first retrospective analysis of the associated morbidities of patients with achondroplasia from a single center in Turkey but also will provide a reference point for future studies.
PubMed: 38879704
DOI: 10.1007/s00431-024-05643-y -
Seminars in Diagnostic Pathology Jul 2024Basaloid salivary gland neoplasms are a diverse and varied group of benign and malignant tumors. The term 'basaloid' is broadly used in reference to cells with elevated... (Review)
Review
Basaloid salivary gland neoplasms are a diverse and varied group of benign and malignant tumors. The term 'basaloid' is broadly used in reference to cells with elevated nuclear to cytoplasmic ratio, sparse cytoplasm, and hyperchromatic nuclei. However, a subset may also fit within the "small round blue cell tumor" morphologic category or the "biphasic" salivary gland tumor category. Furthermore, there are no established thresholds for the proportion of basaloid tumor cells needed to consider a tumor within the basaloid spectrum. Given the implicit variability in what is considered a basaloid salivary gland tumor, one may question the inclusion of certain entities (canalicular adenoma, HMGA2::WIF1 pleomorphic adenoma, polymorphous adenocarcinoma) in this review based on classic morphologic features. However, salivary gland tumors with even minor basaloid components may appear 'basaloid' in small biopsy specimens and, thus, a choice was made to focus on common and uncommon diagnostic differentials with this in mind. Entities that will be covered in this review also include basal cell adenoma and basal cell adenocarcinoma, adenoid cystic carcinoma, lymphoepithelial carcinoma, sialoblastoma, adamantinoma-like Ewing Sarcoma, NUT carcinoma, and carcinoma showing thymus-like differentiation.
Topics: Humans; Salivary Gland Neoplasms; Diagnosis, Differential; Biomarkers, Tumor
PubMed: 38879353
DOI: 10.1053/j.semdp.2024.06.003 -
International Journal of Pediatric... Jun 2024The purpose of this study was to investigate the prevalence of otitis media and other otolaryngologic disorders in children with Obstructive Sleep Apnea (OSA) compared...
INTRODUCTION
The purpose of this study was to investigate the prevalence of otitis media and other otolaryngologic disorders in children with Obstructive Sleep Apnea (OSA) compared to those without OSA in a large cohort.
METHODS
A retrospective cohort study was carried within the US Collaborative Network within TriNetX. The OSA group was defined by ICD-10 code G47.33 and non-OSA group excluded patients with OSA. Both groups were required to have a CPT code for an outpatient visit to act as a control: 99202-99215. Propensity score matching for age, sex, and race was performed. Prevalence of otitis media (ICD-10H65, H66), chronic otitis media (ICD-10H66.1, H66.2, H66.3, H65.2, H65.3, and H65.4), tympanostomy (CPT 69433, 69436), adenoidectomy (CPT 42830, 42831), tonsillectomy (CPT 42825, 42826), adenotonsillectomy (CPT 42820, 42821), and allergic rhinitis (ICD-10 J30.9) were compared in this cohort.
RESULTS
Propensity score matching yielded 165,665 (M = 95949, F = 69901) patients with a mean age of 10.7 (SD = 4.07) for each cohort. Children with OSA were 1.27x and 3.86x more likely to be diagnosed with otitis media and chronic otitis media, respectively (P<.0001). They were 3.81x more likely to undergo a tympanostomy (P<.0001). Children with OSA were 4.1x, 18.2x, and 24.7x more likely undergo an adenoidectomy, tonsillectomy, and adenotonsillectomy, respectively (P<.0001). Children with OSA were also 2.03x as likely to have a diagnosis of allergic rhinitis (P<.0001).
CONCLUSION
Children with OSA experience otitis media and related surgical intervention more than children without OSA. Since allergic rhinitis and adenoid hypertrophy are contributors to both OSA and AOM, their increased prevalence in children with OSA may explain their increased frequency of AOM.
PubMed: 38878449
DOI: 10.1016/j.ijporl.2024.112014 -
Zhonghua Jie He He Hu Xi Za Zhi =... Jun 2024Obstructive sleep apnea (OSA) shows sex differences in the pathophysiology, epidemiology, and clinical presentation. Women have different characteristics of OSA at...
Obstructive sleep apnea (OSA) shows sex differences in the pathophysiology, epidemiology, and clinical presentation. Women have different characteristics of OSA at different life stages. Based on 26 guidelines and consensus, 121 English literatures, and 24 Chinese literatures, the Sleep Disorder Group of Chinese Thoracic Society has drafted a consensus with multidisciplinary experts to summarize the epidemiology, clinical characteristics, diagnosis, treatment, and follow-up of OSA in women at different life stages, particularly issues related to OSA during pregnancy. The consensus is divided into four parts: epidemiology, diagnosis, treatment, and issues for pregnant women with OSA, with 34 recommendations covering 13 clinical issues. The aim was to improve the understanding and managements of OSA in women. What is the prevalence of OSA in women at different life stages?The prevalence of OSA varies among women at different life stages. Sex differences are not significant in childhood and adolescence. The prevalence of OSA in women of childbearing age is significantly lower than that in men. The prevalence of OSA increases during pregnancy due to changes in hormone levels and the influence of pregnancy physiology, as well as with gestational weeks. In postmenopausal women, the prevalence of OSA increases significantly, and the sex differences are no longer significant. What are the risk factors for OSA in women at different life stages?The risk factors for OSA in women at different life stages are not identical. (1) Childhood and adolescence: Tonsillar and adenoid hypertrophy, obesity, and craniofacial structural anomalies increase the risk of OSA; (2) Childbearing age: The prevalence of OSA in women is lower than in men. However, obesity, hypothyroidism, acromegaly, and polycystic ovary syndrome increase the risk of OSA, and these patients should be screened for OSA; (3) Pregnancy: hormonal effects, uterine enlargement, and weight changes increase the risk of OSA, especially in those with a history of snoring or OSA before pregnancy; (4) Perimenopausal and post-menopausal periods: Decreased levels of estrogen/progesterone reduce the protective effects on the upper airways, and increase the risk of OSA. Menopause is an important risk factor for OSA in women. What are the harms of OSA in women?OSA is an independent risk factor for diseases such as hypertension, cardiovascular and cerebrovascular diseases, metabolic disorders, emotional and cognitive impairments, and malignant tumors in women. OSA during pregnancy has several adverse effects on maternal and infant health, and is associated with increased risks of preeclampsia, hypertensive disorders complicating pregnancy (HDP), gestational diabetes mellitus (HDM), premature birth, neonatal asphyxia, fetal growth restriction, . What are the clinical symptoms and physical signs of OSA in women?The symptoms of OSA in women are different from those in men. Attention should be paid to whether women snore and the frequency of snoring, especially among postmenopausal and obese women. The atypical symptoms of OSA, including insomnia, daytime fatigue, morning headache, anxiety and nightmares, should not be ignored, especially in postmenopausal, obese, and pregnant women. When should women be screened for OSA?(1) Postmenopausal and pregnant women, as well as women with a first-degree relative with OSA. It should be noted that the clinical symptoms of OSA in women are not typical; (2) Women with polycystic ovary syndrome, hypothyroidism, and acromegaly; (3) Women engaged in various occupations, including driving and working at heights. How to screen OSA in women?Many screening tools and questionnaires can be used to screen for OSA, but should not be used to diagnose OSA in the absence of objective sleep tests. (1) Questionnaires and screening tools: The STOP-Bang questionnaire targeting the general population has higher sensitivity than Berlin Questionnaire (BQ), Epworth Sleepiness Scale (ESS), and others. STOP Bang≥3 points combined with ESS can further improve its specificity and can be used for OSA screening in women. However, the questionnaire has poor sensitivity for female OSA. Type Ⅳ monitoring devices can be used for OSA screening in women with a weak recommendation; (2) PSG is the gold standard for diagnosis. Type Ⅱ or Ⅲ portable monitoring (PM) devices are recommended for the diagnosis of OSA in women in the following conditions: 1) Diagnosis of high-risk OSA patients without complex comorbidities; 2) OSA patients who are immobile or critically ill and unable to undergo PSG monitoring in a sleep center; 3) Diagnosis of perioperative OSA patients; 4) Pregnant women with high suspicion of OSA. How to diagnose OSA in women?The diagnostic and grading criteria for adult non-pregnant women with OSA are the same as the diagnostic criteria for adult OSA; for diagnosis and grading of OSA in pregnant women, see "Section 4: OSA in Pregnancy". How to treat OSA in women?For all the OSA patients with varying degrees of severity in women, the general treatment can be applied: weight loss, dietary control, exercise, position therapy, reduction of alcohol intake, and cautious use of sedative and hypnotic drugs. Medical costs and the risk of comorbidities with OSA in women are higher than those in men. Therefore, OSA patients in women should be promptly evaluated and treated. How to optimize non-invasive positive pressure ventilation (NPPV) treatment and improve compliance for OSA patients in women?(1) NPPV is the first-line treatment for moderate to severe OSA in women. It can relieve upper airway obstruction, eliminate sleep hypoxia, improve sleep quality and quality of life, and reduce the incidence of related complications and mortality; (2) To improve compliance with NPPV treatment, behavioral interventions and patient education are recommended. Selecting an appropriate human-machine interface, improving the humidification effect, promptly handling adverse reactions, and applying remote medical models may improve the compliance. What are the other options for OSA treatment in women?Other treatment methods include oral appliances, upper airway surgery, and sublingual nerve stimulation therapy, which have moderate therapeutic effects in women. Postmenopausal hormone therapy (MHT) in women has a certain therapeutic effect on OSA, but its safety needs further evaluation. What is follow-up evaluation for OSA in women?(1) Follow-up every 6 months or 1 year after receiving NPPV treatment; (2) PSG should be rechecked at the 3rd and 6th months after surgical treatment to evaluate the therapeutic effects. For patients with poor therapeutic effects after surgery, it is recommended to use treatments such as NPPV; (3) PSG should be rechecked at the 3rd and 6th months after oral appliance treatment. Oral appliances should be adjusted as needed to consolidate long-term efficacy, or switched to a treatment such as NPPV; (4) During follow-up, attention should be paid to the improvement of apnea hypopnea index(AHI), symptoms, and side effects; (5) It is recommended that NPPV treatment be remotely managed via the internet, which can provide high-quality and comprehensive sleep care; (6) Follow-up of OSA during pregnancy can be found in "Section 4: OSA in Pregnancy ". How to diagnose and evaluate OSA during pregnancy?OSA during pregnancy has adverse effects on maternal and infant outcomes. It is recommended that high-risk pregnant women be screened and diagnosed for OSA during pregnancy management and healthcare.(1) Screening of the high-risk population: Individuals who meet any of the following criteria are considered at high risk for OSA during pregnancy. 1) Symptoms: snoring during sleep, arousal, headache in the morning, insomnia, depression, excessive daytime sleepiness, and fatigue; 2) Pregnant women over 35 years old; 3) Physical signs: weight exceeding standard body weight by 20% or more, BMI≥28 kg/m, and neck circumference>40 cm; anatomical abnormalities of the upper airways, such as nasal obstruction, tonsil hypertrophy, and mandibular retrognathia, .; 4) Combined internal medicine diseases, such as refractory hypertension, unknown arrhythmia, chronic congestive heart failure, refractory diabetes and insulin resistance, refractory asthma, hypothyroidism, primary aldosteronism; 5) Those with obstetric related diseases, such as preeclampsia, HDP, GDM, and intrauterine growth restriction of the fetus, and with symptoms of chest tightness and apnea that cannot be explained by other factors, and with previous history of gestational OSA or family history.(2) Screening time: There is currently no strong evidence to support the recommendation for optimal screening time. Given the adverse effects of OSA on mothers and infants, it is recommended that high-risk individuals of OSA be screened for OSA between12 and 18 weeks of pregnancy.(3) Screening tools: The main manifestations of OSA in pregnant women are insomnia and poor sleep quality, whereas daytime drowsiness is often not severe. Various sleep questionnaires and models for OSA in pregnancy have poor sensitivity and specificity. Type Ⅳ and consumer-level monitoring devices are lack of sufficient clinical validation. It is recommended that the results of the above screening tools should only have an indicative role in the diagnosis of OSA during pregnancy.(4) Diagnostic tools: PSG is the gold standard for the diagnosis of OSA in pregnancy. PM may be the first choice diagnostic technique for OSA in pregnancy, and Type Ⅲ monitoring devices are the most commonly used devices.(5) Diagnostic criteria: Diagnosis of OSA during pregnancy should be based on symptoms, signs, and PSG or PM monitoring results. Diagnostic criteria for OSA during pregnancy are as follows: 1) PSG or PM monitoring shows AHI≥5 times/h with symptoms or signs of OSA in women, or with related complications (such as diagnosed hypertension, emotional disorders, unexplained arrhythmias, chronic congestive heart failure, HDP, HDM, intrauterine growth restriction that cannot be explained by other factors, chest tightness and apnea excluding other reasons), or with previous history of OSA or family history of OSA; 2) PSG or PM monitoring shows AHI≥10 times/h in those with less daytime drowsiness (ESS≤9 points). How to manage OSA during pregnancy?(1) Once OSA is diagnosed during pregnancy, personalized treatment plans from pregnancy to birth should be developed through collaborative discussions between sleep center professionals, obstetricians, pregnant women, and their families. Multidisciplinary collaboration among anaesthesia, neonatology, and critical care medicine may be required in some cases. A comprehensive management approach should be adopted based on the patient's condition, which includes strengthening weight management, positioning treatment, NPPV treatment, oral appliances, and management of maternal and infant complications; (2) Considering the Regarding continuous weight gain during pregnancy, APAP treatment is more appropriate mode for pregnant women with OSA; (3) Oral appliances are suitable for patients with snoring or mild to moderate OSA, especially those with combined mandibular retraction or NPPV intolerance. However, oral appliances are not recommended as the first-line treatment; (4) It is not recommended to use surgical methods to treat OSA during pregnancy; (5) Follow-up and evaluation: Patients' conditions should be re-evaluated and treatment plans should be adjusted at around 24 weeks of pregnancy. Postpartum PSG or PM monitoring should be repeated to assess the need for continued treatment after delivery.
Topics: Humans; Sleep Apnea, Obstructive; Female; Pregnancy; Pregnancy Complications; Consensus
PubMed: 38858201
DOI: 10.3760/cma.j.cn112147-20240206-00072 -
Lin Chuang Er Bi Yan Hou Tou Jing Wai... Jun 2024To explore the methods of resection, dura and skull base repair and reconstruction of cranionasal communication tumor. Data of 31 patients with cranionasal communication...
To explore the methods of resection, dura and skull base repair and reconstruction of cranionasal communication tumor. Data of 31 patients with cranionasal communication tumor who underwent dura and skull base reconstruction after tumor resection from 2018 to 2022 were collected. Follow-up lasted for 3 to 41 months. A total of 31 patients were enrolled, including 20 males and 11 females. The ages ranged from 19 to 74 years, with a median age of 57 years old. There were 17 benign lesions(one case of hemangioma, one case of Rathke cyst, one case of squamous papilloma, one case of craniopharyngioma, two cases of meningocele, two cases of varus papilloma, two cases of meningioma of grade Ⅰ, three cases of schwannoma, four cases of pituitary tumor) and 14 malignant lesions(one case of osteosarcoma, one case of poorly differentiated carcinoma, two cases of varus papilloma malignancy, two cases of olfactory neuroblastoma, two cases of adenocarcinoma, two cases of adenoid cystic carcinoma, four cases of squamous cell carcinoma) . Sixteen cases underwent nasal endoscopy combined with craniofacial incision and 15 cases underwent nasal endoscopy surgery alone. Complete resection of the mass and dura and skull base reconstruction were performed in all 31 patients, and free graft repair was performed in 8 cases(fascia lata in 5 cases and nasal mucosa in 3 cases). Twenty-three cases were repaired with pedicled flaps(septal mucosal flap alone in 11 cases, septal mucosal flap combined with free graft in 6 cases, and cap aponeurosis combined with free graft in 6 cases). Eight out of 31 patients underwent skull base bone repair. Postoperative cerebral hemorrhage occurred in 1 case, cerebrospinal fluid leakage in 1 case, intracranial infection in 2 cases. All patients were successfully treated without severe sequelae. Cerebrospinal fluid leakage and intracranial infection occurred in one patient after radiotherapy, who recovered after conservative treatment. All 17 patients with benign lesions survived. Thirteen out of 14 patients with malignant lesions received radiotherapy after surgery, nine survived without recurrence, five cases recurred, of which 2 survived with tumor, one underwent reoperation and 2 died. Cranionasal communication tumors are high-risk diseases of anterior and middle skull base, and various surgical repair methods could be selected after complete resection of the tumor. Successful reconstruction and multidisciplinary cooperation are crucial for treatment outcome.
Topics: Humans; Male; Middle Aged; Female; Adult; Plastic Surgery Procedures; Aged; Skull Base; Young Adult; Dura Mater; Skull Base Neoplasms
PubMed: 38858114
DOI: 10.13201/j.issn.2096-7993.2024.06.008 -
Lin Chuang Er Bi Yan Hou Tou Jing Wai... Jun 2024To analyze the difference in 5-year survival between maxillary sinus adenoidal cystic carcinoma(maxillary sinus adenoid cystic carcinoma, MSACC) and squamous cell... (Comparative Study)
Comparative Study
[Comparison of 5-year survival rate and analysis of prognostic factors influencing the prognosis of adenoid cystic carcinoma and squamous cell carcinoma of the maxillary sinus: based on the SEER database].
To analyze the difference in 5-year survival between maxillary sinus adenoidal cystic carcinoma(maxillary sinus adenoid cystic carcinoma, MSACC) and squamous cell carcinoma(maxillary sinus squamous cell carcinoma, MSSCC) using the National Cancer Institute's Surveillance, Epidemiology, and End. database(SEER) and to explore the factors associated with the prognosis of the two tumors. The data of 161 patients with MSACC and 929 patients with MSSCC were collected from SEER database, and the 5-year overall survival rate(OS) and tumor specific survival rate(CSS) were compared between the two groups before and after propensity score matching. The forest map of multivariate Cox proportional hazard regression model was established to analyze the prognostic factors affecting the survival rate of patients with MSACC and MSSCC. There were statistical differences in 5-year OS and CSS between MSACC and MSSCC before and after propensity score matching(<0.001). Multivariate regression analysis showed that age, side of the disease, lymph node metastasis, operation and radiotherapy were the influencing factors of OS in MSACC, while age and operation were the influencing factors of CSS. Age, race, T grade, lymph node metastasis, systemic metastasis, surgery, radiotherapy and chemotherapy are the influencing factors of OS of MSSCC. Age, T grade, lymph node metastasis, systemic metastasis, surgery, radiotherapy and chemotherapy are the influencing factors of CSS. The 5-year survival rate of MSACC is higher than that of MSSCC. Surgery plays a positive role in the prognosis of the two kinds of tumors. The analysis results can provide some reference for their survival expectations and treatment choices.
Topics: Humans; Female; Male; Carcinoma, Adenoid Cystic; Prognosis; Carcinoma, Squamous Cell; SEER Program; Middle Aged; Survival Rate; Propensity Score; Maxillary Sinus Neoplasms; Maxillary Sinus; Proportional Hazards Models; Lymphatic Metastasis; Aged; Adult
PubMed: 38858112
DOI: 10.13201/j.issn.2096-7993.2024.06.006 -
Is epithelial-mesenchymal transition related to the biological behavior of salivary gland neoplasms?Archives of Oral Biology Sep 2024To evaluate and compare the expression of E-cadherin, Snail1 and Twist1 in pleomorphic adenomas (PAs), adenoid cystic carcinomas (AdCCa) and carcinoma ex-pleomorphic...
OBJECTIVE
To evaluate and compare the expression of E-cadherin, Snail1 and Twist1 in pleomorphic adenomas (PAs), adenoid cystic carcinomas (AdCCa) and carcinoma ex-pleomorphic adenomas (CaexPA) of salivary glands, as well as investigate possible associations with clinicopathological parameters.
STUDY DESIGN
E-cadherin, Snail1 and Twist1 antibody immunostaining were analyzed semiquantitatively in 20 PAs, 20 AdCCas and 10 CaexPAs. Cases were classified as low and high expression for analysis of the association with clinicopathological parameters.
RESULTS
Compared to PAs, AdCCas and CaexPAs exhibited higher nuclear expression of Snail1 (p = 0.021 and p = 0.028, respectively) and Twist1 (p = 0.009 and p = 0.001). Membranous and cytoplasmic expression of E-cadherin were positively correlated in PAs, AdCCas and CaexPAs (r = 0.645, p = 0.002; r = 0.824, p < 0.001; r = 0.677, p = 0.031). In PAs, positive correlation was found between nuclear expression of Snail1 and membrane expression of E-cadherin (r = 0.634; p = 0.003), as well as between nuclear expression of Snail1 and Twist1 (r = 0.580; p = 0.007). Negative correlations were detected between membrane expression of E-cadherin and cytoplasmic expression of Snail1 in AdCCas (r = - 0.489; p = 0.029).
CONCLUSIONS
E-cadherin, Twist1, and Snail1 may participate in modulating events related to cell differentiation and adhesion in PAs and to biological behavior in AdCCas and CaexPAs, which indicates the involvement of EMT in these processes. Furthermore, the expression of these proteins in these carcinomas may reflect the plasticity feature of EMT.
Topics: Humans; Epithelial-Mesenchymal Transition; Salivary Gland Neoplasms; Snail Family Transcription Factors; Cadherins; Female; Male; Twist-Related Protein 1; Middle Aged; Carcinoma, Adenoid Cystic; Nuclear Proteins; Adult; Adenoma, Pleomorphic; Aged; Twist Transcription Factors; Immunohistochemistry; Transcription Factors; Biomarkers, Tumor
PubMed: 38852529
DOI: 10.1016/j.archoralbio.2024.106017 -
Auris, Nasus, Larynx Jun 2024While several studies reported epidermal growth factor receptor (EGFR) expression in salivary gland cancer (SGC), results varied due to a lack of unified definition of...
OBJECTIVE
While several studies reported epidermal growth factor receptor (EGFR) expression in salivary gland cancer (SGC), results varied due to a lack of unified definition of EGFR positivity. In this study, we assessed the EGFR expression level using both EGFR positive score and cumulative EGFR score in the patients with SGC.
METHODS
Between January 2010 and April 2021, 102 patients with SGC who underwent surgical resection were reviewed retrospectively by immunohistochemistry. The membrane staining intensity was scored as follows: no staining (0), weak staining (1+), intermediate staining (2+), and strong staining (3+). The cumulative EGFR score was determined on a continuous scale of 0-300 using the formula:1 × (1+: percentage of weakly stained cells) + 2 × (2+: percentage of moderately stained cells) + 3 × (3+: percentage of strongly stained cells).
RESULTS
EGFR expression in SGC varied widely even among the same as well as different histopathological types. The average EGFR positive scores were 46.0 %, 55.7 %, 51.6 %, 1.0 %, 26.8 %, 50 %, and 76.8 % for mucoepidermoid carcinoma (MEC), salivary duct carcinoma (SDC), adenoid cystic carcinoma (AdCC), acinic cell carcinoma (AcCC), adenocarcinoma NOS (ACNOS), carcinoma ex pleomorphic adenoma (CAexPA), and squamous cell carcinoma (SqCC), respectively. The average cumulative EGFR scores were 82, 91, 80, 1, 52, 93, and 185 for MEC, SDC, AdCC, AcCC, ACNOS, CAexPA, and SqCC, respectively.
CONCLUSIONS
EGFR positive scores and cumulative EGFR scores in SGCs varied among the various histological types, and even in the same histological type. These scores may predict the clinical outcome of SGC treated with EGFR-targeting therapies, such as head and neck photoimmunotherapy, and need to be evaluated in future studies.
PubMed: 38852332
DOI: 10.1016/j.anl.2024.05.007 -
Applied Immunohistochemistry &... Jun 2024There is a limited amount of data on the role of programmed cell death ligand (PD-L) -1 and PD-L2 in salivary gland carcinomas. We aimed to evaluate the prognostic value...
There is a limited amount of data on the role of programmed cell death ligand (PD-L) -1 and PD-L2 in salivary gland carcinomas. We aimed to evaluate the prognostic value of PD-L1 and PD-L2 expressions, which are closely related to immune mechanisms, with respect to salivary gland tumor types and stages. Data from patients with salivary gland masses surgically removed between 2006 and 2021, diagnosed with a malignant salivary gland neoplasm, were retrospectively analyzed. Immunoreactivity for PD-L1 and PD-L2 was performed on resection materials. The mean age of 90 patients was 52.1±18.8 and 46.7% were male. Overall, 55.6% of patients were diagnosed with adenoid cystic carcinoma (ACC), 23.3% with mucoepidermoid carcinoma (MEC), 16.7% with acinic cell carcinoma (AciCC), 3.3% with ductal carcinoma (DC), and 1 patient with pleomorphic adenoma ex carcinoma (PA-ex-CA). In all, 52% of ACC, 12% of AciCC, 24% of MEC, and 12% of DC cases were at stage IV. The tumor diameter, frequencies of lymphovascular invasion, metastasis, positive surgical margin, recurrence, and mortality rates of patients at stages III and IV were significantly larger than those at stages I and II (P<0.05). The percentages of tumor cell score (TCS) and immune cell score (ICS) for PD-L1 were significantly higher among patients with MEC compared with those with other types of tumors (P=0.0011). However, the percentages of combined score (CS) for PD-L1 and tumor cell score for PD-L2 were comparable among tumor types (P>0.05). No significant difference was found in these scores for PD-L1 between tumor stages (P>0.05), but for PD-L2, all patients at stage I had TCS <1% for PD-L2, while all patients at stages II and III, and 92% of patients at stage IV had TCS ≥1% (P<0.0001). High expression of PD-L1 was mostly observed in MEC cases (P=0.0016), while all patients with AciCC had a low PD-L1 expression level (P=0.0206). The mean tumor diameter, rate of lymphovascular invasion, perineural invasion, metastasis, positive surgical margin, recurrence, type of treatment, mortality, and TILs ratio did not differ significantly according to PD-L1 expression level (P>0.05). The percentage of tumor-infiltrating lymphocytes was comparable among negative and positive PD-L1 scores according to both 1% and 5% threshold values (P>0.05). High PD-L1 expression is rare in AciCC, while PD-L1 expression is high in MEC. Our findings underline the importance of future screening for PD-L1 and PD-L2 before patients undergoing immunotherapies in all salivary gland tumors.
PubMed: 38847110
DOI: 10.1097/PAI.0000000000001209 -
BMC Cancer Jun 2024Primary tracheal tumors are very rare and their management is not definitely established. Due to its rarity, providing patient care in terms of optimal management poses...
OBJECTIVE
Primary tracheal tumors are very rare and their management is not definitely established. Due to its rarity, providing patient care in terms of optimal management poses a considerable challenge. The purpose of this study was to investigate treatment outcomes in patients with these rare tumors.
METHODS
We carried out a retrospective analysis of 89 patients with primary tracheal tumors treated at the Maria Sklodowska-Curie National Research Institute of Oncology in Warsaw, Poland, over sixteen years. The study assessed patient demographics, tumor characteristics and treatment. Different treatment options were compared in terms of overall survival, disease-free survival, and progression-free survival.
RESULTS
A total of 89 patients were included in the study. In the group presented, 45 patients underwent primary radical treatment and 44 were qualified for palliative treatment. Surgical resection was performed in 13 patients out of radically treated patients. The 5 year OS rates in the group of patients who underwent radical treatment and in the group of patients who underwent palliative treatment were 45.9% and 2.3%, respectively. In the group of patients who underwent radical surgical treatment, the 5 year OS was 76.9% compared to 35.8% in the group of patients who underwent nonsurgical treatment.
CONCLUSION
A multidisciplinary team should decide treatment options, including in-depth consideration of surgical treatment options.
Topics: Humans; Male; Tracheal Neoplasms; Retrospective Studies; Female; Middle Aged; Aged; Adult; Treatment Outcome; Aged, 80 and over; Palliative Care; Young Adult; Poland; Adolescent
PubMed: 38840114
DOI: 10.1186/s12885-024-12450-z