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Journal of Medical Case Reports Jun 2024Salivary gland-type lung carcinomas are uncommon neoplasms of the lung, representing less than 1% of all lung tumors. The two most common among them are adenoid cystic...
BACKGROUND
Salivary gland-type lung carcinomas are uncommon neoplasms of the lung, representing less than 1% of all lung tumors. The two most common among them are adenoid cystic carcinoma and mucoepidermoid carcinoma. Although they usually have an indolent behavior, adenoid cystic carcinomas can be more aggressive, with 5-year survival as low as 55%. Very few cases are reported in literature. We report a similar rare case of salivary gland type lung carcinoma that presented for the first time with unilateral opacification of left hemithorax.
CASE PRESENTATION
A 38-year-old man of North Indian origin, who was a a nonsmoker, presented with complaints of shortness of breath and cough for 1 year, which has increased in the last 2 months and was associated with significant weight loss. A frontal radiograph of the chest and computed tomography of the chest were performed, which showed a mass in the left upper lobe of the lung with its epicenter in the left main bronchus. A bronchoscopic guided biopsy was performed, and histopathology confirmed the diagnosis of lung carcinoma of salivary gland type (adenoid cystic carcinoma). There was invasion of major vessels, hence the patient was offered and started on palliative management instead of surgical treatment. In spite of palliative management of two cycles of chemotherapy and radiotherapy, the patient succumbed to the disease within 2 months from the time of diagnosis.
CONCLUSION
Lung carcinoma of the salivary gland type (especially adenoid cystic carcinoma) usually presents at a later stage. The resectability of the tumor depends on the involvement of the surrounding major vessels. Interestingly, these cancers have no association with smoking. The prognosis depends on the extent of the disease at the time of diagnosis. Hence, imaging plays a major role in deciding the further plan of management.
Topics: Humans; Male; Adult; Lung Neoplasms; Tomography, X-Ray Computed; Carcinoma, Adenoid Cystic; Liver Neoplasms; Fatal Outcome; Bone Neoplasms; Palliative Care
PubMed: 38909203
DOI: 10.1186/s13256-024-04607-y -
Paediatric Respiratory Reviews Jun 2024Obstructive sleep apnea (OSA) due to a hypertrophy of the adenoids and/or the tonsils in otherwise healthy children is associated with neurocognitive dysfunction and... (Review)
Review
Obstructive sleep apnea (OSA) due to a hypertrophy of the adenoids and/or the tonsils in otherwise healthy children is associated with neurocognitive dysfunction and behavioural disorders with various degrees of hyperactivity, aggressiveness, sometimes evolving to a label of attention-deficit hyperactivity disorder. Children with anatomical and/or functional abnormalities of the upper airways represent a very specific population which is at high risk of OSA (also called complex OSA or OSA type III). Surprisingly, the neurocognitive consequences of OSA have been poorly studied in these children, despite the fact that OSA is more common and more severe than in their healthy counterparts. This may be explained by that fact that screening for OSA and sleep-disordered breathing is not systematically performed, the performance of sleep studies and neurocognitive tests may be challenging, and the respective role of the underlining disease, OSA, but also poor sleep quality, is complex. However, the few studies that have been performed in these children, and mainly children with Down syndrome, tend to show that OSA, but even more disruption of sleep architecture and poor sleep quality, aggravate the neurocognitive impairment and abnormal behaviour in these patients, underlining the need for a systematic and early in life assessment of sleep and neurocognitive function and behaviour in children with OSA type III.
PubMed: 38908984
DOI: 10.1016/j.prrv.2024.06.004 -
Przeglad Epidemiologiczny Jun 2024Respiratory tract infections in children are an interdisciplinary problem that pediatricians, allergists, laryngologists and immunologists encounter on a daily basis. In...
INTRODUCTION
Respiratory tract infections in children are an interdisciplinary problem that pediatricians, allergists, laryngologists and immunologists encounter on a daily basis. In the youngest children, these diseases are caused by the structure of the respiratory tract, which is shorter and narrower than in an adult, as well as the immaturity of the immune system. Among all children under 5 years of age hospitalized due to respiratory diseases, 20% of cases are acute respiratory infections.
OBJECTIVE
The aim of the study is to discuss selected respiratory diseases in children aged 0-18 years hospitalized at the Pediatric Hospital in Bielsko-Biała.
MATERIAL AND METHODS
In June 2023, statistical data from the Pediatric Hospital was received regarding the number of hospitalized children aged 0-18 in 2015-2022. This article covers the following respiratory diseases: acute laryngitis, acute pharyngitis, pneumonia, bronchitis and bronchiolitis, bronchial asthma, adenoid hypertrophy and palatine tonsil hypertrophy coexisting with adenoid hypertrophy. Then, a table was prepared illustrating the trends of individual disease entities in the discussed time period.
RESULTS
A total of 5,573 hospitalizations were analyzed for the period from 2015-2022. The largest group of children (1,583) were hospitalized due to acute bronchitis and bronchiolitis (28.41%), due to hypertrophy of the adenoid (1,093) and palatine tonsils (1,039), which is 19.6% and 18.64% respectively. The smallest number of children and adolescents were hospitalized due to acute laryngotracheitis (474) and pharyngitis (361), which is 8.51% and 6.47%, respectively, and due to asthma (54), which is 0.97%. It has been observed that from 2017 to 2022 the number of hospitalized patients is constantly increasing due to acute pharyngitis and pneumonia, and from 2018 to 2022 due to acute laryngotracheitis.
CONCLUSIONS
In the analyzed Pediatric Hospital in Bielsko-Biała, the number of hospitalized children (from 0 to 18 years of age) due to pharyngitis, laryngotracheitis and pneumonia increased during the COVID-19 pandemic (2020-2022). The number of hospitalized patients due to pneumonia increased by as many as 70 from 2021 (197) to 2022 (267). In the case of hospitalizations for pharyngitis during the COVID-19 period, the number ranged from 46 in 2019 to 69 in 2022. Also in the case of acute laryngotracheitis in the period 2019-2022, the number of hospitalized young patients increases and ranges from 61 to 76. Respiratory tract infections are an important and common health problem for children. The vast majority of respiratory infections are caused by viruses.
Topics: Humans; Child; Child, Preschool; Infant; Adolescent; Poland; Infant, Newborn; Respiratory Tract Diseases; Hospitalization; Male; Female; Hospitals, Pediatric; Respiratory Tract Infections; Pneumonia; Asthma
PubMed: 38904312
DOI: 10.32394/pe.77.49 -
Head and Neck Pathology Jun 2024MYB RNA in situ hybridization (ISH) has emerged as a reliable and accessible marker to support adenoid cystic carcinoma (ACC) diagnosis, though still not well studied....
BACKGROUND
MYB RNA in situ hybridization (ISH) has emerged as a reliable and accessible marker to support adenoid cystic carcinoma (ACC) diagnosis, though still not well studied. Here, we report our results in a validation and prospective cohort to improve MYB RNA ISH diagnostic accuracy.
METHODS
79 cases (23 retrospective and 56 prospective) underwent MYB RNA ISH testing (44 ACC and 35 non-ACC). MYB RNA ISH results were initially interpreted based on previously established (original) scoring criteria. Weighted "i-scores", percent positive tumor cells, percent tumor cells with large signals (% LS), and staining pattern (abluminal, diffuse, focal non-patterned, or negative) were inputs for logistic regression models. Final model performance characteristics were compared with original scoring criteria and MYB::NFIB FISH results.
RESULTS
An abluminal pattern was characteristic and exclusive to ACC. All i-scores, % LS, and percent positive were significantly higher in ACC. Original scoring criteria yielded a 95.5% sensitivity (Sn), 68.6% specificity (Sp), and 83.5% accuracy. MYB::NFIB FISH yielded a 42.9% sensitivity, 100% specificity, and 60% accuracy. Optimizing for performance, simplicity, and minimal collinearity, our final model was defined as: abluminal pattern and/or % LS > 16.5%, which resulted in a 93.2% Sn, 97.1% Sp, and 94.9% accuracy for ACC diagnosis. False negatives included an ACC with striking tubular eosinophilia and a MYBL1::NFIB translocated ACC. One false positive exclusive to the final model was a nasopharyngeal carcinoma with MYB amplification.
CONCLUSIONS
MYB RNA ISH has a higher Sn than MYB::NFIB FISH while retaining high Sp. Our model provides improvements to specificity compared to original scoring criteria and highlight the importance of abluminal staining pattern and % LS. Nonetheless, alternate fusions remain key false negatives while rare non-ACC with other mechanisms of MYB activation may present as false positives.
Topics: Humans; Carcinoma, Adenoid Cystic; Proto-Oncogene Proteins c-myb; Female; Sensitivity and Specificity; Middle Aged; Male; Aged; Adult; Biomarkers, Tumor; Retrospective Studies; In Situ Hybridization; Prospective Studies; Aged, 80 and over; In Situ Hybridization, Fluorescence; Young Adult
PubMed: 38896376
DOI: 10.1007/s12105-024-01656-z -
Ear, Nose, & Throat Journal Jun 2024This study aimed to describe the experience of a single institution in China in treating adenoid cystic carcinoma of the nasopharynx. We reviewed the previous...
This study aimed to describe the experience of a single institution in China in treating adenoid cystic carcinoma of the nasopharynx. We reviewed the previous literature and conducted a retrospective analysis of 12 patients who diagnosed with nasopharyngeal adenoid cystic carcinoma (NACC) in clinical data, treatment, and follow-up data during 2019 to 2021. Patients ranged in age from 32 to 68 years (mean 40.7 years, median 48.5 years), with a male to female ratio of 5:7. Most of our patients have T4a and T4b diseases (50% and 25%, respectively). A quarter of patients develop distant metastases. Among the 12 patients, 7 of them have positive margins under the microscope (7/12, 58.3%). The chief clinical manifestations were epistaxis, facial swelling, facial pain, headache ear stuffy, and hearing loss. If the tumors involved with cavernous sinus, brain stem infiltrated, and internal carotid artery circumvented, patients will undertake routine enhanced magnetic resonance imaging with Magnetic Resonance Angiography/Magnetic Resonance Venogram (MRA/MRV) to clearly show the lesion region. All patients underwent endoscopic endonasal approach. Fifty percent of patients received radiotherapy and 25% of patients received chemotherapy. None of the patients was lost and the follow-up time ranged from 16 to 45 months. The mean and median follow-up were 2.08 and 1.58 years. Two patients were dead of distant metastasis within 18 and 20 months after the surgery, and another patient with recurrent NACC died of hemorrhage. NACC is a rare malignant tumor that occurs in the nasopharynx, which can grow along the nerve, destroy the bone of the skull base, and metastasize to other organs. Up to now, there is no standard treatment. Our results show that endoscopic sinus surgery is a better choice for advanced or recurrent NACC.
PubMed: 38895961
DOI: 10.1177/01455613241259357 -
Cureus May 2024Adenoid cystic carcinoma (ACC) is a rare type of tumor that usually originates from minor salivary glands in the oral cavity. ACC of the larynx is even rare. This case...
Adenoid cystic carcinoma (ACC) is a rare type of tumor that usually originates from minor salivary glands in the oral cavity. ACC of the larynx is even rare. This case study describes a 36-year-old non-smoking male farmer who initially presented with dyspnea and was misdiagnosed with bronchial asthma. Spirometry revealed fixed airway obstruction. Further evaluation revealed a pedunculated mass obstructing the airway, which was diagnosed as ACC by histopathological examination of the biopsy specimen. The patient was treated with radiation therapy, resulting in clinical improvement after six weeks. ACC is highly invasive and slow-growing, with perineural extension and a higher risk of recurrence. Metastasis in the lungs is common. Adequate preoperative staging, including imaging with computed tomography (CT) and magnetic resonance imaging, is important for planning treatment. The role of radiation therapy with concurrent chemotherapy is still under trial.
PubMed: 38894795
DOI: 10.7759/cureus.60571 -
Cancers Jun 2024Primary tracheobronchial adenoid cystic carcinoma (ACC) is a rare malignancy, so the optimal radiotherapy (RT) dose remains unestablished. We aimed to evaluate the...
Primary tracheobronchial adenoid cystic carcinoma (ACC) is a rare malignancy, so the optimal radiotherapy (RT) dose remains unestablished. We aimed to evaluate the effectiveness of dose-escalated RT for primary tracheobronchial ACC. We retrospectively reviewed 48 patients who had undergone definitive or postoperative RT. Patients classified into the low- and high-dose groups received RT doses <70.0 and ≥70.0 Gy in EQD2, respectively. The primary endpoint was freedom from local progression (FFLP) and overall survival (OS). Throughout the follow-up period, seven patients (14.6%) experienced local progression, while 31 (64.6%) exhibited distant metastasis, most commonly in the lungs. In total, the 5-year FFLP and OS rates were 85.7 and 84.7%, respectively. Multivariate analysis revealed that regional lymph node metastasis at diagnosis and receipt of definitive RT were associated with poorer OS. In the subgroup analysis, the definitive RT group had a 5-year FFLP rate of 33.3 and 78.2% in the low- and high-dose groups ( = 0.065), whereas 5-year OS rates were 66.7 and 79.0%, respectively ( = 0.022). Four patients (8.3%) experienced Grade 3 toxicity with tracheal or main bronchus stenosis. Dose-escalated RT with conventional fractionation may be effective in patients with tracheobronchial ACC, especially for a definitive aim.
PubMed: 38893246
DOI: 10.3390/cancers16112127 -
BMC Cancer Jun 2024Nasopharyngeal adenoid cystic carcinoma (NACC) is a relatively rare salivary gland tumor that is generally associated with poor outcomes. High-dose radiotherapy is a key...
BACKGROUND
Nasopharyngeal adenoid cystic carcinoma (NACC) is a relatively rare salivary gland tumor that is generally associated with poor outcomes. High-dose radiotherapy is a key treatment for patients with NACC. This study reported the long-term efficacy and safety of particle beam radiation therapy (PBRT) for NACC.
METHODS AND MATERIALS
Twenty-six patients with nonmetastatic NACC who received definitive PBRT alone were included in this retrospective study. The majority of patients (92.3%) had locally advanced disease. Twenty-five (96.15%) patients received intensity-modulated proton radiotherapy (IMPT) followed by a carbon ion radiotherapy (CIRT) boost, and one patient received CIRT alone. Overall survival (OS), local control (LC), regional control (RC), and distant metastasis control (DMC) rates were calculated via the Kaplan-Meier method.
RESULTS
The median follow-up time was 46.95 months for the entire cohort. Seven patients experienced local recurrence, and one patient experience neck lymph node recurrence. The 3- and 4-year OS, LC, RC, and DMC rates were 100% and 91.7%, 92.3% and 84.6%, 95.8% and 87.8%, and 90.2% and 71.3%, respectively. A total of 91.3% of the patients achieved complete remission of gross tumors at 1 year after PBRT. Severe acute toxicity was observed in only two patients. A grade 4 decrease in visual acuity was observed in one patient with orbital apex invasion. No late grade 3 or 5 toxicity was observed.
CONCLUSION
Definitive PBRT provided a satisfactory 4-year OS for patients with locally advanced NACC. The toxicity was acceptable and mild. Further follow-up is necessary to confirm the efficacy and safety of definitive PBRT for patients with NACC.
Topics: Humans; Carcinoma, Adenoid Cystic; Male; Female; Middle Aged; Nasopharyngeal Neoplasms; Adult; Retrospective Studies; Treatment Outcome; Aged; Proton Therapy; Radiotherapy, Intensity-Modulated; Young Adult; Follow-Up Studies; Nasopharyngeal Carcinoma; Neoplasm Recurrence, Local; Heavy Ion Radiotherapy
PubMed: 38890585
DOI: 10.1186/s12885-024-12471-8 -
Journal of Thoracic Disease May 2024Primitive tracheal tumors represent a rare entity whose management, when unresectable, remains challenging. Primary aim of this study was to explore the survival and the...
BACKGROUND
Primitive tracheal tumors represent a rare entity whose management, when unresectable, remains challenging. Primary aim of this study was to explore the survival and the factors influencing prognosis of patients with unresectable primitive tracheal tumor undergoing multimodal treatment integrating interventional bronchoscopy and radiotherapy.
METHODS
This retrospective cohort study was conducted at the University Hospital of Modena (Italy) over a 12-year period (January 2010 to January 2022) analyzing patients with unresectable primary tracheal tumor receiving interventional bronchoscopy treatment followed by radiotherapy. Survival analysis was conducted for the whole population and according to histology, development of metastasis, stent placement and the onset of disease relapse. The raw and independent association between potential risk factor and 5-year mortality and the reported complications were investigated.
RESULTS
A total of 12 patients were included. Five-year survival rate was 42% with a median survival time of 26.7 (interquartile range, 4.1-82) months. Survivors showed a higher prevalence of cystic-adenoid histology (80% 14%), while patients who were dead at 5 years were those with a more advanced T (prevalence of T2: 71% 0%) and a lower response to first line treatment (57% 0%). Treatment complications accounted for stent dislocation (33%) and the onset of granuloma (18%), while no major side effects were reported. The presence of cystic-adenoid histology resulted in significantly improved 5-year survival rate (80% 14%). The onset of distal metastasis, the occurrence of disease relapse and the placement of tracheal stent did not result significantly associated with lower survival. Among analysed variables, only the presence of cystic-adenoid histology resulted independently associated with survival (odds ratio =0.1, P=0.04).
CONCLUSIONS
Multimodal treatment including interventional bronchoscopy and associated radiotherapy for unresectable primary tracheal tumors seems not burdened by significant complications and may provide benefits in terms of survival for those patients with cystic-adenoid histology.
PubMed: 38883644
DOI: 10.21037/jtd-23-738 -
Indian Journal of Otolaryngology and... Jun 2024Adenoid hypertrophy (AH) and its sequel like nasal obstruction, obstructive sleep apnoea (OSA), recurrent rhinitis and middle ear disorders are common diseases of...
Adenoid hypertrophy (AH) and its sequel like nasal obstruction, obstructive sleep apnoea (OSA), recurrent rhinitis and middle ear disorders are common diseases of pediatric age group, forming the major bulk of pediatric outpatient visits. The recommended approach to treating OSA in children is through adenotonsillectomy. Adenoidectomy is the surgical procedure of removal of hypertrophied adenoid tissues, which is the most common surgery performed by Ear, Nose, and Throat (ENT) surgeons. Given that adenoidectomy alone might lead to reduced instances of adverse outcomes, decreased risk of complications, and lower expenses, our objective was to explore the viability and suitability of adenoidectomy as a standalone treatment for pediatric OSA and to learn the significant effect of adenoidectomy on the quality of life (QOL) of children. Multicentric prospective study conducted in Department of ENT, Head and Neck Oncosurgery, Smita Memorial Hospital, Thodupuzha & Department of ENT, Head and Neck surgery, MCS Hospital, Muvattupuzha, Kerala, India from June 2022 to June 2023. Children between the age group of 5-10 years, who underwent adenoidectomy during the study period, satisfying the inclusion criteria were subjected for the study. Adenoidectomy was done for them using endoscope assisted coblation technique. Postoperative improvement in symptoms and change in quality of life were analyzed using obstructive sleep disorders-6 (OSD-6) questionnaire at the end of 3 months follow up. The surgeon observed a statistically significant improvement in all domains of OSD-6; and there by improvement in QOL in all patients, who underwent adenoidectomy in a course of three months. Adenoid hypertrophy is the most common cause of nasal obstruction in pediatric population and is creating a significant negative impact on the quality of life of children. Adenoidectomy is a safe and simple procedure which can provide significant improvement in quality of life of kids. Adenoidectomy in isolation could be a valid and recommended surgical choice for selected population of children displaying symptoms of pediatric OSA.
PubMed: 38883488
DOI: 10.1007/s12070-024-04506-0