Lungs Of Adults Research Articles

Case Report: Giant lung hamartoma : An usual cause of lobectomy in a five-year child

Pulmonary hamartomas are the most common benign tumors of the lung in adults. They are usually asymptomatic because of their small size and their slow-growing character. We report the case of a 5-year-old child presenting with a giant lung mass causing recurrent right pneumonia. Surgical resection with middle lobectomy was performed. Final histology revealed pulmonary hamartoma with predominant adenofibromatous and lipomatous differentiation.

Case Report: Giant lung hamartoma : An usual cause of lobectomy in a five-year child

Pulmonary hamartomas are the most common benign tumors of the lung in adults. They are usually asymptomatic because of their small size and their slow-growing character. We report the case of a 5-year-old child presenting with a giant lung mass causing recurrent right pneumonia. Surgical resection with middle lobectomy was performed. Final histology revealed pulmonary hamartoma with predominant adenofibromatous and lipomatous differentiation.

Thoracoscopic surgery for rare congenital pulmonary airway malformations in adults: a decade of retrospective study.

Congenital pulmonary airway malformation (CPAM) is a rare benign deformity of the lungs in adults. Our study aimed to evaluate the clinical features and compare the effect of thoracoscopic lobectomy and wedge resection for adult CPAMs. This was a retrospective study including eighteen adults with CPAMs recruited between 2013 and 2023. Radiological scans and pulmonary function test (PFT) were performed before operation. All the patients were treated with thoracoscopic approach, which were categorized into groups of lobectomy and wedge resection. The baseline, preoperative, and operative data were evaluated and analyzed. Four males and fourteen females were diagnosed with CPAMs at a median age of 57.5 years. Cough was the main symptom, reported by 55.6% of the patients. CPAMs were always initially misdiagnosed as other conditions due to heterogeneous computed tomography (CT) characteristics. The mean of PFT results showed normal (>80% predicted) in forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), and FEV1 to FVC ratio (FEV1/FVC), but less than 70% predicted in forced expiratory flow (FEF) at 25-75%, 50% and 75% of FVC. All patients underwent video-assisted thoracic surgery (VATS) with a total of nine wedge resections and nine lobectomies. Age at surgery varied statistically between the two groups. Whereas, duration of surgery, blood loss, postoperative drainage, days of drainage, days of hospitalization and postoperative complications showed no statistical difference between the two groups. There were 27.8% of the lesions showing CPAMs mixed with other diseases during histological evaluations. CPAM in adults showed a complex presentation in terms of clinical symptoms, imaging performance and pathological findings. Half of the patients were detected with small airway dysfunction preoperatively. Thoracoscopic lobectomy and wedge resection for the treatment can achieve satisfactory short-term outcomes.

Prospective bidirectional relations between depression and metabolic health: 30-year follow-up from the National Heart, Lung, and Blood Institute (NHLBI) Coronary Artery Disease in Young Adults (CARDIA) study.

This study investigated prospective bidirectional relationships between depressive symptoms and metabolic syndrome (MetS) and the moderating effects of race, sex, and health behaviors in a diverse cohort followed for 30 years. Data were analyzed from the National Heart, Lung, and Blood Institute (NHLBI) Coronary Artery Disease in Young Adults (CARDIA) study, a 30-year prospective study of young adults (N = 5,113; Mage = 24.76 [SD = 3.63] at baseline; 45% male) who were tested every 5 years between 1985 and 2015. Measures included biological assessments of MetS components and self-reported depressive symptoms based on the Center for Epidemiologic Studies Depression (CESD) scale. Data analyses included bidirectional general estimating equations analyses of time-lagged associations between depressive symptoms and MetS. There was a consistent, bidirectional relationship between depressive symptoms and MetS over time. Individuals with more CESD depressive symptoms were more likely to develop MetS over time compared to those reporting fewer symptoms, Wald χ²(1) = 7.09, p < .008, and MetS was similarly predictive of CESD. MetS more consistently predicted CESD scores at each 5-year exam than CESD predicted MetS. Race and sex moderated these relationships, with White females, White individuals overall, and females overall demonstrating significant relationships between CESD depressive symptoms and MetS. Health behaviors were not related to associations between CESD and MetS. In a diverse young adult population prospectively followed into late middle age, MetS more consistently predicted depressive symptoms over time than depressive symptoms predicted MetS. The relation between MetS and depressive symptoms was moderated by race and sex, but not health behaviors. (PsycInfo Database Record (c) 2024 APA, all rights reserved).

Clinicopathological and genetic characteristics of congenital cystic adenomatoid malformation of lung and its associated lung cancer in adults

Objective: To investigate the clinicopathological features and genetic characteristics of congenital cystic adenomatoid malformation (CCAM) of lung and CCAM associated lung cancer in adults. Methods: A total of 13 cases of CCAM of lung in adults, diagnosed from June 2015 to May 2023, were collected from the Department of Pathology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, China. Their histopathological features were correlated with probable development into lung cancer. Next-generation sequencing was performed on the benign and malignant areas of all cases. Results: The pathological classification of all cases were of CCAM of lung type 1. There were 4 male and 9 female cases, age ranged from 18 to 65 years, with a mean age of 41 years. Six cases were accompanied by lung cancer, all of them were mucinous adenocarcinoma. Next-generation sequencing showed no gene mutation in 2 of the 13 cases; KRAS mutations in exon 2 were detected in 7 cases, in which there were 6 cases complicated with lung mucinous adenocarcinoma and no matter in the malignant or benign regions, the same case exhibited the same mutation sites in KRAS gene. Conclusions: CCAM of the lung is a congenital disease, and in adults, type 1 is most commonly found in the pathological classification, and it is often accompanied by cancer. Gene mutations are frequently detected in CCAM of the lung, KRAS being the most recurrent mutation which may play an important role in the carcinogenesis.

Supplemental Material for Prospective Bidirectional Relations Between Depression and Metabolic Health: 30-Year Follow-Up From the National Heart, Lung, and Blood Institute (NHLBI) Coronary Artery Disease in Young Adults (CARDIA) Study

Supplemental Material for Prospective Bidirectional Relations Between Depression and Metabolic Health: 30-Year Follow-Up From the National Heart, Lung, and Blood Institute (NHLBI) Coronary Artery Disease in Young Adults (CARDIA) Study

National and subnational trends in cancer burden in China, 2005–20: an analysis of national mortality surveillance data

Cancer has been the leading cause of death since 2010 in China, with increasing incidence, mortality, and burden. We aimed to assess national and subnational changes in the cancer burden from 2005 to 2020 in China using data from the National Mortality Surveillance System. We extracted data on cancer-related deaths from the National Mortality Surveillance System, which accounts for 24·3% of the country's population with national and provincial representativeness. Data for the surveillance population stratified by age and sex were extracted from the National Bureau of Statistics of China. We estimated mortality and years of life lost (YLLs) for all cancers and for 23 cancer groups by age and sex, nationally, and for 31 provinces in China between 2005 and 2020. We calculated age-standardised mortality and YLL rates using the China 2020 census as the reference population. Average annual percent changes in age-standardised rates for mortality and YLLs were calculated to assess trends over the study period. Decomposition analysis was used to assess the drivers of changes in cancer-related death due to three explanatory components: population growth, population ageing, and age-specific mortality rates in China. The total number of cancer-related deaths increased by 21·6% to 2 397 772 and YLLs increased by 5·0% to 56 598 975 between 2005 and 2020. The three leading fatal cancer types remained stable for both sexes over the study period: tracheal, bronchus, and lung cancer; liver cancer; and stomach cancer. The fourth and fifth leading cancers also remained stable among males (oesophageal, and colon and rectum), while colon and rectum cancer replaced oesophageal cancer as the fourth and breast cancer replaced colon and rectum cancer as the fifth leading cause of cancer-related death among females. Age-standardised mortality rates and age-standardised YLL rates for almost all cancer types (except for prostate for male and multiple myeloma for female) decreased significantly in both sexes in urban areas. Age-standardised YLL rates increased for about half of all cancers for both sexes in rural areas. Leading fatal types were leukaemia and brain and nervous system cancer in younger groups (aged 0-19 years); liver, tracheal, bronchus, and lung, or breast cancers in middle-aged groups (aged 40-59 years); and tracheal, bronchus, and lung, liver, or stomach cancers in older adults (aged ≥60 years) in 2020. The leading causes of cancer-related mortality varied for each province, with tracheal, bronchus, and lung or liver cancer at the top in 30 provinces. The cancer burden in China appeared to be shifting towards that in high-income countries from 2005 to 2020. Adjustments to existing health plans and actions are needed to reduce the burdens of tracheal, bronchus, and lung cancer or other leading and emerging cancers. National Key Research and Development Program of China.

Diagnostic yield of endobronchial ultrasound and virtual CT navigational bronchoscopy for biopsy of pulmonary nodules, mediastinal lymph nodes, and thoracic tumors in children.

Endobronchial ultrasound-guided transbronchial biopsy and needle aspiration (EBUS-TBB/EBUS-TBNA) are first line investigative modalities for lung and mediastinal pathology in adults. We aimed to characterize and assess the diagnostic yield of EBUS and virtual CT navigation guided biopsies in children. This single center, retrospective cohort study included patients who underwent radial or linear EBUS procedures (+/- CT navigation) for biopsy of mediastinal lymph nodes, tumors, and pulmonary nodules. Demographic, procedural, and outcome were collected. Sixty procedures were performed in 56 patients aged 2-22 years of age between January 2015 and May 2023. The most common indications for biopsy were pulmonary nodules (45%) and hilar/mediastinal lymphadenopathy (33%). For cases in which a final diagnosis was ascertained by any means, the diagnostic yield for linear EBUS (mediastinal pathology) was 76% and the diagnostic yield from radial EBUS (pulmonary nodules and lung masses) was 85%. The most common diagnoses were infection (45%), malignancy (17%), and sarcoidosis (11%). Among patients in whom infection was the final diagnosis, a total of 31 pathogens were identified. Eighteen were identified on bronchoalveolar lavage and an additional 14 pathogens identified on EBUS-TBB, representing an increase of 77% (p < .005). The sensitivity, specificity, negative and positive predictive values for malignancy detection were 73%, 100%, 94%, and 100%, respectively. EBUS-TBB/TBNA is a safe and effective way to diagnose lung and mediastinal pathology in children. Pediatric interventional pulmonology is a growing field offering minimally-invasive diagnostic opportunities for children in whom more invasive procedures were previously the only option.

Abstract 11061: Incidence of Cancer in Hospitalized Adults With Congenital Heart Disease

Introduction: Many patients with congenital heart disease (CHD) are repeatedly exposed to low-dose ionizing radiation for diagnosis, treatment and long-term management of their care. Hypothesis: We hypothesized that these repeated exposures put CHD patients at higher risk for radiation-associated cancers (RAC). Methods: Review of Vizient (a national, administrative database) from 12/2018 - 12/2021 for admissions aged 18-85 years with ICD-10 codes for moderate or severe CHD and RAC (bladder, breast, colon, esophageal, leukemia/myeloma, liver, lung, ovarian and stomach). Demographics, hospital outcomes and costs were compared to admissions with RAC and no CHD (NO-CHD). Results: Out of 34,793 CHD admissions, 6.4% had RAC, and out of 24,999,735 NO-CHD admissions, 11.2% had RAC (p&lt;0.001; Table 1). CHD with RAC were younger, had longer length of stay, higher complications, in-hospital mortality and costs. Comparisons for each RAC type are in Table 2. There were higher rates of breast, liver and lung cancer in CHD (p&lt;0.05 for all) and higher costs for all CHD RAC except esophageal. Conclusions: This national database review shows increased incidence of some types of RAC (lung, liver and breast) in adults with CHD, with worse hospital outcomes and higher costs for several cancer types. While frequent radiation in the CHD population may contribute to these findings, it will be important to explore further. These findings also highlight the importance of adherence to the “as low as reasonably achievable” (ALARA) principle in the care of children and adults with CHD and stresses the importance of imaging modalities that do not use ionizing radiation.

Long-Term Health Effects of Curative Therapies on Heart, Lungs, and Kidneys for Individuals with Sickle Cell Disease Compared to Those with Hematologic Malignancies.

The goal of curing children and adults with sickle cell disease (SCD) is to maximize benefits and minimize intermediate and long-term adverse outcomes so that individuals can live an average life span with a high quality of life. While greater than 2000 individuals with SCD have been treated with curative therapy, systematic studies have not been performed to evaluate the long-term health effects of hematopoietic stem cell transplant (HSCT) in this population. Individuals with SCD suffer progressive heart, lung, and kidney disease prior to curative therapy. In adults, these sequalae are associated with earlier death. In comparison, individuals who undergo HSCT for cancer are heavily pretreated with chemotherapy, resulting in potential acute and chronic heart, lung, and kidney disease. The long-term health effects on the heart, lung, and kidney for children and adults undergoing HSCT for cancer have been extensively investigated. These studies provide the best available data to extrapolate the possible late health effects after curative therapy for SCD. Future research is needed to evaluate whether HSCT abates, stabilizes, or exacerbates heart, lung, kidney, and other diseases in children and adults with SCD receiving myeloablative and non-myeloablative conditioning regimens for curative therapy.

The Aging Human Lung Mucosa: A Proteomics Study.

The older adult population, estimated to double by 2050, is at increased risk of respiratory infections and other pulmonary diseases. Biochemical changes in the lung alveolar lining fluid (ALF) and in alveolar compartment cells can alter local immune responses as we age, generating opportunities for invading pathogens to establish successful infections. Indeed, the lung alveolar space of older adults is a pro-inflammatory, pro-oxidative, dysregulated environment that remains understudied. We performed an exploratory, quantitative proteomic profiling of the soluble proteins present in ALF, developing insight into molecular fingerprints, pathways, and regulatory networks that characterize the alveolar space in old age, comparing it to that of younger individuals. We identified 457 proteins that were significantly differentially expressed in older adult ALF, including increased production of matrix metalloproteinases, markers of cellular senescence, antimicrobials, and proteins of neutrophilic granule origin, among others, suggesting that neutrophils in the lungs of older adults could be potential contributors to the dysregulated alveolar environment with increasing age. Finally, we describe a hypothetical regulatory network mediated by the serum response factor that could explain the neutrophilic profile observed in the older adult population.

A Comprehensive Evaluation of Health-Related Life Quality Assessment Through Head and Neck, Prostate, Breast, Lung, and Skin Cancer in Adults.

Health assessment data assists the well-being and patient care teams' process in drawing up a care and assistance plan and comprehending the requirements of the patient. Comprehensive and precise data about the Quality of Life of cancer patients play a significant part in the development and organization of cancer patient care. Quality of Life has been used to mean a variety of various things, such as health situation, physical function, symptoms, psychosocial modification, well-being, enjoyment of life, and happiness. Chronic diseases such as cancer are among the disorders that severely affect people's health and consequently their Quality of Life. Cancer patients experience a range of symptoms, including pain and various physical and mental conditions that negatively affect their Quality of Life. In this article, we examined cancer and the impact that this disease can have on the Quality of Life of cancer patients. The cancers examined in this article include head and neck, prostate, breast, lung, and skin cancers. We also discussed health assessment and the importance and purpose of studying patients' Quality of Life, especially cancer patients. The various signs and symptoms of the disease that affect the Quality of Life of patients were also reviewed.

Bivalirudin exerts antiviral activity against respiratory syncytial virus-induced lung infections in neonatal mice.

Respiratory syncytial virus (RSV) is the most common cause of small airways inflammation in the lungs (bronchiolitis) in neonates and immunocompromised adults. The deregulation of cellular and plasma components leads to increased morbidity and mortality. The activation of the clotting cascade plays a key role in the progression of disease severity during viral infection. The current investigation studied the effect of bivalirudin (BR) on the progression and cellular effects of RSV-induced infection in the neonatal mice model. Mice (5-7 days old) were inoculated intranasally with RSV with or without BR administration (2 mg kg-1 day-1, i.v.) for 2 weeks. Tissue histopathology, inflammatory signalling genes such as TLR, and cytokines were analyzed. The results showed pneumocytes exhibiting nuclear pyknosis, cellular infiltration in lung tissue and increased lung titers in RSV-infected mice compared to the control. Furthermore, RSV-infected mice demonstrated altered clotting parameters such as D-dimer, soluble thrombomodulin, and increased inflammatory cytokines IL-5, 6, IFN-γ, IL-13, and CXCL1. Additionally, the mRNA expression analysis displayed increased levels of IL-33, TLR3, and TLR7 genes in RSV-infected lung tissue. Further, to delineate the role of micro RNAs, the qRT-PCR analysis was done, and the results displayed an increase in miR-136, miR-30b, and let-7i. At the same time, the down-regulated expression of miR-221 in RSV-infected mice compared to the control. BR treatment reduced the cellular infiltration with reduced inflammatory cytokines and normalized clotting indices. Thus, the study shows that RSV infection induces specific changes in lung tissue and the clotting related signalling mechanism. Additionally, BR treatment significantly reduces bronchiolitis and prevents the severity of the infections suggesting that BR can possibly be used to reduce the viral-mediated infections in neonates.

Lung and Mediastinal Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration in Young Adults

Introduction: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is used to investigate pulmonary nodules, mediastinal lymphadenopathy, and mediastinal masses in both malignant and nonmalignant etiologies. EBUS-TBNA is most commonly used in the diagnosis and staging of patients with non-small-cell lung cancer in the middle-age and elderly populations. As lung cancer is uncommon in young adults, it is assumed that there are a distinct disease population and clinical background in young adults who undergo EBUS-TBNA. However, this population has not been well investigated. Methods: We identified all EBUS-TBNA cases in young adults (aged 18–39 years) between January 1, 2008, and December 31, 2018, at our institution. Cytology diagnoses were correlated with the concurrent/subsequent histologic diagnosis and clinical decisions. A final patient classification was created based on the worst cytologic or histologic diagnosis (benign, low-grade, or malignant), with the exception of atypical cytology with subsequent long clinical follow-up with no evidence of malignancy, who were considered benign. All discordant cases and positive/suspicious cases with available slides were rereviewed together by the authors to confirm the diagnosis. Results: In total, 257 EBUS-TBNA procedures were performed in 249 young adults (mean age of 31.2 years). The majority of indications were lymphadenopathy and lung nodule/mass. Final cytologic interpretations included 214 (83%) benign, 14 (5%) atypical, 5 (2%) low-grade neoplasm (carcinoid tumor), and 15 (6%) malignant cases. The final patient classification was 213 (86%) benign, 6 (2%) low-grade, and 30 (12%) malignant. Discordant results were found in 24 cases, most frequently due to sampling error (50%). Of 213 benign cases, 58% had granulomatous disease, with sarcoidosis being the most common, followed by histoplasmosis. Of 30 cases with a final malignant classification, metastatic tumor was the most common (n = 12, 4.8%), followed by primary lung tumor (n = 11, 4.4%) and lymphoma (n = 7, 2.8%). There was a variety of malignancies among primary lung cancer, including adenocarcinoma (n = 5), squamous-cell carcinoma (n = 3), inflammatory myofibroblastic tumor (n = 2), and epithelioid hemangioendothelioma (n = 1). Conclusion: In young adults, EBUS-TBNA was most frequently performed to evaluate lymphadenopathy and lung nodules, and granulomatous disease was the most common benign finding. Although rare, primary lung malignancies do occur in young adults along with metastasis from a variety of other sites, with sarcoma being the most common pathology.

Possible Use of Tuberculous Recombinant Allergen Test in the Differential Diagnosis of Diseases in the Upper Lung Lobes in Adults

The objective of the study: to evaluate the possible use of the subcutaneous test with tuberculous recombinant allergen (TRA) in the differential diagnosis of pulmonary tuberculosis localized in the upper lobes.Subjects and Methods. Data of examinations with subcutaneous TRA and Mantoux tests with 2 units in 648 patients aged 18 to 90 years with various lung pathologies and HIV-negative status and 122 healthy individuals were retrospectively analyzed. Among these subjects, the following diseases were diagnosed: tuberculosis, pneumonia, cancer, post-tuberculosis changes, and sclerotic changes after nonspecific diseases.Results. The sensitivity of TRA test in adults with tuberculous lesions in the upper lobes was 59.87%; the rate of negative responses in those without active tuberculosis was 76.0%; the sensitivity of Mantoux test with 2 units was higher and made 83.6%, but the rate of negative responses in those without tuberculosis was lower – 44.1%. TRA test is most important in the differential diagnosis of tuberculosis and pneumonia in which positive and hyperergic reactions are observed in 59.87 and 13.95% of cases, respectively.The comparative analysis of the rates for tuberculosis and post-tuberculosis changes showed no statistically significant difference in both Mantoux test (83.6 and 78.4%, respectively, p &gt; 0.05) and TRA test (59.87 and 50.77%, respectively, p &gt; 0.05). This indicates that these tests cannot be used to assess the activity of tuberculosis localized in the upper lobes of the lungs in adults.

Aerosol Particle Transport and Deposition in Upper and Lower Airways of Infant, Child and Adult Human Lungs

Understanding transportation and deposition (TD) of aerosol particles in the human respiratory system can help clinical treatment of lung diseases using medicines. The lung airway diameters and the breathing capacity of human lungs normally increase with age until the age of 30. Many studies have analyzed the particle TD in the human lung airways. However, the knowledge of the nanoparticle TD in airways of infants and children with varying inhalation flow rates is still limited in the literature. This study investigates nanoparticle (5 nm ≤ dp ≤ 500 nm) TD in the lungs of infants, children, and adults. The inhalation air flow rates corresponding to three ages are considered as Qin=3.22 L/min (infant), 8.09 L/min (Child), and Qin=14 L/min (adult). It is found that less particles are deposited in upper lung airways (G0–G3) than in lower airways (G12–G15) in the lungs of all the three age groups. The results suggest that the particle deposition efficiency in lung airways increases with the decrease of particle size due to the Brownian diffusion mechanism. About 3% of 500 nm particles are deposited in airways G12–G15 for the three age groups. As the particle size is decreased to 5 nm, the deposition rate in G12–G15 is increased to over 95%. The present findings can help medical therapy by individually simulating the distribution of drug-aerosol for the patient-specific lung.

To the doctrine of syphilis of the lungs. F. M. Abramovich (Doctor. Case, 1926, No. 12-13)

According to FM Abramovich (Doctor. Delo, 1926, No. 12-13), syphilitic lesions of the lungs in adults are not as rare as it is usually thought, and they can occur in two forms: more rarely, in the form of gummy lesions, more often in the form of interstitial pneumonia with peribronchitis and perivasculitis.

Social Media and mHealth Technology for Cancer Screening: Systematic Review and Meta-analysis.

BackgroundCancer is a leading cause of death, and although screening can reduce cancer morbidity and mortality, participation in screening remains suboptimal.ObjectiveThis systematic review and meta-analysis aims to evaluate the effectiveness of social media and mobile health (mHealth) interventions for cancer screening.MethodsWe searched for randomized controlled trials and quasi-experimental studies of social media and mHealth interventions promoting cancer screening (breast, cervical, colorectal, lung, and prostate cancers) in adults in MEDLINE, Embase, PsycINFO, Scopus, CINAHL, Cochrane Central Register of Controlled Trials, and Communication & Mass Media Complete from January 1, 2000, to July 17, 2020. Two independent reviewers screened the titles, abstracts, and full-text articles and completed the risk of bias assessments. We pooled odds ratios for screening participation using the Mantel-Haenszel method in a random-effects model.ResultsWe screened 18,008 records identifying 39 studies (35 mHealth and 4 social media). The types of interventions included peer support (n=1), education or awareness (n=6), reminders (n=13), or mixed (n=19). The overall pooled odds ratio was 1.49 (95% CI 1.31-1.70), with similar effect sizes across cancer types.ConclusionsScreening programs should consider mHealth interventions because of their promising role in promoting cancer screening participation. Given the limited number of studies identified, further research is needed for social media interventions.Trial RegistrationPROSPERO International Prospective Register of Systematic Reviews CRD42019139615; https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=139615International Registered Report Identifier (IRRID)RR2-10.1136/bmjopen-2019-035411

RARE-20. A RARE CASE OF A PRIMARY CENTRAL NERVOUS SYSTEM NEUROENDOCRINE CARCINOMA AND SUCCESSFULL THERAPY IN A FIVE-YEAR-OLD CHILD

Neuroendocrine tumors (NETs) are rare neoplasms predominantly arising in the GI-tract or the lungs of adults. To date, only ten cases of primary CNS NETs have been reported with just three of them describing a neuroendocrine carcinoma (NEC) in patients aged 34–77 years and none occurring in a child. We report on a previously healthy 5-year-old boy, who presented with headaches, nausea and vomiting and was diagnosed with a left cerebellar solid mass with a cystic component, radiologically suggestive of a pilocytic astrocytoma. After gross-total resection, histological analysis revealed an epithelial tumor growing in a nest-like pattern with a very high mitotic frequency, staining positive for CK8, CK18 and CK19. Chromogranin A and synaptophysin expression indicated a neuroendocrine differentiation. Molecular analysis of the tumor tissue revealed a KRAS- splice-site mutation (c451-3C>T). After extensive search for an extracranial primary, including Ga-68 DOTANOC-PET-CT, the diagnosis of a primary CNS NEC was made, and proton irradiation was performed. However, the patient developed an in-field recurrence just five weeks after the end of radiotherapy. The tumor was re-resected en-bloc, showing vital tumor tissue, demonstrating its aggressiveness. Chemotherapy consisting of etoposide, cisplatin and ifosfamide was initiated. After two cycles chemotherapy was continued with etoposide and carboplatin for another four cycles. The patient remains disease free one year after the end of relapse-treatment, supporting the beneficial effect of platinum- and etoposide-based chemotherapy for this tumor entity. Physical exam revealed a sagittal synostosis with a mild dolichocephaly. Interestingly, the KRAS-mutation was discovered to be a maternal germline mutation, previously described as likely benign. However, alterations of the RAS/MAPK pathway have been described in NECs and in craniosynostosis cases. It remains to be elucidated whether the KRAS-mutation is merely a variant of uncertain significance or might have been implicated in the development of this exceptional tumor.

Initial reporting from the prospective National Cancer Institute (NCI) COVID-19 in Cancer Patients Study (NCCAPS).

6565 Background: Patients (pts) with cancer are at increased risk of SARS-CoV-2 infection and severe COVID-19 disease. Longitudinal follow-up is needed to characterize the severity, sequelae and outcomes in pts with cancer who develop COVID-19. Methods: NCCAPS is a prospective, longitudinal study (NCT04387656) aiming to accrue 2,000 pts with cancer undergoing active treatment or prior stem cell transplant for hematologic or solid tumor malignancy. Adult patients are eligible to enroll within 14 days of their first positive SARS-CoV-2 test; pediatric patients may also enroll retrospectively. Clinical data, patient-reported outcomes, blood specimens, and imaging are collected for up to 2 years. This abstract provides initial baseline and 2-month follow-up data. Results: As of Jan 22, 2021, 585 pts (552 adults and 33 pediatric pts) had complete baseline data and of these pts, 215 adults had 2 months of complete follow-up data. 23.4% of adults and 42.4% of pediatric pts were of non-White race and/or Hispanic/Latinx ethnicity. The most common cancer diagnoses were breast (19.6%), lung (9.9%) and multiple myeloma (8.9%) in adults and acute leukemia (AML/ALL; 63.6%) in children. The most recent treatment was chemotherapy in 38.2%, immunotherapy in 9.6%, and radiation in 5.4%. Median time from positive SARS-CoV-2 test to study enrollment was 10.5 days in adults and 18 days in pediatric pts. Preliminary analysis of plasma cytokines will be presented. At enrollment, 84.6% of adults had COVID-19 symptoms. 55.9% reported symptoms 2 weeks after their positive SARS-CoV-2 test; this fell to 39.0% at 1 month and 28.8% at 2 months (see Table). Of the 215 adults with complete data at 2 months, sequelae included pulmonary (n=22, 10%), cardiovascular (n=12, 6%) thromboembolic (n=9, 4%), bleeding (n=9, 4%) and gastrointestinal (n=11, 5%). 144 (67%) reported at least one cancer treatment disruption in the first 2 months, most commonly delayed therapy (n=98; 46%).Of the 348 adults with baseline data and SARS-CoV-2 test date prior to Nov 23, 2020, 6.3% had died (median time from SARS-CoV-2 test to death: 27 days), and 22.1% reported at least one hospitalization for COVID-19. No deaths were reported in the pediatric population. Conclusion: Cancer pts with COVID-19 report ongoing symptoms after acute infection and a substantial number develop sequelae. Cancer treatment disruptions are common in the initial months following SARS-CoV-2 infection. Longer follow-up will inform whether these treatment disruptions are associated with adverse outcomes. Clinical trial information: NCT04387656. [Table: see text]