Pediatric Blood & Cancer最新文献

{"title":"Re-Irradiation for the Progressive Pediatric Diffuse Intrinsic Pontine Glioma: A Report on 109 Children From a Single Center","authors":"Mohamed Saad Zaghloul,&nbsp;Mai K. Bishr,&nbsp;Amal Refaat,&nbsp;Ahmed El Hemaly,&nbsp;Moatssem Al Ayadi,&nbsp;Soha Ahmed,&nbsp;Eslam Maher,&nbsp;Engy S. Todary","doi":"10.1002/pbc.31587","DOIUrl":"10.1002/pbc.31587","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Diffuse intrinsic pontine glioma (DIPG) is a challenging pediatric tumor that frequently progresses within the first year following local radiotherapy. However, several small studies have suggested that re-irradiation may improve quality of life and extend overall survival.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Patients and Methods</h3>\u0000 \u0000 <p>This retrospective study included 109 children who experienced disease progression ≥3 months after their initial radiotherapy, and subsequently received re-irradiation at a single institution. These patients were compared with a cohort of 60 children, meeting the same criteria, who were treated before adopting the re-irradiation policy and received only the best supportive care (BSC). Most of the re-irradiated children (94%) received first radiation dose as hypofractionation (39 Gy/13 fractions).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The re-irradiation group demonstrated significantly higher overall survival (OS) rates after the first progression, with a 6-month OS of 42% (95% CI: 34%–53%) compared to 16% (95% CI: 8.9%–32%) in the BSC group (<i>p</i> &lt; 0.001). Re-irradiation reduced the hazard of death by more than half (HR = 0.45, <i>p</i> &lt; 0.001). Clinical response (<i>p</i> &lt; 0.001) and radiological response (<i>p</i> = 0.016) were significant predictors of improved survival. While the time from initial radiotherapy to progression (<i>p</i> = 0.059) and higher re-irradiation doses (<i>p</i> = 0.074) were associated with improved OS, these factors did not reach statistical significance but may represent potential prognostic indicators.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Re-irradiation improved the OS in children with progression of DIPG and alleviated their signs and symptoms.</p>\u0000 </section>\u0000 </div>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":"72 4","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143189887","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Shared Priorities for Sibling Psychosocial Support in Pediatric Cancer Care: A Value-Weighting Study","authors":"Kathryn A. Davis,&nbsp;Samuel Lai,&nbsp;Melissa A. Alderfer,&nbsp;Kristin A. Long","doi":"10.1002/pbc.31565","DOIUrl":"10.1002/pbc.31565","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Although psychosocial support for siblings of youth with cancer is a standard of care, what sibling supportive services should entail remains unclear. Given limited resources for sibling care, establishing clinical and research priorities may guide resource allocation toward supports perceived as holding the greatest potential benefit. The current study used a two-round, value-weighting approach to identify priorities for sibling support services.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Procedure</h3>\u0000 \u0000 <p>Participants were recruited from a group of sibling experts (clinicians, researchers, community program leaders, and adults who had a sibling with childhood cancer) invited to attend an international sibling summit. In Round 1, 27 participants provided feedback on a list of potential priorities for sibling psychosocial support. In Round 2, 30 participants completed a web-based value-weighting questionnaire indicating how they would allocate 100 units of hypothetical funding among various priorities and qualitatively described the rationale for their decisions.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Funding allocations generally averaged out across participants, highlighting the need for investments across all domains of sibling support. Participants allocated the greatest proportion of hypothetical funding to community-based sibling supports, which they perceived as more accessible to siblings than hospital-based supports. Participants allocated a particularly high level of funding to sibling supports in local schools. Within sibling subpopulations, bereaved siblings, siblings during active cancer treatment, and siblings with more adverse social determinants of health were allocated the largest proportion of funds.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Sibling-focused researchers, clinicians, program leaders, and adult siblings endorse broad investments in sibling support. Investments in community-based supports particularly may improve access to sibling support services.</p>\u0000 </section>\u0000 </div>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":"72 4","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143080700","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Screening Cognition, Sleep, and Physical Activity in Pediatric Oncology Long-Term Follow-Up Care","authors":"Kaitlin A. Oswald-McCloskey,&nbsp;Nicole Kubinec,&nbsp;Kimberley Heinrich","doi":"10.1002/pbc.31579","DOIUrl":"10.1002/pbc.31579","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Children treated for pediatric cancer are at risk for cognitive late effects, as well as impairments in sleep and physical activity. The aim of the present study was to examine the psychometric properties of clinical screening of child- and caregiver-reported cognitive functioning, sleep, and physical activity and the relationship between cognitive functioning and health behaviors within a pediatric oncology long-term follow-up clinic.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Procedure</h3>\u0000 \u0000 <p>The study included a retrospective chart review of 99 caregivers and 80 children (8–17 years old) who completed the Conners Short Form (parent only) and PROMIS cognitive functioning, sleep-related impairment, sleep disturbance, and physical activity scales at the child's annual long-term follow-up visit. Test statistics and <i>T</i>-tests were used to assess psychometrics of the PROMIS scales. Bivariate correlations were used to examine the relationship between cognitive function and health behaviors.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The child- and parent-report, short-version PROMIS cognitive functioning, sleep, and physical activity scales demonstrated high internal consistency and inter-rater reliability. High convergent validity was observed between PROMIS cognitive functioning and Conners Short Form. Caregiver- and child-reported cognitive functioning and health behaviors were significantly related (<i>p</i> ≤ 0.042).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>The PROMIS short-version scales are reliable and valid measures for screening cognitive function and health behaviors in pediatric oncology long-term follow-up care. Further research examining the predictive validity and longitudinal utility of the PROMIS scales in survivors is warranted. There was a positive association between cognitive functioning and health behaviors in survivors, warranting further investigation to inform potential targets of intervention in long-term follow-up care.</p>\u0000 </section>\u0000 </div>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":"72 4","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/pbc.31579","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143123255","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ho Preso Un Granchio: The Sitcom Created by Young Patients That Turns the Cancer Journey Into Comedy.","authors":"Andrea Ferrari, Matteo Silva, Alice Patriccioli, Elena Pagani Bagliacca, Stefano Signoroni, Maura Massimino","doi":"10.1002/pbc.31582","DOIUrl":"https://doi.org/10.1002/pbc.31582","url":null,"abstract":"","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":" ","pages":"e31582"},"PeriodicalIF":2.4,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143123246","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"COVID-19 Vaccine Response in Pediatric Oncology Patients","authors":"Brittni Kam,&nbsp;Yiwen Wang,&nbsp;FeiFei Qin,&nbsp;Adrienne H. Long,&nbsp;Orly R. Klein,&nbsp;Catherine Aftandilian","doi":"10.1002/pbc.31572","DOIUrl":"10.1002/pbc.31572","url":null,"abstract":"<div>\u0000 \u0000 <p>Pediatric oncology and hematopoietic stem cell transplant (HSCT) patients may fail to mount an appropriate immune response to COVID-19 vaccinations. The immunologic response to initial SARS-CoV-2 vaccination was studied in 93 pediatric patients with hematologic malignancy, solid tumors, or post-HSCT. The majority of patients who were not receiving chemotherapy showed a positive humoral and cell-mediated response to COVID-19 vaccination. In contrast, variable responses to vaccination were seen in patients receiving chemotherapy. Patients with a normal lymphocyte count showed higher rates of seroconversion than their lymphocytopenic counterparts. This report details pediatric oncology and HSCT patients’ immunologic responses to COVID-19 vaccination during all phases of treatment.</p>\u0000 </div>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":"72 4","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143123322","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hydroxyurea Therapy and Sleep-Disordered Breathing in Children With Sickle Cell Disease","authors":"Zachary Abramson,&nbsp;Ayobami Olanrewaju,&nbsp;Guolian Kang,&nbsp;Yunusa Olufadi,&nbsp;Pei-Lin Chen,&nbsp;Parul Rai,&nbsp;Andrew M. Heitzer,&nbsp;Clifford M. Takemoto,&nbsp;Ahsan Bashir,&nbsp;Nour Akil,&nbsp;Jane S. Hankins","doi":"10.1002/pbc.31531","DOIUrl":"10.1002/pbc.31531","url":null,"abstract":"<div>\u0000 \u0000 <p>Bidirectional relationships exist among sickle cell disease (SCD), sleep-disordered breathing (SDB), and hydroxyurea therapy, and this complex interplay poses challenges to research attempting to elucidate causal mechanisms and determine therapeutic targets. We therefore set out to study all three components of this clinical triad simultaneously (SCD, SDB, and hydroxyurea therapy), documenting correlations and suggesting underlying pathophysiological mechanisms. Hydroxyurea therapy was associated with improved SDB and with decreased inflammation, implicating the anti-inflammation effects of hydroxyurea as a plausible mechanism driving these correlations. These results underscore the need for longitudinal studies to elucidate causal relationships.</p>\u0000 </div>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":"72 4","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143080697","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Streamlining Diagnosis: Reducing Time for Brain Tumor Detection in Children of an Upper-Middle-Income Country","authors":"Natália Dassi,&nbsp;Andrea Maria Cappellano,&nbsp;Fabiola Isabel Suano de Souza,&nbsp;Rosana Fiorini Puccini,&nbsp;Nasjla Saba da Silva,&nbsp;Maria Wany Louzada Strufaldi","doi":"10.1002/pbc.31574","DOIUrl":"10.1002/pbc.31574","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Delays in diagnosing pediatric brain tumors are often associated with limited awareness of the signs and symptoms by parents and healthcare professionals, as well as the absence of routine childcare follow-ups and delays in healthcare system referrals.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Aims</h3>\u0000 \u0000 <p>To explore opportunities for reducing diagnostic delays, the study assessed the knowledge of pediatric brain tumor signs and symptoms, routine follow-up care for children, use of the Child Health Book (CHB), and referral intervals of a suspected case to a specialized center.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Procedure</h3>\u0000 \u0000 <p>This cross-sectional study collected data through interviews and virtual questionnaires.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Between August and November 2023, 200 parents (pediatric and oncology departments) and 147 healthcare professionals (primary and tertiary care) participated. Except for headaches and seizures, the rates of parental recognition of warning signs were below 70%. Physicians in tertiary care demonstrated greater recognition of these warning signs than those in primary care (<i>p</i> = 0.011). Recognition rates among nurses were below 75%. Primary and tertiary care professionals reported referral intervals &gt;1 month in 10%–15% cases. Children routine follow-up care was reported in both levels. Over 75% of all participants reported that the CHB could be a useful tool for educating about childhood cancer.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Our study provides essential insights to improve the early diagnosis of pediatric brain tumors. The findings emphasize the need to strengthen pediatric care follow-ups and use of CHB by parents and healthcare professionals to raise awareness of warning signs and symptoms, along with a flowchart for timely and accurate referrals to specialized centers.</p>\u0000 </section>\u0000 </div>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":"72 4","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143080718","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Clinical Impact of Somatic Copy Number Variations in Patients With Stage IV Wilms Tumor Enrolled in the SIOP 2001 Trial and Study","authors":"Nils Welter,&nbsp;Reem Al-Saadi,&nbsp;Robinson Gravier-Dumonceau,&nbsp;Rhoikos Furtwängler,&nbsp;Norbert Graf,&nbsp;Jenny Wegert,&nbsp;Manfred Gessler,&nbsp;Richard D. Williams,&nbsp;Kathy Pritchard-Jones,&nbsp;Aurore Coulomb-L'Hermine,&nbsp;Marry M. van den Heuvel-Eibrink,&nbsp;Arnauld C. Verschuur","doi":"10.1002/pbc.31580","DOIUrl":"10.1002/pbc.31580","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Recent research elucidated the prognostic significance of molecular biology in Wilms tumor (WT) by linking somatic genomic variants (such as gain of chromosome 1q) to unfavorable patient outcomes. This analysis describes the clinical impact of copy number variations (CNV) in tumor samples of WT patients with stage IV disease.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Tumor samples of 55 WT patients with stage IV disease from the United Kingdom, France, and Germany enrolled in the SIOP 2001 study and treated with preoperative chemotherapy (pCHT) were examined for their CNVs of chromosome 1q and other regions of interest using multiplex ligation-dependent probe amplification (MLPA). The identified CNV were analyzed regarding their prognostic impact.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Chromosome 1q gain (1q+) and <i>TP53</i> loss occurred in 38.2% and 16.4% of tumors and were associated with older patient age at diagnosis (median [months]: 65 and 64 vs. 49 each, <i>p</i> = 0.03 and 0.02, respectively) and poorer 5-year event-free survival (40.0% and 11.1% vs. 67.7% and 82.6%, <i>p</i> = 0.04 and &lt;0.01, respectively) compared to their specific control group of tumors without the respective CNV. In patients with pulmonary-only metastasis, 1q+ was an adverse prognostic marker irrespective of remission status after pCHT with or without metastasectomy. A simultaneous <i>MYCN</i> gain occurred more frequently in tumors with 1q+ than in tumors without 1q+ (<i>p</i> = 0.03). <i>TP53</i> loss was linked to high-risk histology and inferior 5-year overall survival (<i>p</i> &lt; 0.001).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>We confirm the prognostic relevance of 1q+ and <i>TP53</i> loss in stage IV WTs and emphasize their potential utility for future treatment stratification.</p>\u0000 </section>\u0000 </div>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":"72 4","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/pbc.31580","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143080731","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cervical Lymph Node Invasion in Pediatric Salivary Gland Malignancies: Clinical Overview and Therapeutic Implications","authors":"Celine Richard,&nbsp;Matthieu Carton,&nbsp;Inbal Hazkani,&nbsp;Vincent Couloigner,&nbsp;Anthony Sheyn,&nbsp;Jeffrey Rastatter,&nbsp;Linda Haroun,&nbsp;Sara Helmig,&nbsp;Mary Beth Houston,&nbsp;Sylvie Helfre,&nbsp;Eric Thebault,&nbsp;Nicolas Andre,&nbsp;Cecile Faure Conter,&nbsp;Natacha Teissier,&nbsp;Brice Fresneau,&nbsp;Daniel Orbach","doi":"10.1002/pbc.31581","DOIUrl":"10.1002/pbc.31581","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Pediatric salivary gland malignancies (SGM) present challenges in managing cervical nodes. We aimed to characterize lymph node invasion to inform decisions regarding the need of systematic wide lymph node dissection (WLND).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>International retrospective study, conducted across seven large French and American pediatric centers, including pediatric patients (0–18 years) diagnosed with SGM from 2000 to 2020.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among the 82 patients (median age 13 years), the parotid gland was frequently affected (60 cases). Histotypes comprised mucoepidermoid (mucoepidermoid carcinoma [MEC], 43 cases), acinic cells (acinic cells carcinoma [AcCC], 22 cases), adenoid cystic (adenoid cystic carcinoma [AdCC], 8 cases), (MASC, 6 cases), and adenocarcinoma (3 cases). Primary treatments were surgery (82 cases) and radiotherapy (20 cases; median dosage 64 gray). Cervical nodes therapy included WLND (≥2 levels, 29 cases), limited nodes resection (LNR; one level, 13 cases), and/or irradiation (4 cases; median 54 gray; range 52.0–63.0). At diagnosis, six patients had cervical node invasion (CNI) managed with LNR (four cases), WLND (two cases), and radiotherapy (three cases). After a median follow-up of 6 years (range 1–22), nine patients had tumor event: local (four cases), cervical relapse/progression (three cases) or combined (two cases). Of the nine with CNI, at diagnosis or relapse, four had MASC. Five-year event-free and overall survival (OS) rates were, respectively, 90.1% and 98.8%.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>CNI is rare in pediatric SGM but noted in 11% of cases, with higher incidence in MASC. Overall, outcome in SGM is good with a tailored locoregional multidisciplinary approach. Systematic lymph node dissection should be reconsidered.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> SUMMARY</h3>\u0000 \u0000 <p>This international multi-institutional study analyzed the clinical presentation and the cervical pattern of relapse of 82 pediatric patients with newly diagnosed salivary gland malignancies. Overall, nodal invasion was rare at diagnosis and only noted in 7%. In addition, 6% developed nodal relapse during follow-up. Incidence of nodal spread was frequent in mammary analogue secretory carcinoma (MASC). The overall outcome was promising with a tailored locoregional multidisciplinary approach. Systematic lymph node dissection should be reconsidered in pediatric salivary gland tumors.</p>\u0000 </section>\u0000 </div>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":"72 4","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143080695","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical and Genetic Characteristics of Pediatric Colorectal Cancer","authors":"Qiyang Shen,&nbsp;Yong Zhou,&nbsp;Xingyu Liu,&nbsp;Jian Li,&nbsp;Sirui Pan,&nbsp;Nan Xie,&nbsp;Xinrong Lin,&nbsp;Li Zhou,&nbsp;Jianfeng Zhou,&nbsp;Tao Li","doi":"10.1002/pbc.31569","DOIUrl":"10.1002/pbc.31569","url":null,"abstract":"&lt;div&gt;\u0000 \u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Background&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Compared to colorectal cancer (CRC) in adults, CRC in children is extremely rare. Although its incidence has increased recently, there is a lack of clinical research on the disease. Inherited cancer susceptibility syndromes (ICSS), a group of disorders in which patients are predisposed to susceptibility to a wide range of tumors as a result of pathogenic mutations in genes in their germ line, are an important cause of CRC in children. Delayed diagnosis due to atypical clinical presentation, as well as limited awareness of ICSS among doctors, contributes to poor outcomes in juvenile CRC patients. Therefore, improving clinicians’ understanding of the diagnosis and treatment of the disease is crucial to enhancing children's prognosis with CRC.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Methods&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Clinical data and laboratory reports were collected from eight pediatric patients diagnosed with CRC at the Children's Hospital of Nanjing Medical University between 2020 and 2023. The clinical and genetic characteristics of these patients were evaluated and compared with other patients with early-onset CRC in the literature.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Results&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;A total of 8 children with CRC were enrolled in the study, including 5 male and 3 female children, with a median age of 140 (73–177) months. The main clinical manifestations were unexplained abdominal pain, abdominal distension, vomiting, and hematochezia. Three cases of intestinal obstruction and two cases of intestinal intussusception occurred among the patients. All eight children underwent surgical treatment, including one case of snare resection of rectal polyp, five cases of subtotal colectomy, and two cases of radical resection of CRC. One case of radical resection of CRC utilized laparoscopic and colonoscopic combined resection guided by indocyanine green (ICG) fluorescence navigation system. Postoperative combination of pathological pictures and immunohistochemical (IHC) staining results confirmed high-grade squamous intraepithelial lesion (HSIL) in Case 1, and mucinous adenocarcinoma in the remaining seven cases. Out of eight pediatric patients with CRC, except for Case 1 and Case 7, who did not undergo chemotherapy, the remaining six patients all received postoperative chemotherapy; among them, the patients in Cases 1, 6, 7, and 8 achieved complete remission, whereas the patients in Cases 2 and 4 died due to postoperative recurrence and distant metastasis, the patient in Case 3 is still undergoing chemotherapy, and the patient in Case 5 was lost to follow-up after surgery. The results of the genetic test report showed that two children had ICSS caused by mismatch gene repair system defects (deficient MMR, dMMR);","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":"72 4","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143067112","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}