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1
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Farina Junior MA, Utz-Melere M, da Silva CS, Nader LS, Trein CS, Lucchese AM, Machry M, Mariano R, Ferreira CT, Kalil AN, Feier FH. Ten years of a pediatric living donor liver transplantation program in Brazil. World J Transplant 2025; 15:98616. [DOI: 10.5500/wjt.v15.i2.98616] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/01/2024] [Revised: 11/15/2024] [Accepted: 12/11/2024] [Indexed: 02/21/2025] Open
Abstract
BACKGROUND Pediatric living-donor liver transplantation is considered a safe alternative for the treatment of children with end-stage liver disease. Experienced tertiary centers and specialized medical staff are necessary to ensure compatible long-term survival rates and quality-of-life for these children.
AIM To report the results and the 10-year learning curve of a pediatric living-donor liver transplantation program.
METHODS We conducted a retrospective cohort study of pediatric recipients from 2013 to 2023. Post-transplant outcomes and patient survival rates were compared between two 5-year periods of the program.
RESULTS A total of 25 and 48 patients underwent transplantation in the first (2013-2017) and second period (2018-2023), respectively. Portal vein and hepatic artery thrombosis occurred in 11 (15.1%) and seven (9.6%) patients, respectively. Biliary complications were observed in 39 of 73 patients (53.4%). A lower warm ischemia time was observed in the second period compared to the first (32.6 ± 8.6 minutes vs 38.4 ± 9.8 minutes, P = 0.018, respectively). Patient survival rates at 1 and 5 years were 84% in the first period and 91.7% in the second period, with no significant difference (P = 0.32).
CONCLUSION The reported indications and outcomes align with the current literature. Our findings provide crucial evidence regarding the feasibility of establishing a living donor program with consistent results over time.
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Affiliation(s)
- Marco Aurélio Farina Junior
- Department of Hepatology and Liver Transplantation, Santa Casa de Porto Alegre, Porto Alegre 90050-170, Brazil
| | - Melina Utz-Melere
- Department of Hepatology and Liver Transplantation, Santa Casa de Porto Alegre, Porto Alegre 90050-170, Brazil
| | - Carolina Soares da Silva
- Department of Hepatology and Liver Transplantation, Santa Casa de Porto Alegre, Porto Alegre 90050-170, Brazil
| | - Luiza Salgado Nader
- Department of Hepatology and Liver Transplantation, Santa Casa de Porto Alegre, Porto Alegre 90050-170, Brazil
| | - Cristine Suzana Trein
- Department of Hepatology and Liver Transplantation, Santa Casa de Porto Alegre, Porto Alegre 90050-170, Brazil
| | - Angelica Maria Lucchese
- Department of Hepato-Biliary-Pancreatic Surgery and Liver Transplantation, Irmandade Santa Casa de Misericórdia de Porto Alegre, Porto Alegre 90020-090, Brazil
| | - Mayara Machry
- Department of Hepato-Biliary-Pancreatic Surgery and Liver Transplantation, Irmandade Santa Casa de Misericórdia de Porto Alegre, Porto Alegre 90020-090, Brazil
| | - Rodrigo Mariano
- Department of Hepato-Biliary-Pancreatic Surgery and Liver Transplantation, Irmandade Santa Casa de Misericórdia de Porto Alegre, Porto Alegre 90020-090, Brazil
| | - Cristina Targa Ferreira
- Department of Hepatology and Liver Transplantation, Santa Casa de Porto Alegre, Porto Alegre 90050-170, Brazil
| | - Antônio Nocchi Kalil
- Department of Hepato-Biliary-Pancreatic Surgery and Liver Transplantation, Irmandade Santa Casa de Misericórdia de Porto Alegre, Porto Alegre 90020-090, Brazil
- Department of Surgical Oncology, Santa Rita Hospital/Santa Casa de Misericórdia de Porto Alegre, Porto Alegre 90050-170, Rio Grande do Sul, Brazil
| | - Flávia Heinz Feier
- Department of Hepato-Biliary-Pancreatic Surgery and Liver Transplantation, Irmandade Santa Casa de Misericórdia de Porto Alegre, Porto Alegre 90020-090, Brazil
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2
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Wen Y, Chen C, Zhou T, Liu C, Zhang Z, Gu G. Single-cell analysis unveils immune dysregulation and EBV-driven lymphoproliferative disorder in pediatric liver transplant recipients. Hum Immunol 2025:111309. [PMID: 40263002 DOI: 10.1016/j.humimm.2025.111309] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/24/2025] [Revised: 03/29/2025] [Accepted: 04/05/2025] [Indexed: 04/24/2025]
Abstract
Post-transplant lymphoproliferative disorder (PTLD) is a severe complication following liver transplantation, particularly in pediatric patients, with a high incidence rate associated with Epstein-Barr virus (EBV) infection. The immunological microenvironment of PTLD, particularly the cellular and molecular changes associated with EBV infection, remains unclear. Using scRNA-seq, we examined changes in the immune microenvironment of PBMC from pediatric liver transplant recipients across four groups: PTLD patients with EBV infection, EBV-positive non-PTLD transplant recipients, EBV-negative transplant recipients, and healthy children. PTLD patients exhibited profound immune dysregulation, marked by weakened cytotoxicity in NK cells, reduced B cell differentiation and activation, and an inflammatory shift in myeloid cells. EBV infection primarily targeted memory B cells and plasma cells in PTLD patients, with uninfected memory B cells showing impaired functional potential. Furthermore, CD8+ T cells in PTLD were characterized by increased exhaustion and low cytotoxic activity. In addition, regulatory T cells in PTLD displayed enhanced suppressive functions. Our findings present an in-depth view of the immune landscape in PTLD, identifying key immune cell alterations associated with EBV infection and PTLD development. These insights could inform the development of targeted therapies aimed at restoring immune function and controlling EBV-driven lymphoproliferation in pediatric liver transplant recipients.
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Affiliation(s)
- Yanling Wen
- Institute for Hepatology, National Clinical Research Center for Infectious Disease, Shenzhen Third People's Hospital, The Second Affiliated Hospital, School of Medicine, Southern University of Science and Technology, Shenzhen, China
| | - Chen Chen
- Department of Liver Surgery, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China
| | - Tao Zhou
- Department of Liver Surgery, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China
| | - Chao Liu
- Department of Liver Transplantation, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China.
| | - Zheng Zhang
- Institute for Hepatology, National Clinical Research Center for Infectious Disease, Shenzhen Third People's Hospital, The Second Affiliated Hospital, School of Medicine, Southern University of Science and Technology, Shenzhen, China.
| | - Guangxiang Gu
- Department of Liver Surgery, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China; Department of Liver Transplantation, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China.
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3
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Gao L, Hoke A, Jackson J, Petrauskas L, Mason EF, Sarma A, Friedman D, Bearl D, Dulek D, Wootten C, Park J. Multisite Head and Neck Pediatric Posttransplant Lymphoproliferative Disorder: A Case Report. Clin Case Rep 2025; 13:e70200. [PMID: 39959555 PMCID: PMC11828662 DOI: 10.1002/ccr3.70200] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/03/2024] [Revised: 01/16/2025] [Accepted: 01/31/2025] [Indexed: 02/18/2025] Open
Abstract
Posttransplant lymphoproliferative disorder (PTLD) can mimic infectious processes in the head and neck. A high index of suspicion for PTLD must be maintained in pediatric transplant patients, and minimal response to antibiotics/corticosteroids should prompt timely biopsy of suspicious tissue to expedite the diagnosis and treatment of PTLD.
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Affiliation(s)
- Lily Gao
- Vanderbilt University School of MedicineNashvilleTennesseeUSA
| | - Austin Hoke
- Department of Otolaryngology‐Head & Neck SurgeryVanderbilt University Medical CenterNashvilleTennesseeUSA
| | - Jarrett Jackson
- Department of Otolaryngology‐Head & Neck SurgeryVanderbilt University Medical CenterNashvilleTennesseeUSA
| | - Laura Petrauskas
- Department of Otolaryngology‐Head & Neck SurgeryVanderbilt University Medical CenterNashvilleTennesseeUSA
| | - Emily F. Mason
- Department of Pathology, Microbiology and ImmunologyVanderbilt University Medical CenterNashvilleTennesseeUSA
| | - Asha Sarma
- Department of RadiologyVanderbilt University Medical CenterNashvilleTennesseeUSA
| | - Debra Friedman
- Department of Pediatrics, Hematology and OncologyVanderbilt Children's HospitalNashvilleTennesseeUSA
| | - David Bearl
- Department of Pediatrics, CardiologyVanderbilt Children's HospitalNashvilleTennesseeUSA
| | - Daniel Dulek
- Department of Pediatrics, Infectious DiseasesVanderbilt Children's HospitalNashvilleTennesseeUSA
| | - Christopher Wootten
- Department of Otolaryngology‐Head & Neck SurgeryVanderbilt University Medical CenterNashvilleTennesseeUSA
| | - Jason Park
- Department of Otolaryngology‐Head & Neck SurgeryVanderbilt University Medical CenterNashvilleTennesseeUSA
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4
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Ellis J, Grammatikopoulos T, Cook J, Deep A. Respiratory problems associated with liver disease in children. Breathe (Sheff) 2024; 20:230150. [PMID: 38595937 PMCID: PMC11003522 DOI: 10.1183/20734735.0150-2023] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/29/2023] [Accepted: 03/05/2024] [Indexed: 04/11/2024] Open
Abstract
Respiratory manifestations of chronic liver disease have a profound impact on patient clinical outcomes. Certain conditions within paediatric liver disease have an associated respiratory pathology. This overlap between liver and respiratory manifestations can result in complex challenges when managing patients and requires clinicians to be able to recognise when referral to specialists is required. While liver transplantation is at the centre of treatment, it opens up further potential for respiratory complications. It is established that these complications place patients at risk of longer stays in hospital and reduced survival. Additionally, late post-transplant complications can occur, including post-transplant lymphoproliferative disease and immunosuppression-induced interstitial lung disease. Although rare, it is important for clinicians to recognise these conditions to allow for prompt management. Finally, as liver disease progresses in children, respiratory complications can occur. Hepatopulmonary syndrome can occur in the context of portal hypertension, resulting in increased mortality and poorer quality of life for patients. Another consequence is portopulmonary hypertension, which can be associated with poor survival. Failure to recognise these complications in children may result in poorer outcomes and therefore it is vital that clinicians can establish when referral to a paediatric respiratory medicine specialist is required.
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Affiliation(s)
- Jordache Ellis
- Paediatric Liver, GI and Nutrition Centre and MowatLabs, King's College Hospital, London, UK
| | - Tassos Grammatikopoulos
- Paediatric Liver, GI and Nutrition Centre and MowatLabs, King's College Hospital, London, UK
- Institute of Liver Studies, King's College London, London, UK
| | - James Cook
- Department of Paediatric Respiratory Medicine, King's College Hospital, London, UK
| | - Akash Deep
- Paediatric Intensive Care Unit, King's College Hospital, London, UK
- Department of Women and Children's Health, King's College London, London, UK
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5
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Janeela AM, Fouzia NA, Zachariah UG. Post-transplantation Lymphoproliferative Disorder (PTLD): In the Liver Transplant Recipient. J Clin Exp Hepatol 2024; 14:101286. [PMID: 38076446 PMCID: PMC10709502 DOI: 10.1016/j.jceh.2023.09.007] [Citation(s) in RCA: 2] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/28/2023] [Accepted: 09/14/2023] [Indexed: 03/03/2025] Open
Abstract
Post- transplantation lymphoproliferative disorders (PTLD) are uncommon neoplasms that complicate the post transplantation period. The incidence of PTLD and outcome post liver transplantation is sparsely described. Children who undergo liver transplantation are at higher risk of PTLD than adults. Risk factors for PTLD include the level of immunosuppression and Epstein-Barr virus status. Immunosuppression in post-transplant patients can cause uncontrolled expansion of B cells. The diagnosis requires high degree of clinical suspicion, radiological evaluation, and tissue biopsy. Risk reduction depends mainly on decreasing patients' exposure to aggressive immunosuppressive regimens and is the initial step in management. Rituximab with or without chemotherapy is the mainstay of treatment. In refractory or persistent disease, alternative treatment options like adoptive immunotherapy and autologous stem cell transplant have been explored. Prognosis is determined by clonality of the PTLD and severity of the disease.
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Chan SS, Coblentz A, Bhatia A, Kaste SC, Mhlanga J, Parisi MT, Thacker P, Voss SD, Weidman EK, Siegel MJ. Imaging of pediatric hematopoietic stem cell transplant recipients: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper. Pediatr Blood Cancer 2023; 70 Suppl 4:e30013. [PMID: 36546505 PMCID: PMC10644273 DOI: 10.1002/pbc.30013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/01/2022] [Revised: 09/28/2022] [Accepted: 09/05/2022] [Indexed: 12/24/2022]
Abstract
Imaging in hematopoietic stem cell transplantation patients is not targeted at evaluating the transplant per se. Rather, imaging is largely confined to evaluating peri-procedural and post-procedural complications. Alternatively, imaging may be performed to establish a baseline study for comparison should the patient develop certain post-procedural complications. This article looks to describe the various imaging modalities available with recommendations for which imaging study should be performed in specific complications. We also provide select imaging protocols for different indications and modalities for the purpose of establishing a set minimal standard for imaging in these complex patients.
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Affiliation(s)
- Sherwin S Chan
- Department of Radiology, Children’s Mercy Kansas City, Kansas City, MO; Department of Radiology, University of Missouri at Kansas City School of Medicine, Kansas City, MO
| | - Ailish Coblentz
- Department of Diagnostic Imaging, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
| | - Aashim Bhatia
- Department of Radiology, Division of Neuroradiology Children’s Hospital of Philadelphia, Philadelphia, PA
| | - Sue C. Kaste
- Department of Diagnostic Imaging, St. Jude Children’s Research Hospital, Memphis, TN
| | - Joyce Mhlanga
- Department of Radiology, Edward Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis, MO
| | - Marguerite T. Parisi
- Departments of Radiology and Pediatrics, University of Washington School of Medicine and Seattle Children’s Hospital, Seattle, WA
| | | | - Stephan D. Voss
- Department of Radiology, Boston Children’s Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA. 02115
| | - Elizabeth K. Weidman
- Department of Radiology, Weill Cornell Medicine – New York Presbyterian Hospital, New York, NY
| | - Marilyn J Siegel
- Department of Diagnostic Imaging, St. Jude Children’s Research Hospital, Memphis, TN
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7
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Ruijter BN, Wolterbeek R, Hew M, van Reeven M, van der Helm D, Dubbeld J, Tushuizen ME, Metselaar H, Vossen ACTM, van Hoek B. Epstein-Barr Viral Load Monitoring Strategy and the Risk for Posttransplant Lymphoproliferative Disease in Adult Liver Transplantation : A Cohort Study. Ann Intern Med 2023; 176:174-181. [PMID: 36645888 DOI: 10.7326/m22-0364] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/17/2023] Open
Abstract
BACKGROUND Primary infection with or reactivation of Epstein-Barr virus (EBV) can occur after liver transplant (LT) and can lead to posttransplant lymphoproliferative disease (PTLD). In pediatric LT, an EBV-DNA viral load (EBV VL) monitoring strategy, including the reduction of immunosuppression, has led to a lower incidence of PTLD. For adult LT recipients with less primary infection and more EBV reactivation, it is unknown whether this strategy is effective. OBJECTIVE To examine the effect of an EBV VL monitoring strategy on the incidence of PTLD after LT in adults. DESIGN Cohort study. SETTING Two university medical centers in the Netherlands. PATIENTS Adult recipients of first LT in Leiden between September 2003 and January 2017 with an EBV VL monitoring strategy formed the monitoring group (M1), recipients of first LT in Rotterdam between January 2003 and January 2017 without such a strategy formed the contemporary control group (C1), and those who had transplants in Leiden between September 1992 and September 2003 or Rotterdam between 1986 and January 2003 formed the historical control groups (M0 and C0, respectively). MEASUREMENTS Influence of EBV VL monitoring on incidence of PTLD. RESULTS After inverse probability of treatment weighting of the 4 groups to achieve a balance among the groups for important patient characteristics, differences within hospitals between the historical and recent era in cumulative incidences-expressed as the number of events per 1000 patients measured at 5-, 10-, and 15-year follow-up-showed fewer events in the contemporary era in both centers. This difference was considerably larger in the monitoring center, whereas the 95% CI included the null value of 0 for point estimates. LIMITATION Retrospective, low statistical power, and incompletely balanced groups, and non-EBV PTLD cannot be prevented. CONCLUSION Monitoring EBV VL may reduce PTLD incidence after LT in adults; larger studies are warranted. PRIMARY FUNDING SOURCE None.
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Affiliation(s)
- Bastian N Ruijter
- Department of Gastroenterology and Hepatology, LUMC Transplant Center, Leiden University Medical Center, Leiden, the Netherlands (B.N.R., M.H., M.E.T., B.v.H.)
| | - Ron Wolterbeek
- Department of Biomedical Data Sciences, Medical Statistics, Leiden University Medical Center, Leiden, the Netherlands (R.W.)
| | - Mitchell Hew
- Department of Gastroenterology and Hepatology, LUMC Transplant Center, Leiden University Medical Center, Leiden, the Netherlands (B.N.R., M.H., M.E.T., B.v.H.)
| | - Marjolein van Reeven
- Department of Surgery, Erasmus MC Transplant Institute, Erasmus Medical Center, Rotterdam, the Netherlands (M.v.R.)
| | - Danny van der Helm
- Department of Gastroenterology and Hepatology and Department of Surgery, LUMC Transplant Center, Leiden University Medical Center, Leiden, the Netherlands (D.v.d.H.)
| | - Jeroen Dubbeld
- Department of Surgery, LUMC Transplant Center, Leiden University Medical Center, Leiden, the Netherlands (J.D.)
| | - Maarten E Tushuizen
- Department of Gastroenterology and Hepatology, LUMC Transplant Center, Leiden University Medical Center, Leiden, the Netherlands (B.N.R., M.H., M.E.T., B.v.H.)
| | - Herold Metselaar
- Department of Gastroenterology and Hepatology, Erasmus MC Transplant Institute, Erasmus Medical Center, Rotterdam, the Netherlands (H.M.)
| | - Ann C T M Vossen
- Department of Medical Microbiology, Leiden University Medical Center, Leiden, the Netherlands (A.C.T.M.V.)
| | - Bart van Hoek
- Department of Gastroenterology and Hepatology, LUMC Transplant Center, Leiden University Medical Center, Leiden, the Netherlands (B.N.R., M.H., M.E.T., B.v.H.)
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8
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Guja KE, Nadel H, Iagaru A. Overview and Recent Advances in 18F-FDG PET/CT for Evaluation of Pediatric Lymphoma. Semin Nucl Med 2022. [DOI: 10.1053/j.semnuclmed.2022.10.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
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9
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Marie E, Navallas M, Katz DS, Farajirad E, Punnett A, Davda S, Shammas A, Oudjhane K, Vali R. Non-Hodgkin Lymphoma Imaging Spectrum in Children, Adolescents, and Young Adults. Radiographics 2022; 42:1214-1238. [PMID: 35714040 DOI: 10.1148/rg.210162] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
In children, adolescents, and young adults (CAYA), non-Hodgkin lymphoma (NHL) is characterized by various age-related dissimilarities in tumor aggressiveness, prevailing pathologic subtypes, and imaging features, as well as potentially different treatment outcomes. Understanding the imaging spectrum of NHL in CAYA with particular attention to children and adolescents is critical for radiologists to support the clinical decision making by the treating physicians and other health care practitioners. The authors discuss the currently performed imaging modalities including radiography, US, CT, MRI, and PET in the diagnosis, staging, and assessment of the treatment response. Familiarity with diagnostic imaging challenges during image acquisition, processing, and interpretation is required when managing patients with NHL. The authors describe potentially problematic and life-threatening scenarios that require prompt management. Moreover, the authors address the unprecedented urge to understand the imaging patterns of possible treatment-related complications of the therapeutic agents used in NHL clinical trials and in practice. Online supplemental material is available for this article. ©RSNA, 2022.
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Affiliation(s)
- Eman Marie
- From the Department of Diagnostic Imaging, McMaster Children's Hospital, McMaster University, 1200 Main St W, Hamilton, ON, Canada L8N 3Z5 (E.M.); Department of Diagnostic Imaging, Hospital Universitario 12 de Octubre, Madrid, Spain (M.N.); Department of Radiology, NYU Winthrop Hospital, Mineola, NY (D.S.K.); LHSC Victoria Hospital, Western Ontario University, London, ON, Canada (E.F.); Department of Pediatrics, Division of Hematology/Oncology (A.P.), Department of Diagnostic Imaging (K.O), Division of Nuclear Medicine (A.S., R.V.), The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada; Great Ormond Street Hospital for Children, NHS, London, England (S.D.); and Department of Medical Imaging, University of Toronto, Toronto, ON, Canada (K.O.)
| | - María Navallas
- From the Department of Diagnostic Imaging, McMaster Children's Hospital, McMaster University, 1200 Main St W, Hamilton, ON, Canada L8N 3Z5 (E.M.); Department of Diagnostic Imaging, Hospital Universitario 12 de Octubre, Madrid, Spain (M.N.); Department of Radiology, NYU Winthrop Hospital, Mineola, NY (D.S.K.); LHSC Victoria Hospital, Western Ontario University, London, ON, Canada (E.F.); Department of Pediatrics, Division of Hematology/Oncology (A.P.), Department of Diagnostic Imaging (K.O), Division of Nuclear Medicine (A.S., R.V.), The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada; Great Ormond Street Hospital for Children, NHS, London, England (S.D.); and Department of Medical Imaging, University of Toronto, Toronto, ON, Canada (K.O.)
| | - Douglas S Katz
- From the Department of Diagnostic Imaging, McMaster Children's Hospital, McMaster University, 1200 Main St W, Hamilton, ON, Canada L8N 3Z5 (E.M.); Department of Diagnostic Imaging, Hospital Universitario 12 de Octubre, Madrid, Spain (M.N.); Department of Radiology, NYU Winthrop Hospital, Mineola, NY (D.S.K.); LHSC Victoria Hospital, Western Ontario University, London, ON, Canada (E.F.); Department of Pediatrics, Division of Hematology/Oncology (A.P.), Department of Diagnostic Imaging (K.O), Division of Nuclear Medicine (A.S., R.V.), The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada; Great Ormond Street Hospital for Children, NHS, London, England (S.D.); and Department of Medical Imaging, University of Toronto, Toronto, ON, Canada (K.O.)
| | - Elnaz Farajirad
- From the Department of Diagnostic Imaging, McMaster Children's Hospital, McMaster University, 1200 Main St W, Hamilton, ON, Canada L8N 3Z5 (E.M.); Department of Diagnostic Imaging, Hospital Universitario 12 de Octubre, Madrid, Spain (M.N.); Department of Radiology, NYU Winthrop Hospital, Mineola, NY (D.S.K.); LHSC Victoria Hospital, Western Ontario University, London, ON, Canada (E.F.); Department of Pediatrics, Division of Hematology/Oncology (A.P.), Department of Diagnostic Imaging (K.O), Division of Nuclear Medicine (A.S., R.V.), The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada; Great Ormond Street Hospital for Children, NHS, London, England (S.D.); and Department of Medical Imaging, University of Toronto, Toronto, ON, Canada (K.O.)
| | - Angela Punnett
- From the Department of Diagnostic Imaging, McMaster Children's Hospital, McMaster University, 1200 Main St W, Hamilton, ON, Canada L8N 3Z5 (E.M.); Department of Diagnostic Imaging, Hospital Universitario 12 de Octubre, Madrid, Spain (M.N.); Department of Radiology, NYU Winthrop Hospital, Mineola, NY (D.S.K.); LHSC Victoria Hospital, Western Ontario University, London, ON, Canada (E.F.); Department of Pediatrics, Division of Hematology/Oncology (A.P.), Department of Diagnostic Imaging (K.O), Division of Nuclear Medicine (A.S., R.V.), The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada; Great Ormond Street Hospital for Children, NHS, London, England (S.D.); and Department of Medical Imaging, University of Toronto, Toronto, ON, Canada (K.O.)
| | - Sunit Davda
- From the Department of Diagnostic Imaging, McMaster Children's Hospital, McMaster University, 1200 Main St W, Hamilton, ON, Canada L8N 3Z5 (E.M.); Department of Diagnostic Imaging, Hospital Universitario 12 de Octubre, Madrid, Spain (M.N.); Department of Radiology, NYU Winthrop Hospital, Mineola, NY (D.S.K.); LHSC Victoria Hospital, Western Ontario University, London, ON, Canada (E.F.); Department of Pediatrics, Division of Hematology/Oncology (A.P.), Department of Diagnostic Imaging (K.O), Division of Nuclear Medicine (A.S., R.V.), The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada; Great Ormond Street Hospital for Children, NHS, London, England (S.D.); and Department of Medical Imaging, University of Toronto, Toronto, ON, Canada (K.O.)
| | - Amer Shammas
- From the Department of Diagnostic Imaging, McMaster Children's Hospital, McMaster University, 1200 Main St W, Hamilton, ON, Canada L8N 3Z5 (E.M.); Department of Diagnostic Imaging, Hospital Universitario 12 de Octubre, Madrid, Spain (M.N.); Department of Radiology, NYU Winthrop Hospital, Mineola, NY (D.S.K.); LHSC Victoria Hospital, Western Ontario University, London, ON, Canada (E.F.); Department of Pediatrics, Division of Hematology/Oncology (A.P.), Department of Diagnostic Imaging (K.O), Division of Nuclear Medicine (A.S., R.V.), The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada; Great Ormond Street Hospital for Children, NHS, London, England (S.D.); and Department of Medical Imaging, University of Toronto, Toronto, ON, Canada (K.O.)
| | - Kamaldine Oudjhane
- From the Department of Diagnostic Imaging, McMaster Children's Hospital, McMaster University, 1200 Main St W, Hamilton, ON, Canada L8N 3Z5 (E.M.); Department of Diagnostic Imaging, Hospital Universitario 12 de Octubre, Madrid, Spain (M.N.); Department of Radiology, NYU Winthrop Hospital, Mineola, NY (D.S.K.); LHSC Victoria Hospital, Western Ontario University, London, ON, Canada (E.F.); Department of Pediatrics, Division of Hematology/Oncology (A.P.), Department of Diagnostic Imaging (K.O), Division of Nuclear Medicine (A.S., R.V.), The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada; Great Ormond Street Hospital for Children, NHS, London, England (S.D.); and Department of Medical Imaging, University of Toronto, Toronto, ON, Canada (K.O.)
| | - Reza Vali
- From the Department of Diagnostic Imaging, McMaster Children's Hospital, McMaster University, 1200 Main St W, Hamilton, ON, Canada L8N 3Z5 (E.M.); Department of Diagnostic Imaging, Hospital Universitario 12 de Octubre, Madrid, Spain (M.N.); Department of Radiology, NYU Winthrop Hospital, Mineola, NY (D.S.K.); LHSC Victoria Hospital, Western Ontario University, London, ON, Canada (E.F.); Department of Pediatrics, Division of Hematology/Oncology (A.P.), Department of Diagnostic Imaging (K.O), Division of Nuclear Medicine (A.S., R.V.), The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada; Great Ormond Street Hospital for Children, NHS, London, England (S.D.); and Department of Medical Imaging, University of Toronto, Toronto, ON, Canada (K.O.)
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10
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Update of pediatric soft tissue tumors with review of conventional MRI appearance-part 2: vascular lesions, fibrohistiocytic tumors, muscle tumors, peripheral nerve sheath tumors, tumors of uncertain differentiation, and undifferentiated small round cell sarcomas. Skeletal Radiol 2022; 51:701-725. [PMID: 34297167 DOI: 10.1007/s00256-021-03837-1] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/17/2020] [Revised: 05/22/2021] [Accepted: 06/02/2021] [Indexed: 02/02/2023]
Abstract
There are numerous soft tissue tumors and tumor-like conditions in the pediatric population. Magnetic resonance imaging is the most useful modality for imaging these lesions. Although certain soft tissue lesions exhibit magnetic resonance features characteristic of a specific diagnosis, most lesions are indeterminate, and a biopsy is necessary for diagnosis. We provide a detailed update of soft tissue tumors and tumor-like conditions that occur in the pediatric population, emphasizing each lesion's conventional magnetic resonance imaging appearance, using the recently released 5th edition of the World Health Organization Classification of Soft Tissue and Bone Tumors as a guide. In part one of this review, pediatric tumor-like lesions, adipocytic tumors, fibroblastic and myofibroblastic tumors, and perivascular tumors are discussed. In part two, vascular lesions, fibrohistiocytic tumors, muscle tumors, peripheral nerve sheath tumors, tumors of uncertain differentiation, and undifferentiated small round cell sarcomas are reviewed. Per the convention of the WHO, these lesions involve the connective, subcutaneous, and other non-parenchymatous organ soft tissues, as well as the peripheral and autonomic nervous system.
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Butzmann A, Sridhar K, Jangam D, Song H, Singh A, Kumar J, Chisholm KM, Pinsky B, Huang F, Ohgami RS. Mutations in JAK/STAT and NOTCH1 Genes Are Enriched in Post-Transplant Lymphoproliferative Disorders. Front Oncol 2022; 11:790481. [PMID: 35111674 PMCID: PMC8801788 DOI: 10.3389/fonc.2021.790481] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/06/2021] [Accepted: 12/13/2021] [Indexed: 12/22/2022] Open
Abstract
Post-transplant lymphoproliferative disorders (PTLD) are diseases occurring in immunocompromised patients after hematopoietic stem cell transplantation (HCT) or solid organ transplantation (SOT). Although PTLD occurs rarely, it may be associated with poor outcomes. In most cases, PTLD is driven by Epstein-Barr virus (EBV) infection. Few studies have investigated the mutational landscape and gene expression profile of PTLD. In our study, we performed targeted deep sequencing and RNA-sequencing (RNA-Seq) on 16 cases of florid follicular hyperplasia (FFH) type PTLD and 15 cases of other PTLD types that include: ten monomorphic (M-PTLD), three polymorphic (P-PTLD), and two classic Hodgkin lymphoma type PTLDs (CHL-PTLD). Our study identified recurrent mutations in JAK3 in five of 15 PTLD cases and one of 16 FFH-PTLD cases, as well as 16 other genes that were mutated in M-PTLD, P-PTLD, CHL-PTLD and FFH-PTLD. Digital image analysis demonstrated significant differences in single cell area, major axis, and diameter when comparing cases of M-PTLD and P-PTLD to FFH-PTLD. No morphometric relationship was identified with regards to a specific genetic mutation. Our findings suggest that immune regulatory pathways play an essential role in PTLD, with the JAK/STAT pathway affected in many PTLDs.
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Affiliation(s)
- Alexandra Butzmann
- Agilent Technologies, Santa Clara, CA, United States
- Department of Pathology, University of California, San Francisco, San Francisco, CA, United States
- *Correspondence: Alexandra Butzmann,
| | - Kaushik Sridhar
- Department of Pathology, University of California, San Francisco, San Francisco, CA, United States
| | - Diwash Jangam
- Department of Pathology, Stanford University, Stanford, CA, United States
| | - Hanbing Song
- Department of Pathology, University of California, San Francisco, San Francisco, CA, United States
| | - Amol Singh
- Department of Pathology, Stanford University, Stanford, CA, United States
| | - Jyoti Kumar
- Department of Pathology, Stanford University, Stanford, CA, United States
| | - Karen M. Chisholm
- Department of Laboratories, Seattle Children’s Hospital, Seattle, WA, United States
| | - Benjamin Pinsky
- Department of Pathology, Stanford University, Stanford, CA, United States
| | - Franklin Huang
- Department of Pathology, University of California, San Francisco, San Francisco, CA, United States
| | - Robert S. Ohgami
- Department of Pathology, University of California, San Francisco, San Francisco, CA, United States
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Primary Central Nervous System Posttransplant Lymphoproliferative Disorder After Kidney Transplant. J Pediatr Hematol Oncol 2021; 43:144-146. [PMID: 32398602 DOI: 10.1097/mph.0000000000001818] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/20/2019] [Accepted: 04/05/2020] [Indexed: 11/25/2022]
Abstract
Primary central nervous system posttransplant lymphoproliferative disorder is a rare complication of solid organ transplantation, with increasing incidence in children given a steady rise in pediatric solid organ transplants. Given similar imaging appearance to many opportunistic infections, a high degree of awareness is required for prompt and early diagnosis. We report a case of primary central nervous system posttransplant lymphoproliferative disorder presenting as a single rim enhancing lesion with central restricted diffusion mimicking an intracranial abscess.
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