RESUMO
Dermal non-neural granular cell tumors, also known as primitive polypoid granular cell tumors, are a rare group of distinct cutaneous non-neural granular cell tumors. Pediatric cases are rare, and to the best of our knowledge, we report the youngest patient with dermal non-neural granular cell tumors.
Assuntos
Tumor de Células Granulares/patologia , Neoplasias Cutâneas/patologia , Pré-Escolar , Diagnóstico Diferencial , Humanos , Pele/patologiaRESUMO
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive hematologic malignancy characterized by frequent skin involvement that most commonly affects older patients. BPDCN is known to have a poor prognosis. Our objective was to assess if outcome and disease prognosis were independently influenced by age when evaluated with clinical presentation, sex, and treatment regimens. We conducted a systematic review to identify BPDCN cases, to compare pediatric BPDCN cases with adult cases. A total of 125 publications were identified detailing 356 cases. Including 1 pediatric case from our institution, 74 were children, and 283 were adults aged 19 or over. Age was shown to be an independent prognostic factor predictive of more favorable outcomes across measures including initial response to therapy, likelihood of relapse, and overall survival at follow-up. The distribution of affected organs at diagnosis was similar across children and adults and type of clinical presentation did not disproportionately influence 1 age group's prognosis over the other. Acute lymphoblastic leukemia-type chemotherapy regimens were shown to be superior to other chemotherapy regimens (acute myeloid leukemia, lymphoma, acute lymphoblastic leukemia/lymphoma, other, or none) in inducing complete remission. Allogeneic stem cell transplantation was shown to increase mean survival time. Future research may be directed toward elucidating the further morphologic, cytogenetic, and cytochemical differences between younger and older BPDCN patients.
Assuntos
Células Dendríticas/patologia , Neoplasias Hematológicas/patologia , Neoplasias Hematológicas/terapia , Adulto , Criança , Neoplasias Hematológicas/classificação , Transplante de Células-Tronco Hematopoéticas , Humanos , Leucemia Mieloide Aguda/patologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Prognóstico , Neoplasias Cutâneas/patologia , Adulto JovemRESUMO
PURPOSE: Coronary computed tomography angiography (CCTA) is an important non-invasive tool for the assessment of coronary artery disease and the delivery of information incremental to coronary anatomy. CCTA measured left ventricular (LV) mid-diastolic volume (LVMDV) and LV mass (LVMass) have important prognostic information but the utility of prospectively ECG-triggered CCTA to predict reduced left ventricular ejection fraction (LVEF) is unknown. The objective of this study was to determine if indexed LVMDV (LVMDVi) and the LVMDV:LVMass ratio on CCTA can identify patients with reduced LVEF. MATERIALS/METHODS: 8179 patients with prospectively ECG-triggered CCTA between November 2014 and December 2019 were reviewed. A subset derivation cohort of 4352 healthy patients was used to define normal LVMDVi and LVMDV:LVMass. Sex-specific thresholds were tested in a validation cohort of 1783 patients, excluded from the derivation cohort, with cardiac disease and known LVEF. The operating characteristics for 1 SD above the mean were tested for the identification of abnormal LVEF, LVEF≤35 â% and ≤30 â%. RESULTS: The derivation cohort had a mean LVMDVi of 61.0 â± â13.7 âmL/m2 and LVMDV:LVMass of 1.11 â± â0.24 âmL/g. LVMDVi and LVMDV:LVMass were both higher in patients with reduced LVEF than those with normal LVEF (98.8 â± â40.8 âmL/m2 vs. 63.3 â± â19.7 âmL/m2, p â< â0.001, and 1.32 â± â0.44 âmL/g vs. 1.05 â± â0.28 âmL/g, p â< â0.001). Both mean LVMDVi and LVMDV:LVMass increased with the severity of LVEF reduction. Sex-specific LVMDVi thresholds were 79 â% and 80 â% specific for identifying abnormal LVEF in females (LVMDVi â≥ â69.9 âmL/m2) and males (LVMDVi â≥ â78.8 âmL/m2), respectively. LVMDV:LVMass thresholds had high specificity (87 â%) in both females (LVMDVi:LVMass â≥ â1.39 âmL/g) and males (LVMDVi:LVMass â≥ â1.30 âmL/g). CONCLUSION: Our study provides reference thresholds for LVMDVi and LVMDV:LVMass on prospectively ECG-triggered CCTA, which may identify patients who require further LV function assessment.