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ABSTRACT: 123I-meta-iodobenzylguanidine (123I-MIBG) is extensively used for initial staging and response evaluation in children with neuroblastoma. Physiological uptake of 123I-MIBG occurs in the salivary glands, liver, adrenal gland, myocardium, bowel, and thyroid gland. 123I-MIBG cannot cross an intact blood-brain barrier. We present the rare case of a 3-year-old boy with neuroblastoma and meningeal metastases who underwent an 123I-MIBG scan for disease restaging that showed abnormal brain uptake. Abnormal MIBG uptake in the brain can occur if there is disruption of the blood-brain barrier either secondary to metastases or after damage to blood-brain barrier.
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Iodobenzenos , Neuroblastoma , Pré-Escolar , Humanos , Masculino , 3-Iodobenzilguanidina , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Neuroblastoma/diagnóstico por imagem , Neuroblastoma/patologia , CintilografiaRESUMO
Soft tissue sarcomas account for 6%-8% of pediatric cancers. The rhabdomyosarcoma family is the most frequent soft tissue sarcoma in this age group accounting for 3% of pediatric cancers. Rhabdomyosarcomas are high-grade tumors with a high propensity to metastasize. The risk-adapted, multimodal therapeutic approach for rhabdomyosarcomas incorporates a combination of surgery, radiotherapy, and multi-agent cytotoxic chemotherapy. Soft tissue sarcomas other than rhabdomyosarcoma account for 3%-4% of pediatric cancers. The nonrhabdomyosarcoma soft tissue sarcomas include both low-grade and high-grade tumors. While surgery is the mainstay of therapy in most non-rhabdomyosarcoma soft tissue sarcomas, many cases require a multimodal therapeutic approach including radiotherapy and chemotherapy. In North America, most pediatric patients with soft tissue sarcomas are treated in Children's Oncology Group clinical trials. In this article, we will primarily focus on the staging, risk stratification, imaging recommendations, and interpretations in accordance with the Children's Oncology Group trials. We will review the results and recommendations of International Soft Tissue Sarcoma Database Consortium and European trials in relevant sections where they provide complementary guidelines.
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Imageamento por Ressonância Magnética , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Sarcoma , Humanos , Sarcoma/diagnóstico por imagem , Sarcoma/terapiaRESUMO
Gender diverse adolescents have low pretreatment bone mineral density (BMD), with variable changes in BMD after initiation of gender-affirming treatment. We aimed to assess factors associated with low BMD in gender diverse youth. Sixty-four patients were included in our analysis (73% assigned male at birth). Subtotal whole-body BMD Z-scores were low in 30% of patients, and total lumbar spine BMD Z-scores low in 14%. There was a positive association with body mass index, and no association with vitamin D level. Male sex assigned at birth was associated with lower pretreatment BMD, with lower average BMD Z-scores compared to previous studies.
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Clinical trials frequently include multiple end points that mature at different times. The initial report, typically based on the primary end point, may be published when key planned co-primary or secondary analyses are not yet available. Clinical Trial Updates provide an opportunity to disseminate additional results from studies, published in JCO or elsewhere, for which the primary end point has already been reported.ARST1321 was a phase II study designed to compare the near complete pathologic response rate after preoperative chemoradiation with/without pazopanib in children and adults with intermediate-/high-risk chemotherapy-sensitive body wall/extremity non-Rhabdomyosarcoma Soft Tissue Sarcoma (ClinicalTrials.gov identifier: NCT02180867). Enrollment was stopped early following a predetermined interim analysis that found the rate of near complete pathologic response to be significantly greater with the addition of pazopanib. As a planned secondary aim of the study, the outcome data for this cohort were analyzed. Eight-five eligible patients were randomly assigned to receive (regimen A) or not receive (regimen B) pazopanib in combination with ifosfamide and doxorubicin + preoperative radiotherapy followed by primary resection at week 13 and then further chemotherapy at week 25. As of December 31, 2021, at a median survivor follow-up of 3.3 years (range, 0.1-5.8 years), the 3-year event-free survival for all patients in the intent-to-treat analysis was 52.5% (95% CI, 34.8 to 70.2) for regimen A and 50.6% (95% CI, 32 to 69.2) for regimen B (P = .8677, log-rank test); the 3-year overall survival was 75.7% (95% CI, 59.7 to 91.7) for regimen A and 65.4% (95% CI, 48.1 to 82.7) for regimen B (P = .1919, log-rank test). Although the rate of near complete pathologic response was significantly greater with the addition of pazopanib, outcomes were not statistically significantly different between the two regimens.
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Sarcoma , Neoplasias de Tecidos Moles , Adulto , Humanos , Criança , Sarcoma/tratamento farmacológico , Neoplasias de Tecidos Moles/patologia , Ifosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêuticoRESUMO
BACKGROUND: Diagnostic mIBG (meta-iodobenzylguanidine) scans are an integral component of response assessment in children with high-risk neuroblastoma. The role of end-of-induction (EOI) Curie scores (CS) was previously described in patients undergoing a single course of high-dose chemotherapy (HDC) and autologous hematopoietic cell transplant (AHCT) as consolidation therapy. OBJECTIVE: We now examine the prognostic significance of CS in patients randomized to tandem HDC and AHCT on the Children's Oncology Group (COG) trial ANBL0532. STUDY DESIGN: A retrospective analysis of mIBG scans obtained from patients enrolled in COG ANBL0532 was performed. Evaluable patients had mIBG-avid, International Neuroblastoma Staging System (INSS) stage 4 disease, did not progress during induction therapy, consented to consolidation randomization, and received either single or tandem HDC (n = 80). Optimal CS cut points maximized the outcome difference (≤CS vs. >CS cut-off) according to the Youden index. RESULTS: For recipients of tandem HDC, the optimal cut point at diagnosis was CS = 12, with superior event-free survival (EFS) from study enrollment for patients with CS ≤ 12 (3-year EFS 74.2% ± 7.9%) versus CS > 12 (59.2% ± 7.1%) (p = .002). At EOI, the optimal cut point was CS = 0, with superior EOI EFS for patients with CS = 0 (72.9% ± 6.4%) versus CS > 0 (46.5% ± 9.1%) (p = .002). CONCLUSION: In the setting of tandem transplantation for children with high-risk neuroblastoma, CS at diagnosis and EOI may identify a more favorable patient group. Patients treated with tandem HDC who exhibited a CS ≤ 12 at diagnosis or CS = 0 at EOI had superior EFS compared to those with CS above these cut points.
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Transplante de Células-Tronco Hematopoéticas , Neuroblastoma , Criança , Humanos , Lactente , 3-Iodobenzilguanidina/uso terapêutico , Transplante Autólogo , Estudos Retrospectivos , Neuroblastoma/patologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Intervalo Livre de DoençaRESUMO
BACKGROUND: In the Children's Oncology Group ANBL1221 phase 2 trial for patients with first relapse/first declaration of refractory high-risk neuroblastoma, irinotecan and temozolomide (I/T) combined with either temsirolimus (TEMS) or immunotherapy (the anti-GD2 antibody dinutuximab (DIN) and granulocyte macrophage colony stimulating factory (GM-CSF)) was administered. The response rate among patients treated with I/T/DIN/GM-CSF in the initial cohort (n=17) was 53%; additional patients were enrolled to permit further evaluation of this chemoimmunotherapy regimen. Potential associations between immune-related biomarkers and clinical outcomes including response and survival were evaluated. METHODS: Patients were evaluated for specific immunogenotypes that influence natural killer (NK) cell activity, including killer immunoglobulin-like receptors (KIRs) and their ligands, Fc gamma receptors, and NCR3. Total white cells and leucocyte subsets were assessed via complete blood counts, and flow cytometry of peripheral blood mononuclear cells was performed to assess the potential association between immune cell subpopulations and surface marker expression and clinical outcomes. Appropriate statistical tests of association were performed. The Bonferroni correction for multiple comparisons was performed where indicated. RESULTS: Of the immunogenotypes assessed, the presence or absence of certain KIR and their ligands was associated with clinical outcomes in patients treated with chemoimmunotherapy rather than I/T/TEMS. While median values of CD161, CD56, and KIR differed in responders and non-responders, statistical significance was not maintained in logistic regression models. White cell and neutrophil counts were associated with differences in survival outcomes, however, increases in risk of event in patients assigned to chemoimmunotherapy were not clinically significant. CONCLUSIONS: These findings are consistent with those of prior studies showing that KIR/KIR-ligand genotypes are associated with clinical outcomes following anti-GD2 immunotherapy in children with neuroblastoma. The current study confirms the importance of KIR/KIR-ligand genotype in the context of I/T/DIN/GM-CSF chemoimmunotherapy administered to patients with relapsed or refractory disease in a clinical trial. These results are important because this regimen is now widely used for treatment of patients at time of first relapse/first declaration of refractory disease. Efforts to assess the role of NK cells and genes that influence their function in response to immunotherapy are ongoing. TRIAL REGISTRATION NUMBER: NCT01767194.
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Fator Estimulador de Colônias de Granulócitos e Macrófagos , Neuroblastoma , Humanos , Criança , Ligantes , Fator Estimulador de Colônias de Granulócitos e Macrófagos/genética , Leucócitos Mononucleares , Neuroblastoma/tratamento farmacológico , Neuroblastoma/genética , Genótipo , Receptores KIR/genética , Antígenos de Histocompatibilidade , Irinotecano/uso terapêutico , Imunoterapia , RecidivaRESUMO
ABSTRACT: 99m Tc-dimercaptosuccinic acid ( 99m Tc-DMSA) scans are used to evaluate renal cortical defects typically related to parenchymal scarring or pyelonephritis, and ectopic renal parenchyma. 99m Tc-DMSA binds to metalloproteins in proximal tubular cells and typically localizes to the renal cortex, with minimal excretion. Planar and SPECT images are obtained 2 to 4 hours after IV administration of 99m Tc-DMSA. Altered 99m Tc-DMSA biodistribution has been reported in various conditions, including renal injury, technical issues, infiltrative processes, and hematologic disorders. Here, we present a case of altered biodistribution, with hepatic and splenic radiotracer uptake in the setting of hepatosplenomegaly and hematologic abnormalities concerning for a systemic hematologic disorder/lymphohistiocytosis.
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Pielonefrite , Ácido Dimercaptossuccínico Tecnécio Tc 99m , Humanos , Distribuição Tecidual , Rim , Cintilografia , Pielonefrite/diagnóstico por imagem , Compostos RadiofarmacêuticosRESUMO
BACKGROUND: There is no standardized approach to iodine-131 (I-131) therapy of hyperthyroidism in pediatric Graves disease. This prevents systematic study of outcomes. OBJECTIVE: To characterize current radioiodine dosing and define therapeutic outcomes at multiple institutions that use ultrasound to measure thyroid size to guide I-131 ablation of Graves disease. MATERIALS AND METHODS: This was a retrospective cohort study conducted at three institutions. The three sites collected demographic data, thyroid volume measured by ultrasound (mL), pre-ablation radioiodine uptake, I-131 activity administered, and outcomes at 6 and 12 months for children younger than 18 years of age treated with I-131 between November 2004 and October 2019. Comparisons of continuous variables were performed using the Mann-Whitney U test. RESULTS: Sixty-nine patients (mean age: 14.5±2.5 years) were included, 59 (85.5%) of whom were female. The mean administered I-131 radioiodine activity was 12.5 mCi (463 MBq) (range: 3.8-29.9 mCi [141-1,106 MBq]). At 6 months post-ablation, 54 (80.5% of 67) patients were hypothyroid, 8 (11.9% of 67) were euthyroid and 5 were hyperthyroid. Two of the five hyperthyroid patients had become euthyroid at 12 months. At 12 months, 1 previously euthyroid patient was hyperthyroid. Administered activity per mL of thyroid tissue adjusted for 24-h uptake was lower (0.18 mCi [6.7 MBq] x %/mL vs. 0.31 mCi [11.5 MBq] x %/mL, P=0.0054) for patients who remained hyperthyroid at 6 months. CONCLUSION: There is substantial variability in administered activity for radioiodine ablation of Graves disease in children. Efforts to standardize practice should start by standardizing administered activity guided by measurement of thyroid size by ultrasound. Our results and those of previous studies suggest the need for administered activities ≥0.25 mCi [9.3 MBq] x %/mL of thyroid tissue.
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Doença de Graves , Hipertireoidismo , Criança , Humanos , Feminino , Adolescente , Masculino , Radioisótopos do Iodo/uso terapêutico , Estudos Retrospectivos , Doença de Graves/diagnóstico por imagem , Doença de Graves/radioterapia , Doença de Graves/tratamento farmacológico , Hipertireoidismo/tratamento farmacológico , Resultado do TratamentoRESUMO
Pediatric thyroid cancer is rare in children; however, incidence is increasing. Papillary thyroid cancer and follicular thyroid cancer are the most common subtypes, comprising about 90% and 10% of cases, respectively. This paper provides consensus imaging recommendations for evaluation of pediatric patients with thyroid cancer at diagnosis and during follow-up.
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Adenocarcinoma Folicular , Neoplasias da Glândula Tireoide , Humanos , Criança , Ressonância de Plasmônio de Superfície , Neoplasias da Glândula Tireoide/diagnóstico por imagem , Neoplasias da Glândula Tireoide/epidemiologia , Adenocarcinoma Folicular/diagnóstico por imagem , Câncer Papilífero da Tireoide , IncidênciaRESUMO
Neuroblastoma is the most common extracranial solid neoplasm in children. This manuscript provides consensus-based imaging recommendations for pediatric neuroblastoma patients at diagnosis and during follow-up.
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Neuroblastoma , Ressonância de Plasmônio de Superfície , Criança , Humanos , Neuroblastoma/patologia , Diagnóstico por Imagem , Estadiamento de NeoplasiasRESUMO
Adrenal tumors other than neuroblastoma are uncommon in children. The most frequently encountered are adrenocortical carcinoma and pheochromocytoma. This paper offers consensus recommendations for imaging of pediatric patients with a known or suspected primary adrenal malignancy other than neuroblastoma at diagnosis and during follow-up.
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Neoplasias do Córtex Suprarrenal , Neoplasias das Glândulas Suprarrenais , Neuroblastoma , Criança , Humanos , Ressonância de Plasmônio de Superfície , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/patologia , Neuroblastoma/diagnóstico por imagem , Diagnóstico por ImagemRESUMO
The most common indication for 18F-FDG PET/CT is tumor imaging, which may be performed for initial diagnosis, staging, therapeutic response monitoring, surveillance, or suspected recurrence. In the routine practice of pediatric nuclear medicine, most infectious, inflammatory, and autoimmune processes that are detected on 18F-FDG PET/CT imaging - except for imaging in fever or inflammation of unknown origin - are coincidental and not the main indication for image acquisition. However, interpreting these "coincidental" findings is of utmost importance to avoid erroneously attributing these findings to a neoplastic process. We review the recent literature on fever of unknown origin as well as inflammation of unknown origin in pediatrics and then focus on the 18F FDG PET/CT imaging findings seen in two specific entities with increased immune reactivity: hemophagocytic lymphohistiocytosis syndrome and the immune-related adverse events associated with checkpoint inhibitors. We will subsequently close with two sections highlighting related topics and relevant references for further reading.
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Febre de Causa Desconhecida , Fluordesoxiglucose F18 , Humanos , Criança , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Compostos Radiofarmacêuticos , Tomografia por Emissão de Pósitrons/métodos , Inflamação/diagnóstico por imagem , Febre de Causa Desconhecida/diagnóstico por imagemRESUMO
Meckel diverticulum, the most common congenital anomaly of the gastrointestinal tract, results from the aberrant involution of the omphalomesenteric duct and accounts for more than 50% of unexplained lower gastrointestinal bleeding in the pediatric population. The most accurate imaging tool to identify a Meckel diverticulum containing ectopic gastric mucosa is the Technetium-99m pertechnetate Meckel scan, a scintigraphic study with a reported accuracy of 90% in the pediatric population. In addition to depicting a Meckel diverticulum with ectopic gastric mucosa, careful attention to the normal biodistribution of the radiotracer can lead to the identification of unexpected pathology with implications for patient management. This article serves to review the embryological origin and anatomical features of Meckel diverticulum, highlight the role of scintigraphy in evaluating Meckel diverticulum, and discuss the proper imaging technique when performing this test. We will focus on pitfalls that can lead to an erroneous diagnosis as well as incidental findings that can affect patient management.
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Divertículo Ileal , Doenças Musculoesqueléticas , Criança , Humanos , Divertículo Ileal/diagnóstico por imagem , Distribuição Tecidual , Compostos Radiofarmacêuticos , Cintilografia , Hemorragia Gastrointestinal/diagnóstico por imagem , Pertecnetato Tc 99m de SódioRESUMO
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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Transplante de Células-Tronco Hematopoéticas , Ressonância de Plasmônio de Superfície , Criança , Humanos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/métodos , Oncologia , TransplantadosRESUMO
PURPOSE: Postconsolidation immunotherapy including dinutuximab, granulocyte-macrophage colony-stimulating factor, and interleukin-2 improved outcomes for patients with high-risk neuroblastoma enrolled on the randomized portion of Children's Oncology Group study ANBL0032. After random assignment ended, all patients were assigned to immunotherapy. Survival and toxicities were assessed. PATIENTS AND METHODS: Patients with a pre-autologous stem cell transplant (ASCT) response (excluding bone marrow) of partial response or better were eligible. Demographics, stage, tumor biology, pre-ASCT response, and adverse events were summarized using descriptive statistics. Event-free survival (EFS) and overall survival (OS) from time of enrollment (up to day +200 from last ASCT) were evaluated. RESULTS: From 2009 to 2015, 1,183 patients were treated. Five-year EFS and OS for the entire cohort were 61.1 ± 1.9% and 71.9 ± 1.7%, respectively. For patients ≥ 18 months old at diagnosis with International Neuroblastoma Staging System stage 4 disease (n = 662) 5-year EFS and OS were 57.0 ± 2.4% and 70.9 ± 2.2%, respectively. EFS was superior for patients with complete response/very good partial response pre-ASCT compared with those with PR (5-year EFS: 64.2 ± 2.2% v 55.4 ± 3.2%, P = .0133); however, OS was not significantly different. Allergic reactions, capillary leak, fever, and hypotension were more frequent during interleukin-2-containing cycles than granulocyte-macrophage colony-stimulating factor-containing cycles (P < .0001). EFS was superior in patients with higher peak dinutuximab levels during cycle 1 (P = .034) and those with a high affinity FCGR3A genotype (P = .0418). Human antichimeric antibody status did not correlate with survival. CONCLUSION: Analysis of a cohort assigned to immunotherapy after cessation of random assignment on ANBL0032 confirmed previously described survival and toxicity outcomes. EFS was highest among patients with end-induction complete response/very good partial response. Among patients with available data, higher dinutuximab levels and FCGR3A genotype were associated with superior EFS. These may be predictive biomarkers for dinutuximab therapy.
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Fator Estimulador de Colônias de Granulócitos e Macrófagos , Interleucina-2 , Criança , Humanos , Lactente , Fator Estimulador de Colônias de Granulócitos e Macrófagos/efeitos adversos , Interleucina-2/efeitos adversos , Projetos de PesquisaRESUMO
Hydrocephalus is the most common neurosurgical disorder in children, and cerebrospinal fluid (CSF) diversion with shunt placement is the most commonly performed pediatric neurosurgical procedure. CT is frequently used to evaluate children with suspected CSF shunt malfunction to assess change in ventricular size. Moreover, careful review of the CT images is important to confirm the integrity of the imaged portions of the shunt system. Subtle shunt disruptions can be missed on multiplanar two-dimensional (2-D) CT images, especially when the disruption lies in the plane of imaging. The use of volume-rendered CT images enables radiologists to view the extracranial shunt tubing within the field of view as a three-dimensional (3-D) object. This allows for a rapid and intuitive method of assessing the integrity of the extracranial shunt tubing. The purpose of this pictorial essay is to discuss how volume-rendered CT images can be generated to evaluate CSF shunts in the pediatric population and to provide several examples of their utility in diagnosing shunt disruption. We also address the potential pitfalls of this technique and ways to avoid them.
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Derivações do Líquido Cefalorraquidiano , Hidrocefalia , Criança , Cabeça/cirurgia , Humanos , Hidrocefalia/diagnóstico por imagem , Hidrocefalia/cirurgia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos , Derivação VentriculoperitonealRESUMO
Hepatobiliary iminodiacetic acid (HIDA) scan is one of the principal imaging modalities for the evaluation of the gallbladder and biliary tree. Congenital biliary anomalies are rare and can be difficult to recognize on HIDA scan. They may also mimic other biliary pathology. The purpose of this article is to review the spectrum of congenital gallbladder and biliary anomalies and describe their imaging appearance on HIDA scan. In addition, the diagnostic utility of functional imaging with HIDA when evaluating biliary tract anomalies is described.
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Sistema Biliar , Vesícula Biliar , Sistema Biliar/diagnóstico por imagem , Vesícula Biliar/diagnóstico por imagem , Humanos , IminoácidosRESUMO
Importance: The incidence of Graves disease (GD) is rising in children, and adequate care of these patients requires a multidisciplinary approach. Whether patients are seen in the context of endocrinology, nuclear medicine, or surgery, it is important to know the nuances of the therapeutic options in children. Observations: Given the rarity of GD in children, it is important to recognize its various clinical presenting signs and symptoms, as well as the tests that may be important for diagnosis. The diagnosis is typically suspected clinically and then confirmed biochemically. Imaging tests, including thyroid ultrasonography and/or nuclear scintigraphy, may also be used as indicated during care. It is important to understand the indications for and interpretation of laboratory and imaging tools so that a diagnosis is made efficiently and unnecessary tests are not ordered. Clinicians should be well-versed in treatment options to appropriately counsel families. There are specific scenarios in which medical therapy, radioactive iodine therapy, or surgery should be offered. Conclusions and Relevance: The diagnosis and treatment of pediatric patients with GD requires a multidisciplinary approach, involving pediatric specialists in the fields of endocrinology, ophthalmology, radiology, nuclear medicine, and surgery/otolaryngology. Antithyroid drugs are typically the first-line treatment, but sustained remission rates with medical management are low in the pediatric population. Consequently, definitive treatment is often necessary, either with radioactive iodine or with surgery, ideally performed by experienced, high-volume pediatric experts. Specific clinical characteristics, such as patients younger than 5 years or the presence of a thyroid nodule, may make surgery the optimal treatment for certain patients.
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Doença de Graves/diagnóstico , Doença de Graves/terapia , Adolescente , Antitireóideos/uso terapêutico , Criança , Pré-Escolar , Terapia Combinada , Doença de Graves/fisiopatologia , Humanos , Lactente , Radioisótopos do Iodo/uso terapêutico , Equipe de Assistência ao Paciente , Cintilografia , Compostos Radiofarmacêuticos/uso terapêutico , Tireoidectomia , UltrassonografiaRESUMO
INTRODUCTION: 131 I-meta-iodobenzylguanidine (131 I-MIBG) is effective in relapsed neuroblastoma. The Children's Oncology Group (COG) conducted a pilot study (NCT01175356) to assess tolerability and feasibility of induction chemotherapy followed by 131 I- MIBG therapy and myeloablative busulfan/melphalan (Bu/Mel) in patients with newly diagnosed high-risk neuroblastoma. METHODS: Patients with MIBG-avid high-risk neuroblastoma were eligible. After the first two patients to receive protocol therapy developed severe sinusoidal obstruction syndrome (SOS), the trial was re-designed to include an 131 I-MIBG dose escalation (12, 15, and 18 mCi/kg), with a required 10-week gap before Bu/Mel administration. Patients who completed induction chemotherapy were evaluable for assessment of 131 I-MIBG feasibility; those who completed 131 I-MIBG therapy were evaluable for assessment of 131 I-MIBG + Bu/Mel feasibility. RESULTS: Fifty-nine of 68 patients (86.8%) who completed induction chemotherapy received 131 I-MIBG. Thirty-seven of 45 patients (82.2%) evaluable for 131 I-MIBG + Bu/Mel received this combination. Among those who received 131 I-MIBG after revision of the study design, one patient per dose level developed severe SOS. Rates of moderate to severe SOS at 12, 15, and 18 mCi/kg were 33.3%, 23.5%, and 25.0%, respectively. There was one toxic death. The 131 I-MIBG and 131 I-MIBG+Bu/Mel feasibility rates at the 15 mCi/kg dose level designated for further study were 96.7% (95% CI: 83.3%-99.4%) and 81.0% (95% CI: 60.0%-92.3%). CONCLUSION: This pilot trial demonstrated feasibility and tolerability of administering 131 I-MIBG followed by myeloablative therapy with Bu/Mel to newly diagnosed children with high-risk neuroblastoma in a cooperative group setting, laying the groundwork for a cooperative randomized trial (NCT03126916) testing the addition of 131 I-MIBG during induction therapy.
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3-Iodobenzilguanidina , Neuroblastoma , 3-Iodobenzilguanidina/efeitos adversos , 3-Iodobenzilguanidina/uso terapêutico , Bussulfano/uso terapêutico , Estudos de Viabilidade , Humanos , Radioisótopos do Iodo , Recidiva Local de Neoplasia , Neuroblastoma/radioterapia , Projetos PilotoRESUMO
Hyperparathyroidism, due to increased secretion of parathyroid hormones, may be primary, secondary or tertiary. Most pediatric patients with sporadic primary hyperparathyroidism will be symptomatic, presenting with either end-organ damage or nonspecific symptoms. In younger patients with primary hyperparathyroidism, there is a higher prevalence of familial hyperparathyroidism including germline inactivating mutations of the calcium-sensing receptor genes that result in either neonatal severe hyperparathyroidism or familial hypocalciuric hypercalcemia. Parathyroid scintigraphy and ultrasound are complementary, first-line imaging modalities for localizing hyperfunctioning parathyroid glands. Second-line imaging modalities are multiphase computed tomography (CT) and magnetic resonance imaging. In pediatrics, multiphase CT protocols should be adjusted to optimize radiation dose. Although, the role of these imaging modalities is better established in preoperative localization of hyperfunctioning parathyroid glands in primary hyperparathyroidism, the same principles apply in secondary and tertiary hyperparathyroidism. In this manuscript, we will review the embryology, anatomy, pathophysiology and preoperative localization of parathyroid glands as well as several subtypes of primary familial hyperparathyroidism. While most of the recent imaging literature centers on adults, we will focus on the issues that are pertinent and applicable to pediatrics.