Soft-tissue sarcomas in children comprise a heterogeneous group of entities with variable manifestation depending on the age of the patient and the location of the tumor. MRI is the modality of ...choice for evaluating musculoskeletal soft-tissue tumors and plays a paramount role in both initial diagnosis and assessment of tumor response during and after treatment. Conventional MRI sequences, such as T1- and T2-weighted imaging, offer morphologic information, which is important for localizing the lesion and describing anatomic relationships but not accurate for determining its malignant or benign nature and may be limited in differentiating tumor response from therapy-related changes. Advanced multiparametric MRI offers further functional information that can help with these tasks by using different imaging sequences and biomarkers. The authors present the role of MRI in rhabdomyosarcoma and other soft-tissue sarcomas in children, emphasizing a multiparametric approach with focus on the utility and potential added value of diffusion-weighted imaging (DWI) and dynamic contrast-enhanced MRI in characterization and staging, determination of pretreatment extent, and evaluation of tumor response and recurrence after treatment.
RSNA, 2020.
Pediatric autoimmune pancreatitis (P-AIP) is an uncommon disease whose diagnosis requires strong clinical suspicion. Late diagnosis increases morbidity. We aimed to compare the usefulness of the 2011 ...International Consensus Diagnostic Criteria (ICDC) for Autoimmune Pancreatitis with the 2018 INSPPIRE (INternational Study Group of Pediatric Pancreatitis: In search for a cuRE) criteria.
We retrospectively analyzed demographics and clinical, laboratory, radiological, and histological findings at diagnosis and during long-term follow-up in children diagnosed with AIP in 2 tertiary hospitals between 2008 and 2021.
We included 11 patients 6 girls; median age at diagnosis, 12.5 (range 2.8-15.7) years. The most common symptom was abdominal pain. Pancreatic enzymes were elevated in 10 patients, and serum immunoglobulin G4 was elevated in 1. Magnetic resonance imaging showed enlargement of the pancreatic head in 10 patients and general pancreatic enlargement in 1. Pancreatic and papilla tissue were obtained from 9 patients. All patients received corticosteroids (prednisolone), and 4 also received azathioprine. According to the ICDC, all patients were classified as probable or non-otherwise specified AIP. According to INSPPIRE criteria, all patients were classified as AIP. Using the INSPPIRE criteria would have avoided biopsies in 6 patients who responded well to corticosteroids.
The INSPPIRE criteria are useful. Using the ICDC in pediatric patients can delay diagnosis and result in unnecessary invasive tests.
CT scan is a life-saving medical diagnostic tool, entailing higher levels of ionising radiation exposure than conventional radiography, which may result in an increase in cancer risk, particularly in ...children. Information about the use and potential health effects of CT scan imaging among young people in Spain is scarce.
This paper aims to estimate the number of radiation-related cancer cases which can be expected due to the use of CT scanning in Spanish children and young adults in a single year (2013).
The 2013 distribution of number and types of CT scans performed in young people was obtained for Catalonia and extrapolated to the whole Spain. Organ doses were estimated based on the technical characteristics of 17,406 CT examinations extracted from radiology records. Age and sex-specific data on cancer incidence and life tables were obtained for the Spanish population. Age and sex-specific risk models developed by the Committee on Health Risks of Exposure to Low Levels of Ionizing Radiations (BEIR VII) and Berrington de Gonzalez were used, together, with the dose estimates to derive the lifetime attributable risks of cancer in Spain due to one year of CT scanning and project the number of future cancer cases to be expected.
In 2013, 105,802 CT scans were estimated to have been performed in people younger than age 21. It was estimated that a total of 168.6 cancer cases (95% CrI: 30.1–421.1) will arise over life due to the ionising radiation exposure received during these CTs. Lifetime attributable risks per 100,000 exposed patients were highest for breast and lung cancer. The largest proportion of CTs was to the head and neck and hence the highest numbers of projected cancer cases were of thyroid and oral cavity/pharynx.
Despite the undeniable medical effectiveness of CT scans, this risk assessment suggests a small excess in cancer cases which underlines the need for justification and optimisation in paediatric scanning. Given the intrinsic uncertainties of these risk projection exercises, care should be taken when interpreting the predicted risks.
•This is the 1st estimation of the CT scan use in 0-20 year old Spanish population•>17,400 CT scans were used to reliably estimate the organ-doses•Chest-to-pelvis CTs conferred the highest lifetime risks•The 2013 CT scanning activity would increase 0.43% the lifetime risk of cancer
Pediatric surgical oncology is a technically challenging field that relies on CT and MRI as the primary imaging tools for surgical planning. However, recent advances in 3D reconstructions, including ...Cinematic Rendering, Volume Rendering, 3D modeling, Virtual Reality, Augmented Reality, and 3D printing, are increasingly being used to plan complex cases bringing new insights into pediatric tumors to guide therapeutic decisions and prognosis in different pediatric surgical oncology areas and locations including thoracic, brain, urology, and abdominal surgery. Despite this, challenges to their adoption remain, especially in soft tissue-based specialties such as pediatric surgical oncology. This work explores the main innovative imaging reconstruction techniques, 3D modeling technologies (CAD, VR, AR), and 3D printing applications through the analysis of three real cases of the most common and surgically challenging pediatric tumors: abdominal neuroblastoma, thoracic inlet neuroblastoma, and a bilateral Wilms tumor candidate for nephron-sparing surgery. The results demonstrate that these new imaging and modeling techniques offer a promising alternative for planning complex pediatric oncological cases. A comprehensive analysis of the advantages and limitations of each technique has been carried out to assist in choosing the optimal approach.
Osteosarcoma and Ewing sarcoma are the most common bone sarcomas in children. Their clinical presentation is very variable depending on the age of the patient and tumor location. MRI is the modality ...of choice to assess these bone sarcomas and has an important function at diagnosis and also for monitoring recurrence or tumor response. Anatomic sequences include T1- and T2-weighted images and provide morphological assessment that is crucial to localize the tumor and describe anatomical boundaries. Multiparametric MRI provides functional information that helps in the assessment of tumor response to therapy by using different imaging sequences and biomarkers. This review manuscript illustrates the role of MRI in osteosarcoma and Ewing sarcoma in the pediatric population, with emphasis on a functional perspective, highlighting the use of diffusion-weighted imaging and dynamic contrast-enhanced MRI at diagnosis, and during and after treatment.
Neuroblastic tumors (NBTs) originate from a block in the process of differentiation. Histologically, NBTs are classified in neuroblastoma (NB), ganglioneuroblastoma (GNB), and ganglioneuroma (GN). ...Current therapy for high-risk (HR) NB includes chemotherapy, surgery, radiotherapy, and anti-GD2 monoclonal antibodies (mAbs). Anti-GD2 mAbs induce immunological cytoxicity but also direct cell death.
We report on patients treated with naxitamab for chemorefractory NB showing lesions with long periods of stable disease. Target lesions with persisting
I-Metaiodobenzylguanidine (MIBG) uptake after 4 cycles of immunotherapy were further evaluated by functional Magnetic Resonance Imaging (MRI) and/or Fluorodeoxyglucose (FDG)-positron emission tomography (PET). MIBG avid lesions that became non-restrictive on MRI (apparent diffusion coefficient (ADC) > 1) and/or FDG-PET negative (SUV < 2) were biopsied.
Twenty-seven relapse/refractory (R/R) HR-NB patients were enrolled on protocol Ymabs 201. Two (7.5%) of the 27 showed persistent bone lesions on MIBG, ADC high, and/or FDG-PET negative. Forty-four R/R HR-NB patients received chemo-immunotherapy. Twelve (27%) of the 44 developed persistent MIBG+ but FDG-PET- and/or high ADC lesions. Twelve (86%) of the 14 cases identified were successfully biopsied producing 16 evaluable samples. Histology showed ganglioneuroma maturing subtype in 6 (37.5%); ganglioneuroma mature subtype with no neuroblastic component in 4 (25%); differentiating NB with no Schwannian stroma in 5 (31%); and undifferentiated NB without Schwannian stroma in one (6%). Overall, 10 (62.5%) of the 16 specimens were histopathologically fully mature NBTs.
Our results disclose an undescribed mechanism of action for naxitamab and highlight the limitations of conventional imaging in the evaluation of anti-GD2 immunotherapy clinical efficacy for HR-NB.
Less common manifestations in TSC Boronat, Susana; Barber, Ignasi
American journal of medical genetics. Part C, Seminars in medical genetics,
September 2018, 2018-09-00, 20180901, Letnik:
178, Številka:
3
Journal Article
Tuberous sclerosis complex (TSC) is due to pathogenic variants in TSC1 or TSC2 genes resulting in hyperactivation of the mTOR pathway. Many organ systems can be affected, such as brain, skin, eye, ...heart, bone, kidney, or lung. Typical lesions of TSC usually are those included as major criteria, including angiofibromas, hypomelanotic macules, tubers, subependymal nodules, angiomyolipomas, cardiac rhabdomyomas, and lymphangioleiomyomatosis. However, there are many other manifestations less frequent and/or less well known, many of them not included as clinical diagnostic criteria that are part of the clinical spectrum of TSC. The focus of this review will be on these less common and less well‐known manifestations of TSC. Among the rare manifestations, we will discuss some clinical findings including arteriopathy, arachnoid cysts, lymphatic involvement, chordomas, gynecological, endocrine, and gastrointestinal findings. Among the manifestations that are very frequent but much less well known, we find the sclerotic bone lesions. Although they are very frequent in TSC they have been largely overlooked and not considered diagnostic criteria, mainly because they are asymptomatic. However, it is important to know their typical characteristics to avoid misdiagnosing them as metastasis.
Abstract Background It has been suggested that platelet-rich plasma (PRP) might enhance bone formation. The aim of this study was to quantify the effect of PRP administered in the early phases of ...distraction osteogenesis in an ovine femur model. Methods Twenty sheep aged 4 months underwent osteotomy of the femoral diaphysis followed by distraction osteogenesis. The sheep were divided into two groups of 10. One group received three injections of PRP on days 0, 10, and 20 of the procedure (PRP group) and the other received no additional treatment (control group). The results were evaluated by computed tomography (CT) and histology on completion of distraction osteogenesis (day 40). CT evaluation included measurement of the callus and bone density. Femur size was also measured proximally and distally. Histological evaluation was used to quantify osteoblasts, osteoclasts, vascular lumens, and trabecular maturity by zones and to calculate trabecular bone, fibrous tissue, and cartilage ratios. Results Radiological and histological evaluation of the regenerate bone showed no significant differences between the PRP group and the control group for any of the variables analysed. The only significant difference detected was a wider femur (increased diaphyseal thickness) at the proximal and distal levels in the PRP group. Conclusion We found no radiological or histological evidence that the administration of PRP in the early phases of distraction osteogenesis enhances bone formation.
Background
To review the safety and efficacy of percutaneous cryoablation for the treatment of chondroblastoma and osteoblastoma in the pediatric and adolescent population.
Materials and methods
A ...retrospective review from 2016 to 2020 was performed to evaluate clinical and imaging response to percutaneous cryoablation in 11 symptomatic patients with diagnosis of chondroblastoma and osteoblastoma treated from two pediatric hospitals with at least 12-month follow-up. Technical success (correct needle placement and potential full coverage of the tumor with the planned ablation zone) and clinical success (relief of the symptoms) were evaluated. The primary objective was to alleviate pain related to the lesion(s). Immediate and late complications were recorded. Patients were followed in clinic and with imaging studies such as MRI or CT for a minimum of 6 months.
Results
A total of 11 patients were included (mean 14 years, age range 9–17; male
n
= 8). Diagnoses were osteoblastoma (
n
= 4) and chondroblastoma (
n
= 7). Locations were proximal humerus (
n
= 1), femur condyle (
n
= 1), and proximal femur (
n
= 1) tibia (
n
= 3), acetabulum (
n
= 3), thoracic vertebra (
n
= 1) and lumbar vertebra (
n
= 1). Cryoablation was technically successful in all patients. Clinical success (cessation of pain) was achieved in all patients. No signs of recurrence were observed on imaging follow-up in any of the patients. One of the patients developed periprocedural right L2–L3 transient radiculopathy as major immediate complication.
Conclusions
Percutaneous image-guided cryoablation can be considered potentially safe and effective treatment for chondroblastoma and osteoblastoma in children and adolescents.
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