Automatic assessment of body composition in children with lymphoma: results of a [18F]FDG-PET/MR study.

OBJECTIVES: To use Dixon-MR images extracted from [18F]FDG-PET/MR scans to perform an automatic, volumetric segmentation and quantification of body composition in pediatric patients with lymphoma. MATERIALS AND METHODS: Pediatric patients with lymphoma examined by [18F]FDG-PET/MR at diagnosis and restaging were included. At each time point, axial fat and water Dixon T1w images of the thighs were automatically segmented and muscle volume, subcutaneous, intramuscular, and intermuscular fat volume were quantified. The metabolic activity of the largest nodal lesion and of muscles and subcutaneous fat was recorded. The paired samples t-test and Spearman's correlation coefficient were applied to evaluate potential differences between the two time points and the relationship between metabolic and body composition metrics, respectively. By logistic regression analysis, the prognostic role of the investigated variables was assessed. The applied significance level was p < 0.05 for all analyses. RESULTS: Thirty-seven patients (mean age ± SD 14 ± 3-years-old; 20 females) matched the inclusion criteria. After chemotherapy (interval between the two PET/MR scans, 56-80 days; median 65 days), muscle volume significantly decreased (629 ± 259 cm3 vs 567 ± 243 cm3, p < 0.001) while subcutaneous, intramuscular and intermuscular fat increased (476 ± 255 cm3 vs 607 ± 254 cm3, p < 0.001; 63 ± 20 cm3 vs 76 ± 26 cm3, p < 0.001; 58 ± 19 cm3 vs 71 ± 23 cm3, p < 0.001); the metabolic activity of the main nodal lesion, muscles, and subcutaneous fat significantly decreased (p < 0.05, each). None of the examined variables acted as predictors of the response to treatment (p = 0.283). A strong correlation between BMI and subcutaneous fat volume at diagnosis (r = 0.675, p < 0.001) and restaging (r = 0.600, p < 0.001) emerged. CONCLUSIONS: The proposed method demonstrated that pediatric patients with lymphoma undergo muscle loss and an increase of subcutaneous fat during treatment. CLINICAL RELEVANCE STATEMENT: The proposed automatic and volumetric MR-based assessment of body composition in children with lymphoma can be used to monitor the effect of chemotherapy and may guide tailored exercise programs during chemotherapy. KEY POINTS: T1w Dixon images can be used for the automatic segmentation and quantification of body composition. Muscle and subcutaneous fat volume do not act as predictors of the response to treatment in children with lymphoma. Chemotherapy induces changes in body composition in children with lymphoma.

Lung relapse pattern in children with metastatic Wilms tumor.

Wilms tumor is the most common pediatric renal cancer, and lungs represent the major site of metastasis and recurrence. Relapse occurs in 15%, months or years after treatment; so due to the small sample, acquiring more data about the pattern of lung relapse remains a challenge. The aim of our study was to evaluate if pulmonary relapse, detected by computed tomography (CT), occurred at the initial site of lung metastases or in a different location. According to our data, the CT pattern of lung relapse showed high probability of recurrence at the same site of initial metastasis.

Mediastinal Germ-cell Tumors Relapse in a Male With Klinefelter Syndrome. Is Longer Surveillance Needed?

Germ cell tumors (GCTs) are a heterogeneous group of pediatric cancers. In up to one-third of male patients, a primary mediastinal location is associated with the presence of Klinefelter syndrome (KS). We describe a case of mediastinal GCT in a patient, with unacknowledged KS, that presented a relapse 7 years from diagnosis, that is, 2 years after the end of the follow-up program usually recommended for patients with GCT. There are no recommendations for screening for KS in patients with mediastinal GCT and there are no specific guidelines for surveillance of GCT in KS patients. Our experience suggests that KS should be suspected in patients with mediastinal GCT, and a longer follow-up plan should be implemented when GCT occurs in patients with KS.

Metastatic rhabdomyosarcoma with exclusive distant lymph node involvement: A European Pediatric Soft tissue sarcoma Study Group (EpSSG) report.

BACKGROUND: The prognosis of patients with metastatic rhabdomyosarcoma (RMS) is not uniformly poor. Tumors with nodal involvement beyond the first lymph node station are currently considered to have distant metastases. The aim of this study is to evaluate the characteristics and outcome of RMS patients with distal nodal involvement as the only site of metastasis. METHODS: This study included all patients with a diagnosis of RMS and distant nodal involvement as the only metastatic site, enrolled in the European Pediatric Soft tissue sarcoma Study Group (EpSSG) protocols. Treatment comprised chemotherapy, surgery, and/or radiotherapy. The main outcome measures were event-free survival (EFS) and overall survival (OS). RESULTS: A total of 22 patients (median age 7.1 years, range 1.4-16.7) fit the inclusion criteria. The extremities were the most common primary tumor site (59%). Twenty-one patients had regional and distant nodal involvement, 12 were PAX3/7-FOXO1 positive. Twenty patients had radiotherapy including 16 to the nodal metastatic area. After a median follow-up of 53.9 months (range 22.8-110.5), 15 patients remain in complete remission, seven had progressive disease or relapse, and six of them died. The 3-year EFS and OS were 67.1% (95% confidence interval [CI]: 42.9-82.9) and 71.9% (95% CI: 47.7-86.3), respectively. Patients with fusion-negative tumors had better outcomes than those with fusion-positive tumors (3-year EFS 100% vs. 46.6%; p = .04). CONCLUSION: In our experience, patients with RMS and distant lymph node involvement as the only site of metastasis present an outcome superior than other metastatic patients and comparable to patients with locoregional nodal involvement. In particular, excellent outcomes were seen in the limited number of patients with fusion-negative tumors.

NUT Carcinoma in Children and Adolescents: The Expert European Standard Clinical Practice Harmonized Recommendations.

BACKGROUND AND AIMS: Nuclear protein of the testis ( NUT ) carcinoma (NC) is a rare and highly aggressive tumor mainly occurring in adolescents and young adults, defined by the presence of a somatic NUTM1 rearrangement. The aim is to establish internationally harmonized consensus recommendations for the diagnosis and treatment of adolescents and young adults with NC in the framework of the European Reference Network for Paediatric Oncology. METHODS: The European Cooperative Study Group for Pediatric Rare Tumors developed recommendations according to the Consensus Conference Standard Operating procedure methodology and reviewed by external "experts." No evidence of level I to II exists. Recommendations were developed based on published prospective (level III), but more frequently retrospective series (level IV), case reports (level V), and personal expertise (level V). In addition, "strength" of recommendations were categorized by grading (grade A to E). RESULTS: Histology is mandatory for the diagnosis of NC, including immunolabeling with anti-NUT antibodies and molecular biology ( NUTM1 rearrangement) (level V; grade A). Treatment of NC usually combines aggressive approaches in multimodal regimens. Chemotherapy should be considered as first-line treatment (neoadjuvant vincristine-adriamycin-ifosfamide/cisplatin-adriamycin-ifsofamide or vincristine-doxorubicin-cyclophosphamide/ifosfamide-etoposide) for unresectable or metastatic tumor (ie, 3 courses), rapidly followed by local treatment (level IV; grade B). Referral to a specialized surgical oncology center is highly recommended (level V; grade A). In localized NC, a complete microscopic surgical resection should be attempted whenever and as soon as possible, followed by primary irradiation (60 to 70 Gy) and involved lymph nodes area (level IV; grade B). For head and neck tumors, a systematic neck dissection might be considered, even if N0 (level V; grade C). Adjuvant postirradiation chemotherapy is recommended, for a total of 9 to 12 courses (level IV; grade B). For first-line resected tumors, concomitant adjuvant chemotherapy to radiotherapy may be discussed (level IV; grade B). Targeted therapies and immunotherapeutic regimens should be delivered in the setting of prospective trials (level V; grade B). CONCLUSIONS: This project leads to a consensus strategy based on international experience with this very rare disease.

Ankle and Foot: Focus on Inflammatory Disease.

The ankle and foot have numerous bones and complex joints that can be affected by several types of inflammatory arthritis with different patterns and various radiologic signs, depending on the phase of the disease. Involvement of these joints is most frequently seen in peripheral spondyloarthritis and rheumatoid arthritis in adults and juvenile idiopathic arthritis in children. Although radiographs are a mainstay in the diagnostic process, ultrasonography and especially magnetic resonance imaging allow early diagnosis and are crucial diagnostic tools. Some diseases have typical features based on target populations (e.g., adults versus children, men versus women), but others may have overlapping imaging characteristics. We highlight key diagnostic features and describe appropriate investigations to guide clinicians toward the correct diagnosis and provide support during disease monitoring.

Entrapment within an ottoman storage bed: an unusual accidental asphyxial death.

Herein, we present an uncommon forensic case of death by asphyxia. The victim was a woman whose body at death scene investigation (DSI) was discovered beside an ottoman storage bed. According to the rescue team, who had moved the body before our arrival, the body was originally found in the prone position and stuck with the neck, thorax and arms within the bed. Examination of the body showed hypostasis that was mainly distributed to the face and the lower chest while sparing the neck and the upper chest. The face was markedly swollen, and the eyes were congested with blood. Dissection and histology revealed pulmonary oedema and emphysema of both lungs. Integrating circumstantial, radiology and autopsy data, it was established that the victim, while trapped between the mattress and the edge of the ottoman storage bed, died by mechanical asphyxia due to cervical-thoracic compression and postural asphyxia acting simultaneously.

It's not over until it's over: the chameleonic behavior of COVID-19 over a six-day period.

The current global outbreak of COVID-19 represents a major challenge in terms of epidemiology, contagiousness, treatment, as well as clinical and radiological behavior of this disease. Radiological imaging plays a key role in the diagnostic process and during the monitoring of the clinical conditions especially of patients with severe symptoms. According to the preliminary data collected in our tertiary center, we have documented a peculiar behavior in patients requiring endotracheal intubation who underwent seriate chest X-rays. In fact, the radiological pattern of COVID-19 patients may worsen despite a prompt amelioration after the onset of mechanical ventilation. Thus, according to our initial evidence, we recommend to perform seriate chest X-rays in the days following the onset of mechanical ventilation even if the immediate monitoring suggests an improvement. Studies on a larger scale are necessary to fully assess the findings at chest radiographs of critical, mechanically ventilated patients and their correlation with the long-term outcome.

Bone marrow edema in children: chronic nonbacterial osteomyelitis and its mimickers.

Bone marrow is a highly cellular tissue undergoing significant developmental and physiologic changes with age. Indeed, with maturation from pediatric to the adult age there is a progressive, centrifugal conversion from red to yellow bone marrow. Histological characteristics of bone marrow are reflected in MR image signal. MR is therefore extremely sensitive in detecting pathological changes which are mostly characterized by increased free water causing high signal intensity on T2. Among the numerous diseases causing bone marrow edema in children chronic nonbacterial osteomyelitis (CNO) certainly has to be mentioned. This idiopathic inflammatory disorder is characterized by nonspecific migrating symptoms like skeletal pain with phases of exacerbations and relapses with alternating acute and chronic MR signs and it is often a diagnosis of exclusion. Hence, with bone marrow edema, various features at imaging should be considered to differentiate malignancies such as osseous lymphoma, osteosarcoma, and Ewing's sarcoma as well as benign lesions like osteomyelitis, post-traumatic, or post-treatment bone marrow edema. The aim of this review is to recall the main characteristics of CNO and provide an overview of its main mimickers highlighting similarities and differences.

Neonatal cerebral ultrasound: anatomical variants and age-related diseases.

Cerebral ultrasound is a non-invasive imaging technique widely used for the assessment of brain anatomy and diseases in neonates and infants. Indeed, it allows a precise characterization of common variants such as cavum septum pellucidum or diseases like intraventricular hemorrhage. The aim of this pictorial review is to provide a comprehensive overview of the main ultrasound features of the most common cerebral anatomical variants and disorders detectable by cerebral ultrasound using an age-related approach which could support non-subspecialized radiologists.

Update on Rheumatic Diseases in Clinical Practice: Recent Concepts and Developments.

Diagnostic imaging is essential in the diagnostic process of rheumatic diseases. Given the heterogeneity of this group of diseases and the tremendous impact of novel therapeutic options, guidelines and recommendations regarding the optimal choice of the most appropriate technique/s are continuously revised and radiologists should always be up-to-date. Last, because of the continuous technological innovations, we will assist to the progressive application of advanced techniques and tools in rheumatology.

COVID-19 musculoskeletal involvement in children.

Since the early phases of the COVID-19 pandemic, it has become clear that children are affected by mild respiratory symptoms rather than the critical pneumonia typical in adults. Nevertheless, it took longer to understand that pediatric patients with SARS-COV2 may develop a severe multisystem inflammatory response (a.k.a. multisystem inflammatory syndrome in children (MIS-C)), which can include musculoskeletal symptoms, and/or arthritis and myositis independently from MIS-C. Diagnostic imaging significantly contributed to the assessment of pulmonary disease due to COVID-19 but it has been rarely applied to evaluate musculoskeletal involvement in children with or without previous rheumatic diseases. Despite the paucity of radiological literature, muscle edema at magnetic resonance and synovitis at ultrasound have been described. Further use of diagnostic imaging for children with articular and muscular symptoms due to COVID-19 is strongly encouraged.

Predictors of pulmonary sequelae after COVID-19 pneumonia: A 12-month follow-up study.

Background: Since the beginning of the SARS-CoV-2 pandemic, over 550 million people have been infected worldwide. Despite these large numbers, the long-term pulmonary consequences of COVID-19 remain unclear. Aims: The aim of this single-center observational cohort study was to identify and characterize pulmonary sequelae of COVID-19 at 12 months from hospitalization and to reveal possible predictors for the persistence of long-term lung consequences. Methods: Based on the persistence or absence of radiological changes after 12 months from hospitalization, the whole population was categorized into NOT-RECOVERED (NOT-REC) and RECOVERED (REC) groups, respectively. Clinical and pulmonary function data tests and clinical data were also collected and compared in the two groups. In the NOT-REC group, high resolution computed tomography (HRCT) images were semiquantitatively scored analyzing ground-glass opacities (GGO), interstitial thickening (IT), consolidations (CO), linear and curvilinear band opacities, and bronchiectasis for each lung lobe. Logistic regression analyses served to detect the factors associated with 12-month radiological consequences. Results: Out of the 421 patients followed after hospitalization for SARS-CoV-2 pneumonia, 347 met inclusion and exclusion criteria and were enrolled in the study. The NOT-REC patients (n = 24; 6.9%) were significantly older [67 (62-76) years vs. 63 (53-71) years; p = 0.02], more frequently current smokers [4 (17%) vs. 12 (4%); p = 0.02], and with more severe respiratory failure at the time of hospitalization [PaO2/FiO2 at admission: 201 (101-314) vs. 295 (223-343); p = 0.01] compared to REC group (n = 323; 93.1%). On multivariable analysis, being a current smoker resulted in an independent predictor for lung sequelae after 12 months from hospitalization [5.6 OR; 95% CI (1.41-22.12); p = 0.01]. Conclusion: After 12 months from hospital admission, a limited number of patients displayed persistent pulmonary sequelae with minimal extension. Being a current smoker at the time of SARS-CoV-2 infection is an independent predictive factor to lung consequences, regardless of the disease severity.

The Omental Cake Sign in Pediatric Tuberculosis.

Ultrasound and computed tomography (CT) images showing ascites and omental infiltration (omental cake sign) in a 12-year-old girl with abdominal pain and fever for two weeks. The presence of abdominal and mediastinal lymphadenopathy as well as of a pulmonary consolidation at CT suggested a diagnosis of tuberculosis which was then clinically confirmed. After treatment with ethambutol, rifampicin and isoniazid, pyrazinamide, and vitamin B6 (i.e., intensive treatment for two months followed by a continuation phase with two drugs regimen for four months) the patient fully recovered. Abdominal involvement is rare in children with tuberculosis but the presence of omental involvement together with ascites and enlarged lymph nodes at imaging may suggest this diagnosis and guide the clinicians to proper testing.

Radiomic features as biomarkers of soft tissue paediatric sarcomas: preliminary results of a PET/MR study.

BACKGROUND: Pediatric soft tissue sarcomas are rare tumors with rhabdomyosarcoma being the most frequent histotype. Diagnostic imaging plays a significant role in the evaluation of this type of tumors. Thus, aim of this study was to assess the prognostic and diagnostic value of radiomic features extracted from axial T2w images of the primary lesion in children with soft tissue sarcomas examined by PET/MR for staging. METHODS: Using an open source software, each lesion was segmented and 33 radiomic features then extracted. Factor and logistic regression analyses were applied to select highly correlating features and evaluate their prognostic role, respectively. Differences in radiomic, demographics, metabolic, and laboratory variables according to tumor grade and histotype were investigated by the Students' and Chi-square tests. In case of differences the diagnostic value of the variable/s was assessed by receiver operating curves. RESULTS: Eighteen children (11 female; mean age 7.8 ± 4.6-year-old) matched the inclusion criteria. The factor analysis allowed the selection of five highly correlating features which, according to regression analysis, did not influence the outcome (p > 0.05, each). The feature lmc1 was significantly higher in low grade lesions (p = 0.045) and showed 70.4% accuracy in classifying high grade tumors while the feature variance was significantly lower in rhabdomyosarcomas (p = 0.008) and showed 83.3% accuracy for this histotype. CONCLUSIONS: In conclusion, our preliminary results suggest that specific radiomic features may act as biomarkers of pediatric soft tissue sarcoma grade and histotype.

Tumor cellularity beyond the visible in soft tissue sarcomas: Results of an ADC-based, single center, and preliminary radiomics study.

Purpose: Soft tissue sarcomas represent approximately 1% of all malignancies, and diagnostic radiology plays a significant role in the overall management of this rare group of tumors. Recently, quantitative imaging and, in particular, radiomics demonstrated to provide significant novel information, for instance, in terms of prognosis and grading. The aim of this study was to evaluate the prognostic role of radiomic variables extracted from apparent diffusion coefficient (ADC) maps collected at diagnosis in patients with soft tissue sarcomas in terms of overall survival and metastatic spread as well as to assess the relationship between radiomics and the tumor grade. Methods: Patients with histologically proven soft tissue sarcomas treated in our tertiary center from 2016 to 2019 who underwent an Magnetic Resonance (MR) scan at diagnosis including diffusion-weighted imaging were included in this retrospective institution review board-approved study. Each primary lesion was segmented using the b50 images; the volumetric region of interest was then applied on the ADC map. A total of 33 radiomic features were extracted, and highly correlating features were selected by factor analysis. In the case of feature/s showing statistically significant results, the diagnostic accuracy was computed. The Spearman correlation coefficient was used to evaluate the relationship between the tumor grade and radiomic features selected by factor analysis. All analyses were performed applying p<0.05 as a significant level. Results: A total of 36 patients matched the inclusion criteria (15 women; mean age 58.9 ± 15 years old). The most frequent histotype was myxofibrosarcoma (16.6%), and most of the patients were affected by high-grade lesions (77.7%). Seven patients had pulmonary metastases, and, altogether, eight were deceased. Only the feature Imc1 turned out to be a predictor of metastatic spread (p=0.045 after Bonferroni correction) with 76.7% accuracy. The value -0.16 showed 73.3% sensitivity and 71.4% specificity, and patients with metastases showed lower values (mean Imc1 of metastatic patients -0.31). None of the examined variables was a predictor of the overall outcome (p>0.05, each). A moderate statistically significant correlation emerged only between Imc1 and the tumor grade (r=0.457, p=0.005). Conclusions: In conclusion, the radiomic feature Imc1 acts as a predictor of metastatic spread in patients with soft tissue sarcomas and correlates with the tumor grade.

Brief communication: Chest radiography score in young COVID-19 patients: Does one size fit all?

During the SARS-CoV-2 pandemic, chest X-Ray (CXR) scores are essential to rapidly assess patients' prognoses. This study evaluates a published CXR score in a different national healthcare system. In our study, this CXR score maintains a prognostic role in predicting length of hospital stay, but not disease severity. However, our results show that the predictive role of CXR score could be influenced by socioeconomic status and healthcare system.

Chest X-ray Does Not Predict the Risk of Endotracheal Intubation and Escalation of Treatment in COVID-19 Patients Requiring Noninvasive Respiratory Support.

Forms of noninvasive respiratory support (NIRS) have been widely used to avoid endotracheal intubation in patients with coronavirus disease-19 (COVID-19). However, inappropriate prolongation of NIRS may delay endotracheal intubation and worsen patient outcomes. The aim of this retrospective study was to assess whether the CARE score, a chest X-ray score previously validated in COVID-19 patients, may predict the need for endotracheal intubation and escalation of respiratory support in COVID-19 patients requiring NIRS. From December 2020 to May 2021, we included 142 patients receiving NIRS who had a first chest X-ray available at NIRS initiation and a second one after 48-72 h. In 94 (66%) patients, the level of respiratory support was increased, while endotracheal intubation was required in 83 (58%) patients. The CARE score at NIRS initiation was not predictive of the need for endotracheal intubation (odds ratio (OR) 1.01, 95% confidence interval (CI) 0.96-1.06) or escalation of treatment (OR 1.01, 95% CI 0.96-1.07). In conclusion, chest X-ray severity, as assessed by the CARE score, did not allow predicting endotracheal intubation or escalation of respiratory support in COVID-19 patients undergoing NIRS.

Is Empowerment of Female Radiologists Still Needed? Findings of a Systematic Review.

Considering that radiology is still a male-dominated specialty in which men make up more than two thirds of the workforce, this systematic review aimed to provide a comprehensive overview of the current role of women in radiological imaging, focusing on the main aspects such as career progression, leadership, academic practice, and perceived discrimination. Three electronic databases were searched up to 21 October 2020. To identify additional records, weekly automatic email alerts were set up on PubMed until December 2020 and reference lists of key studies and included papers were screened. Two reviewers independently performed the search, study selection, quality appraisal, data extraction, and formal narrative synthesis. In case of disagreement, a third reviewer was involved. Across the 61 included articles, women worked more often part-time and held fewer positions of power in hospitals, on editorial boards, and at the academic level (associate and full professors). Women were less often in relevant positions in scientific articles, had fewer publications, and had a lower H-index. Discrimination and sexual harassment were experienced by up to 40% and 47% of female radiologists, respectively. Our study highlights that women in radiology are still underrepresented and play a marginal role in the field, struggling to reach top and leading positions.