The role of pediatric oncologist in prenatal diagnosis: A 10-year retrospective study at Assistance Publique Hôpitaux de Marseille (AP-HM).

Solid tumors or predisposition syndromes are increasingly suspected before birth. However optimal management and outcomes remain unclear. We have performed a ten-year retrospective study of oncologic indications of prenatal diagnosis in public hospitals in Marseille. Data were obtained from prenatal diagnosis center and hospital imaging databases and pediatric oncology department files. Fifty-one cases were identified, 40 with mass: adrenal 17, sacrococcygeal 9, cardiac 7, abdominal 4, ovarian 1, cervical 2; 8 with developmental abnormalities (omphalocele 4, macroglossia 4), 3 WITH familial predisposition syndromes (familial rhabdoid 2, Li-Fraumeni 1). Median detection time was 30 week. Termination of pregnancy was decided for 9 fetuses (4 cardiac lesions and suspected tuberous sclerosis, 2 sacrococcygeal tumors, 1 Beckwith-Wiedemann Syndrome, 2 SMARCB1 mutations. Preterm birth occurred in 8 cases. Eleven newborns (26,1%) required intensive care (8 for mechanical complications). Of of 17 adrenal mass ES, 4 disappeared before birth and 5 before one year. Seventeen newborns underwent surgery: 13 masses (teratoma 7, myelomeningocele 2, cystic nephroma 1, neuroblastoma 2), 4 omphaloceles, one biopsy. Surgery performed after one year for incomplete regression identified 1 neuroblastoma, 2 bronchogenic cysts and 2 nonmalignant masses. Three newborns received chemotherapy. Except one patient with BWS who died of obstructive apnea, all children are alive disease free with a median follow-up of 60 months [9-131 months]. Twelve have sequelae. Various solid tumors and cancer predisposition syndromes can be detected before birth. A multidisciplinary collaboration is strongly recommended for optimal management before and after birth.

Technical feasibility and correlations between shear-wave elastography and histology in kidney fibrosis in children.

BACKGROUND: Ultrasound elastography has been suggested for assessing organ fibrosis. OBJECTIVE: To study the feasibility of shear-wave elastography in children with kidney disease and the correlation between elasticity and kidney fibrosis in order to reduce the indications for kidney biopsy and its complications. MATERIALS AND METHODS: Four operators measured kidney elasticity in children with kidney diseases or transplants, all of whom also had a renal biopsy. We assessed the feasibility and the intraobserver variability of the elasticity measurements for each probe used and each kidney explored. Then we tested the correlation between elasticity measurements and the presence of fibrosis. RESULTS: Overall, we analyzed 95 children and adolescents, 31 of whom had renal transplant. Measurements with the convex probe were possible in 100% of cases. Linear probe analysis was only possible for 20% of native kidneys and 50% of transplants. Intraobserver variabilities ranged from moderate to high, depending on the probe and kidney studied. Elasticity was higher with the linear probe than with the convex probe (P<0.001 for left kidney and P=0.03 for right kidney). Measurements did not differ from one kidney to another in the same child. Elasticity and fibrosis were both higher in transplant patients (P=0.02 with convex probe; P=0.01 with linear probe; P=0.04 overall). There was no correlation between elasticity and fibrosis. CONCLUSION: Of the devices used in this work, kidney elastography was more accurately analyzed with a convex probe. Our study did not identify any correlation between elasticity and kidney fibrosis.

Diffusion-weighted imaging in differentiating mid-course responders to chemotherapy for long-bone osteosarcoma compared to the histologic response: an update.

BACKGROUND: Diffusion-weighted imaging (DWI) has been described to correlate with tumoural necrosis in response to preoperative chemotherapy for osteosarcoma. OBJECTIVE: To assess the accuracy of DWI in evaluating the response to neoadjuvant chemotherapy at the mid-course treatment of long-bone osteosarcoma and in predicting survival. MATERIALS AND METHODS: We conducted a prospective single-centre study over a continuous period of 11 years. Consecutive patients younger than 20 years treated with a neoadjuvant regimen for peripheral conventional osteosarcoma were eligible for inclusion. Magnetic resonance imaging (MRI) with DWI was performed at diagnosis, and mid- and end-course chemotherapy with mean apparent diffusion coefficients (ADC) calculated at each time point. A percentage less than or equal to 10% of the viable residual tissue at the histological analysis of the surgical specimen was defined as a good responder to chemotherapy. Survival comparisons were calculated using the Kaplan-Meier method. Uni- and multivariate analyses with ADC change were performed by Cox modelling. This is an expansion and update of our previous work. RESULTS: Twenty-six patients between the ages of 4.8 and 19.6 years were included, of whom 14 were good responders. At mid-course chemotherapy, good responders had significantly higher mean ADC values (P=0.046) and a higher increase in ADC (P=0.015) than poor responders. The ADC change from diagnosis to mid-course MRI did not appear to be a prognosticator of survival and did not impact survival rates of both groups. CONCLUSION: DWI at mid-course preoperative chemotherapy for osteosarcoma should be considered to evaluate the degree of histological necrosis and to predict survival. The anticipation of a response to neoadjuvant treatment by DWI may have potential implications on preoperative management.

SFCE METRO-01 four-drug metronomic regimen phase II trial for pediatric extracranial tumor.

OBJECTIVE: To investigate the antitumor activity of a four-drug metronomic chemotherapy (MC) regimen in relapsed/progressing pediatric extracranial solid tumors (EST). The primary objective was clinical benefit (complete response /partial response/stable disease [SD]) after two cycles of therapy (four months). METHODS: Patients aged ≥4 to 25 years with progressing EST and adequate organ function were eligible. Treatment consisted of an eight-week cycle of oral celecoxib b.i.d., weekly vinblastine, and oral cyclophosphamide for three weeks alternating with oral methotrexate for three weeks, with a two-week rest. The Kepner-Chang two-stage model was used with 10 patients in the first stage. If primary objective was reached in two or more patients, eight additional patients were included according to four groups: neuroblastoma (NBL), soft-tissue sarcoma (STS), bone sarcoma (BS), and miscellaneous (Misc.). RESULTS: Forty-four patients were evaluable. The NBL cohort could be expanded to 18 patients: 4 of 18 patients stabilized with MC treatment for 6 (n = 1) and 12 (n = 3) months. In STS, two of seven patients (metastatic hemangioendothelioma and angiosarcoma) had SD for > 2 cycles. One of nine Misc. (metastatic myoepithelial carcinoma) had SD for one year. All patients with BS had progressive disease. One-year progression-free survival of the whole cohort was 6.8% and one-year overall survival was 55.3%. Grade 3 toxicity occurred in 18 patients and grade 4 in 15 patients. The most frequent toxicity was hematologic, predominantly neutropenia. CONCLUSIONS: This MC has no activity in BS and limited though interesting activity in NBL with some patients being stable for > 1 year. It is not possible to conclude activity in STS and Misc.

Can diffusion weighting replace gadolinium enhancement in magnetic resonance enterography for inflammatory bowel disease in children?

BACKGROUND: Contrast-enhanced MRI is often used for diagnosis and follow-up of children with inflammatory bowel disease. OBJECTIVE: To compare the accuracy of diffusion-weighted MRI (DWI) to contrast-enhanced MRI in children with known or suspected inflammatory bowel disease. MATERIALS AND METHODS: This retrospective, consecutive study included 55 children. We used ileo-colonoscopy and histology as the reference standard from the terminal ileum to the rectum, and contrast-enhanced MRI as the reference standard proximal to the terminal ileum. DWI and contrast-enhanced MRI sequences were independently reviewed and compared per patient and per segment to these reference standards and to the follow-up for each child. RESULTS: We obtained endoscopic data for 340/385 colonic and ileal segments (88%). The rate of agreement per segment between DWI and endoscopy was 64%, and the rate of agreement between contrast-enhanced MRI and endoscopy was 59%. Per patient, sensitivity and specificity of bowel wall abnormalities as compared to the endoscopy were 87% and 100% for DWI, and 70% and 100% for contrast-enhanced MRI, respectively. Positive and negative predictive values were, respectively, 100% and 57% for DWI, and 96% and 41% for contrast-enhanced MRI. The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of DWI compare to contrast-enhanced MRI in the segments proximal to the terminal ileum were 90%, 98%, 90%, 98% and 96%, respectively. CONCLUSION: The diagnostic performance of DWI is competitive to that of contrast-enhanced MRI in children with known or suspected inflammatory bowel disease.

A prospective pilot study: can the biliary tree be visualized in children younger than 3 months on Magnetic Resonance Cholangiopancreatography?

BACKGROUND: Magnetic resonance cholangiopancreatography (MRCP) could aid in the diagnosis of biliary atresia, a hepatic pathology with thin, irregular or interrupted biliary ducts. There is little published evidence of MRCP appearances in normal neonates and young infants. OBJECTIVE: To assess the use of MR cholangiopancreatography in visualizing the biliary tree in neonates and infants younger than 3 months with no hepatobiliary disorder, and to assess this visibility in relationship to the child's age, weight, and sedation and fasting states. MATERIALS AND METHODS: Between December 2008 and October 2010 our department performed MRI of the brain, orbits and face on 16 full-term neonates and infants. Each child was younger than 3 months (90 days) and without any hepatobiliary disorders. The children were scanned with a respiratory-gated 0.54 × 0.51 × 0.4-mm(3) 3-D MRCP sequence. We used a reading grid to assess subjectively the visibility of the extrahepatic bile ducts along with extrahepatic bile duct confluence. The visibility of the extrahepatic bile duct confluence was assessed against age, weight, and sedation and fasting states. RESULTS: The extrahepatic bile duct confluence was seen in 10 children out of 16 (62.5%). In the neonate sub-group (corrected age younger than 30 days), the MRCP was technically workable and the extrahepatic bile duct confluence was seen in four cases out of eight (50%). This visualization was up to 75% in the subgroup older than 30 days. However, statistically there was no significant difference in visibility of the extrahepatic bile duct confluence in relationship to age, weight or MRCP performance conditions (feeding, fasting or sedation). CONCLUSION: The complete normal biliary system (extrahepatic bile duct confluence included) is not consistently visualized in infants younger than 3 months old on non-enhanced MRCP. Thus the use of MRCP to exclude a diagnosis of biliary atresia is compromised at optimal time of surgery.

Relevance of Routine Abdominopelvic Ultrasound in Suspected Child Abuse in Children Under 2 years of Age: Review of 15 years of Experience.

In France, the current recommendation is to perform a routine abdominopelvic ultrasound in any child under 2 years of age who is suspected to have been abused. We retrospectively studied the relevance of this practice in our center over the past fifteen years. This was a descriptive, retrospective study of all children under 2 years of age who had been subject to suspected abuse. Abdominal images and reports were reviewed and cross-referenced with possible clinical and biological signs. Four hundred and five children were included between 2006 and 2020, of whom 296 underwent abdominal imaging (2 initial abdominopelvic CT scans, 4 ultrasounds followed by CT scans, and 290 ultrasounds alone). Four examinations revealed traumatic abnormalities related to abuse. These four children all had clinical or biological anomalies. In the absence of clinical or biological signs, no imagery showed any abnormality related to abuse.

Chronic inflammatory diseases of the bowel: diagnosis and follow-up.

Approximately one fourth of cases of inflammatory bowel disease (IBD) occur during childhood and children are more prone than their adult counterparts to have severe disease at presentation. To investigate these diseases MR imaging is no longer an emerging tool. Numerous reviews and articles have been published on this topic underlying the advances of imaging but also the complexity and the financial impact on management of such diseases. In children it seems reasonable to consider US as the first imaging examination to perform, especially when the diagnosis of IBD is unknown. However, we believe that recent and future technical progress, especially the ability of MR to display reproducible data, and the need for gold standard evaluation of new medical therapies will increase the role of MR enterography.

Sonographic diagnosis of a common pancreaticobiliary channel in children.

BACKGROUND: A common pancreaticobiliary channel is a very rare condition, but its diagnosis is of paramount importance since it can lead to complications that can be prevented. OBJECTIVE: To illustrate the sonographic diagnosis of a common pancreaticobiliary channel in children referred for abdominal pain or jaundice. MATERIALS AND METHODS: Four children were diagnosed by ultrasonography and the diagnosis was subsequently confirmed by MRI. RESULTS: Sonography demonstrated a pancreaticobiliary junction located in the pancreatic head above the sphincter of Oddi. This rare congenital anomaly was confirmed in all patients by MRI. CONCLUSION: A common pancreaticobiliary channel can be diagnosed by sonography. Nevertheless, our experience is limited, and although sonography can provide an alert and can assist management, it cannot yet replace MRI.