Retrospective and integrative analyses of molecular characteristics and their specific imaging parameters in pediatric grade 1 gliomas.

BACKGROUND: Pediatric low-grade gliomas (PLGG) are the most common brain tumors diagnosed during childhood and represent a heterogeneous group associating variable molecular abnormalities. To go further and develop specific statistical patterns between tumor molecular background, imaging features, and patient outcome, a retrospective study was performed in a group of non-neurofibromatosis type 1 (non-NF1) grade 1 PLGGs. PATIENTS AND METHODS: Seventy-eight children, followed from 2004 to 2017, were retrospectively reported. In this population, we analyzed radiological and molecular parameters. Their therapeutic management comprised surgery or surgery plus chemotherapies. RESULTS: Considering all 78 patients, 59 had only a surgical removal and 19 patients were treated with postoperative chemotherapy. Twelve progressions were reported in the partially resected and chemotherapeutic groups, whereas four deaths occurred only in the highly treated patients. As expected, in the global cohort, PLGG with BRAF p.V600E and/or CDKN2A loss exhibited poor outcomes and we evidenced significant associations between those molecular characteristics and their imaging presentation. In the chemo-treated patients, when associating initial and 6-month magnetic resonance imaging (MRI) parameters to the molecular features, the good risk situations were significantly linked to the presence of a large tumor cyst at diagnosis and the appearance during treatment of a higher cystic proportion that we called cystic conversion. CONCLUSION: So, additionally to the presence of BRAF p.V600E or CDKN2A deletion in grade 1 PLGGs, the absence on diagnostic MRI of cystic parts and/or cystic conversion at 6-month chemotherapy were significantly linked to a worst prognosis and response to treatment. These imaging features should be considered as prognostic markers in future PLGG studies.

Improved stenosis outcome in stroke-free sickle cell anemia children after transplantation compared to chronic transfusion.

We report here the 3-year stenosis outcome in 60 stroke-free children with sickle cell anaemia (SCA) and an abnormal transcranial Doppler history, enrolled in the DREPAGREFFE trial, which compared stem cell transplantation (SCT) with standard-care (chronic transfusion for 1-year minimum). Twenty-eight patients with matched sibling donors were transplanted, while 32 remained on standard-care. Stenosis scores were calculated after performing cerebral/cervical 3D time-of-flight magnetic resonance angiography. Fourteen patients had stenosis at enrollment, but only five SCT versus 10 standard-care patients still had stenosis at 3 years. Stenosis scores remained stable on standard-care, but significantly improved after SCT (P = 0·006). No patient developed stenosis after SCT, while two on standard-care did, indicating better stenosis prevention and improved outcome after SCT.

Imaging features of undifferentiated embryonal sarcoma of the liver: a series of 15 children.

BACKGROUND: Undifferentiated embryonal sarcoma of the liver is a rare malignant mesenchymal tumour occurring mostly in children ages 6-10 years. The discrepancy between its solid appearance on US and cystic-like appearance on CT has been described. OBJECTIVE: To study the imaging particularities and similarities among our cases of undifferentiated embryonal sarcoma and to report the errors in initial diagnoses. MATERIALS AND METHODS: We conducted a retrospective study of 15 children with undifferentiated embryonal sarcoma diagnosed or referred to our hospital during 1997-2015 and analysed the clinical, biological and imaging data. RESULTS: We identified eight boys and seven girls ages 9 months to 14 years. Ten children presented with abdominal pain. Alpha-fetoprotein was slightly increased in one. Initial US and CT had been performed for all, while additional MRI had been done in two children. Initial CT demonstrated a hypoattenuated mass in all. Rupture was seen in five and intratumoural bleeding in seven children. Tumour volumes reduced during neoadjuvant chemotherapy in 10 children. CONCLUSION: Undifferentiated embryonal sarcoma might be suggested in a non-secreting unifocal tumour with well-defined borders, fluid-filled spaces on US, hypoattenuation and serpiginous vessels on CT, and if there are signs of internal bleeding or rupture on CT or MRI.

Pitfalls in the surgical management of undifferentiated sarcoma of the liver and benefits of preoperative chemotherapy.

AIM: Undifferentiated sarcoma of the liver (USL) is the third commonest malignant liver tumor in children. The aim of our study is to evaluate the outcome of this rare entity according to the quality of the surgical resection and the compliance to the European pediatric soft tissue sarcoma group guidelines. PATIENTS AND METHODS: We conducted a monocentric review of patients referred to our department with a definitive pathologic diagnosis of USL between 1997 and 2013. We looked at the diagnosis and management pitfalls, surgical technique, and outcomes. Results are expressed as median (range). RESULTS: There were 13 patients (M/F=7:6=1.1). Age at presentation was 8 years (range, 11 months-16 years). Of the 13 patients, 10 patients (69%) presented with abdominal pain, 5 (38%) with tumoral bleeding, and 2 (15%) with peritoneal rupture. All lesions were unique, nonmetastatic, and heterogeneous with cystic components measuring 14 (6-19) cm. Six (46%) patients had an upfront surgery: five because of wrong clinical diagnosis (three query mesenchymal hamartoma, one spontaneous peritoneal bleeding, and one cystic lymphangioma), and one because of rapid enlargement of the mass. Seven (54%) patients (including one with tumoral bleeding) received neoadjuvant chemotherapy, and had their tumor diameter decreasing by 40% (range, 0-60%). Final surgery consisted of seven right hepatectomies; one right extended hepatectomy; three mesohepatectomies; two left hepatectomies. There were three incomplete resection in the upfront surgery group versus none in the neoadjuvant chemotherapy group. The degree of tumor necrosis after chemotherapy ranged from 95 to 100%. Surgical complications included the following: liver transplantation (LT) following a Budd-Chiari syndrome after a mesohepatectomy, one biliary ducts injury treated by Roux-en-Y loop. All patients received the postoperative chemotherapy according to the European protocol. One of seven patients (14%) with neoadjuvant chemotherapy underwent radiotherapy for rupture at diagnosis versus three of six patients (50%) with upfront surgery: one for rupture at diagnosis and two for rupture during upfront surgery. One patient (17%) with upfront surgery had local recurrence at 2 years after initial surgery, and is in second complete remission 1 year after a redo surgery. All patients are alive at a median 34 months (range, 5-134) follow-up. CONCLUSION: USL presents with painful mixed cystic and solid liver mass. If misdiagnosed and mistreated (enucleation or unroofing), the usual good outcome of this malignancy could be impaired. Preoperative chemotherapy is recommended.

Necrotizing pneumonia in children: report of 41 cases between 2006 and 2011 in a French tertiary care center.

Forty-one children hospitalized for necrotizing pneumonia were retrospectively analyzed. Necrotizing pneumonia represented 0.8% of community-acquired pneumonia and 6% of hospitalized community-acquired pneumonia. The chest radiograph revealed necrosis on admission in onethird of cases. Twenty-one cases (51%) were documented, including 13 Staphylococcus aureus, all Panton-Valentine leukocidin positive, 7 Streptococcus pneumoniae and 1 Fusobacterium nucleatum.