Adrenocortical tumours in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations

Adrenocortical tumours (ACTs) are rare during childhood. A complete surgical resection provides the best chance of cure, but the role and efficacy of the adjuvant therapy are still controversial. Various histologic criteria of malignancy for ACTs adopted in children do not facilitate comparative studies and are not completely shared. Therefore, a sharp demarcation between benign and malignant lesions has not been recognised, making it difficult to identify who potentially needs perioperative therapy. This manuscript presents the internationally harmonised recommendations for the diagnosis and treatment of ACTs in children and adolescents, established by the European Cooperative Study Group for Paediatric Rare Tumours (EXPeRT) group within the EU-funded project PARTNER (Paediatric Rare Tumours Network - European Registry)

Excess healthcare expenditure in adults treated for solid cancer in childhood: a cohort study in France

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Care management for foreign children, adolescents, young adults with cancer, and their families

International audienceBACKGROUND: Little is known about care management for foreign patients in pediatric oncology in European centers. We aimed to describe care given to children, adolescents, and young adults who came to France for cancer treatment, and to determine whether their geographical origin had an influence on decision making. PROCEDURE: We conducted a monocentric retrospective study on all foreign patients aged 0-25 years and hospitalized for at least one night in Institut Curie (Paris, France) from 2009 to 2013. We analyzed the potential advantages of receiving treatment in France as well as their social and familial consequences. RESULTS: A total of 93 foreign patients' files were retrieved. Most of these patients came from Africa (70%). In accord with the specific expertise of the institution, retinoblastoma was the most frequent tumor type (39%). An antitumor treatment had already been administrated in the native country in 44% of patients. We considered that 66% of patients received a significant medical advantage from care in our institution. The treatment provided in France was considered impossible in the native country in 44% of cases. The social and familial impact on the patients' families was high (59%). Almost all patients (96%) received the treatment that would have been proposed to their French counterparts. CONCLUSIONS: There were notable medical advantages for foreign patients who come to France for their oncologic treatment despite important familial consequences. Patients' geographical origin did not have an influence on medical decisions

Increased Cardiac Risk After a Second Malignant Neoplasm Among Childhood Cancer Survivors, a FCCSS Study

International audienceBACKGROUND Advances in cancer treatment have significantly improved childhood cancer survival, with five-year survival exceeding 80% in most European and North American countries today. The adult population of childhood cancer survivors (CCS) exceeds 300,000 people in the USA alone, and is growing. Many late-effects have been observed at a higher rate than the general population. Cardiac disease (CD) and second malignant neoplasms (SMN) are among the most serious and life-threatening late adverse effects experienced by CCS. Previous work has identified important risk factors of CD, but the impact of other late-effects as SMN have been ignored. We have studied the effect of a SMN on both the cumulative incidence and the instantaneous risk of CD accounting for the competing risk of death

Increased Cardiac Risk After a Second Malignant Neoplasm Among Childhood Cancer Survivors, a FCCSS Study

International audienceBACKGROUND Advances in cancer treatment have significantly improved childhood cancer survival, with five-year survival exceeding 80% in most European and North American countries today. The adult population of childhood cancer survivors (CCS) exceeds 300,000 people in the USA alone, and is growing. Many late-effects have been observed at a higher rate than the general population. Cardiac disease (CD) and second malignant neoplasms (SMN) are among the most serious and life-threatening late adverse effects experienced by CCS. Previous work has identified important risk factors of CD, but the impact of other late-effects as SMN have been ignored. We have studied the effect of a SMN on both the cumulative incidence and the instantaneous risk of CD accounting for the competing risk of death

Increased Cardiac Risk After a Second Malignant Neoplasm Among Childhood Cancer Survivors, a FCCSS Study

International audienceBACKGROUND Advances in cancer treatment have significantly improved childhood cancer survival, with five-year survival exceeding 80% in most European and North American countries today. The adult population of childhood cancer survivors (CCS) exceeds 300,000 people in the USA alone, and is growing. Many late-effects have been observed at a higher rate than the general population. Cardiac disease (CD) and second malignant neoplasms (SMN) are among the most serious and life-threatening late adverse effects experienced by CCS. Previous work has identified important risk factors of CD, but the impact of other late-effects as SMN have been ignored. We have studied the effect of a SMN on both the cumulative incidence and the instantaneous risk of CD accounting for the competing risk of death

Increased Cardiac Risk After a Second Malignant Neoplasm Among Childhood Cancer Survivors, a FCCSS Study

International audienceBACKGROUND Advances in cancer treatment have significantly improved childhood cancer survival, with five-year survival exceeding 80% in most European and North American countries today. The adult population of childhood cancer survivors (CCS) exceeds 300,000 people in the USA alone, and is growing. Many late-effects have been observed at a higher rate than the general population. Cardiac disease (CD) and second malignant neoplasms (SMN) are among the most serious and life-threatening late adverse effects experienced by CCS. Previous work has identified important risk factors of CD, but the impact of other late-effects as SMN have been ignored. We have studied the effect of a SMN on both the cumulative incidence and the instantaneous risk of CD accounting for the competing risk of death

Nasopharyngeal carcinoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations

AbstractNasopharyngeal carcinoma (NPC) is a rare pediatric tumor. Collaborative studies performed over the last decades showed improved results compared to historical data, but standardized guidelines for diagnosis and management of pediatric NPC are still unavailable. This study presents a European consensus guideline for the diagnosis and treatment of pediatric NPC developed by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT). Main recommendations include induction chemotherapy with cisplatin and 5‐flurouracil, concomitant chemoradiotherapy in advanced disease, and to consider maintenance treatment with interferon beta (IFN‐β) for selected high‐risk patients. Dose adjustments of radiotherapy based on response to induction chemotherapy may decrease the rates of long‐term treatment‐related complications that affect most of the survivors

Risk of Renal or Urinary Related Hospitalization in Survivors of Childhood Cancer: Results from the French Childhood Cancer Survivor Study

International audienceBACKGROUND: Hospitalization rates can be used as an indirect indicator of the burden and severity of adverse health outcomes in childhood cancer survivors (CCS). We aimed to determine the long-term risks of hospitalization related to renal and urinary diseases among 5-year CCS. METHODS: The French Childhood Cancer Survivor Study cohort was linked with data from the French National Healthcare System database, which enabled the identification of hospitalizations related to renal or urinary diseases. Clinical and detailed treatment data were collected from medical records. Dose-volume histograms were estimated for all patients treated with radiotherapy. Standardized Hospitalization Ratios and absolute excess risks (AER) were calculated. Relative risks were estimated using Poisson regression. RESULTS: A total of 5,498 survivors were followed for 42,118 person-years (PY). Survivors experience 2.9 times more renal hospitalizations than expected in the general population, with an AER of 21.2/10,000 PY. Exposing more than 10% of the kidneys' volume to at least 20 Gray increases the risk of being hospitalized for renal causes by 2.2 (95% confidence interval, 1.3-3.6). Nephrectomized survivors treated with high doses of ifosfamide (>60 g/m²) have an extremely high risk of hospitalization for renal causes. Patients with comorbidities have about a 3-fold higher risk, and nephrectomized patients a 2-fold higher risk of being hospitalized for renal causes compared with other subjects. In the case of hospitalization for urinary causes, treatment by anthracycline administration was found to be associated with an almost 2-fold higher risk of hospitalization compared with the general population. CONCLUSIONS: These results support the need for careful monitoring of long-term renal diseases in survivors who have undergone nephrectomy, those treated with high doses of radiation (≥20 Gy) even to small volumes of the kidneys, and those with predisposing risk factors. IMPACT: This study provides new evidence with potential impact on surveillance guidelines related to dose-volume indicators associated with renal toxicity

MMP2 as an independent prognostic stratifier in oral cavity cancers

Background Around 25% of oral cavity squamous cell carcinoma (OCSCC) are not controlled by the standard of care, but there is currently no validated biomarker to identify those patients. Our objective was to determine a robust biomarker for severe OCSCC, using a biology-driven strategy. Patients and methods Tumor and juxtatumor secretome were analyzed in a prospective discovery cohort of 37 OCSCC treated by primary surgery. Independent biomarker validation was performed by RTqPCR in a retrospective cohort of 145 patients with similar clinical features. An 18-gene signature (18 G) predictive of the response to PD-1 blockade was evaluated in the same cohort. Results Among 29 deregulated molecules identified in a secretome analysis, including chemokines, cytokines, growth factors, and molecules related to tumor growth and tissue remodeling, only soluble MMP2 was a prognostic biomarker. In our validation cohort, high levels of MMP2 and CD276, and low levels of CXCL10 and STAT1 mRNA were associated with poor prognosis in univariate analysis (Kaplan-Meier). MMP2 (p = .001) and extra-nodal extension (ENE) (p = .006) were independent biomarkers of disease-specific survival (DSS) in multivariate analysis and defined prognostic groups with 5-year DSS ranging from 36% (MMP2highENE+) to 88% (MMP2lowENE-). The expression of 18 G was similar in the different prognostic groups, suggesting comparable responsiveness to anti-PD-1. Conclusion High levels of MMP2 were an independent and validated prognostic biomarker, surpassing other molecules of a large panel of the tumor and immune-related processes, which may be used to select poor prognosis patients for intensified neoadjuvant or adjuvant regimens