Risk of subsequent primary oral cancer in a cohort of 69,460 5-year survivors of childhood and adolescent cancer in Europe: the PanCareSurFup study

Background Survivors of childhood cancer are at risk of subsequent primary malignant neoplasms (SPNs), but the risk for rarer types of SPNs, such as oral cancer, is uncertain. Previous studies included few oral SPNs, hence large-scale cohorts are required to identify groups at risks. Methods The PanCareSurFup cohort includes 69,460 5-year survivors of childhood cancer across Europe. Risks of oral SPNs were defined by standardised incidence ratios (SIRs), absolute excess risks and cumulative incidence. Results One hundred and forty-five oral SPNs (64 salivary gland, 38 tongue, 20 pharynx, 2 lip, and 21 other) were ascertained among 143 survivors. Survivors were at 5-fold risk of an oral SPN (95% CI: 4.4-5.6). Survivors of leukaemia were at greatest risk (SIR = 19.2; 95% CI: 14.6-25.2) followed by bone sarcoma (SIR = 6.4, 95% CI: 3.7-11.0), Hodgkin lymphoma (SIR = 6.2, 95% CI: 3.9-9.9) and soft-tissue sarcoma (SIR = 5.0, 95% CI: 3.0-8.5). Survivors treated with radiotherapy were at 33-fold risk of salivary gland SPNs (95% CI: 25.3-44.5), particularly Hodgkin lymphoma (SIR = 66.2, 95% CI: 43.6-100.5) and leukaemia (SIR = 50.5, 95% CI: 36.1-70.7) survivors. Survivors treated with chemotherapy had a substantially increased risk of a tongue SPN (SIR = 15.9, 95% CI: 10.6-23.7). Conclusions Previous radiotherapy increases the risk of salivary gland SPNs considerably, while chemotherapy increases the risk of tongue SPNs substantially. Awareness of these risks among both health-care professionals and survivors could play a crucial role in detecting oral SPNs early.</p

Trends in treatment of childhood cancer and subsequent primary neoplasm risk

BACKGROUND: The aim of the study was to investigate long-term risk and spectrum of subsequent neoplasm (SN) in childhood cancer survivors and to identify how trends in therapy influenced cumulative incidence of SN. PATIENTS AND METHODS: The population-based cohort comprises 3271 childhood cancer patients diagnosed in Slovenia aged ≤ 18 years between 1st January 1961 and 31st December 2013 with a follow-up through 31st December 2018. Main outcome measures are standardised incidence ratios (SIRs), absolute excess risks (AERs), and cumulative incidence of SN. RESULTS: After median follow-up time of 21.5 years for 5-year survivors, 230 patients experienced 273 SN, including 183 subsequent malignant neoplasm (SMN), 34 meningiomas and 56 nonmelanoma skin cancers. 10.5% patients received radiotherapy only, 31% chemotherapy only, 26.9% a combination of chemotherapy and radiotherapy and 16.1% surgery only. The overall SIR was almost 3 times more than expected (SIR 2.9), with survivors still at 2-fold increased risk after attained age 50 years. The observed cumulative incidence of SMN at 30-year after diagnosis was significantly lower for those diagnosed in 1960s, compared with the 1970s and the 1980s (P heterogeneity < 0.001). Despite reduced use of radiotherapy over time, the difference in cumulative incidence for the first 15 years after diagnosis was not significant for patients treated before or after 1995 (p = 0.11). CONCLUSIONS: Risks of developing a SMN in this study are similar to other European population-based cohorts. The intensity of treatment peaked later and use of radiotherapy declined slower compared to high income countries, making continuous surveillance even more important in the future

Treatment of rhabdomyosarcoma in children and adolescent from four low health expenditures average rates countries

Survival of children with cancer in Eastern and Central Europe is 10–20% lower than in high income European countries. We evaluated outcome of children and adolescents with rhabdomyosarcoma (RMS) in Slovenia, Croatia, Slovakia and in Romania

PRIMARY LUNG CARCINOMA IN CHILDREN AND ADOLESCENTS: AN ANALYSIS OF THE EUROPEAN COOPERATIVE STUDY GROUP FOR PEDIATRIC RARE TUMORS (EXPERT)

Background and Aims: Primary lung carcinomas are very rare childhood tumors with an incidence of <2/1,000,000 per year as defined by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT). They represent a challenge to treating physicians as there are few reports on these diseases at this age and no specific recommendations are available. This collaborative analysis of the EXPeRT group was conducted to improve knowledge about the treatment and prognosis of primary lung carcinomas in pediatric patients. Methods:Aretrospective European analysis of all pediatric patients (0- 18 years) with primary lung carcinomas prospectively collected in the EXPeRT databases between 2000 and 2021 was performed. Clinical data including outcomes were analyzed. Results: Thirty-eight patientswere identified with a median age of 12.8 years at diagnosis (range 0 to 17 years). Mucoepidermoid carcinoma (MEC) was the most common entity (n=20), followed by adenocarcinoma (n=12), squamous cell carcinoma (SCC; n=4), adenosquamous carcinoma (n=1) and small-cell lung cancer (n=1). Lymph node metastases occurred rarely in patients with MEC (2 cases), and 19 patients achieved durable remission after surgical resection only. One patient with MEC died after progression of metastasis. Patients with histology other thanMEC often presentedwith advanced disease (stage IV in 14 of 18 cases) and needed multimodality treatment. They had a combined survival rate of 44%. While all patients with SCC died, the 12 patients with adenocarcinoma had a survival rate of 50%. Conclusions: Primary lung carcinoma occur rarely in children. While patients with MEC have a favorable outcome with a survival rate of 95%, patients with other lung carcinoma entities have an unfavorable outcome despite multimodality treatment strategies. This analysis will help propose consensus guidelines for diagnosis and therapy