Nuchal skinfold thickness in pediatric brain tumor patients

BACKGROUND: Severe obesity and tumor relapse/progression have impact on long-term prognosis in pediatric brain tumor patients. METHODS: In a cross-sectional study, we analyzed nuchal skinfold thickness (NST) on magnetic-resonance imaging (MRI) follow-up monitoring as a parameter for assessment of nuchal adipose tissue in 177 brain tumor patients (40 World Health Organization (WHO) grade 1–2 brain tumor; 31 grade 3–4 brain tumor; 106 craniopharyngioma), and 53 healthy controls. Furthermore, body mass index (BMI), waist-to-height ratio, caliper-measured skinfold thickness, and blood pressure were analyzed for association with NST. RESULTS: Craniopharyngioma patients showed higher NST, BMI, waist-to-height ratio, and caliper-measured skinfold thickness when compared to other brain tumors and healthy controls. WHO grade 1–2 brain tumor patients were observed with higher BMI, waist circumference and triceps caliper-measured skinfold thickness when compared to WHO grade 3–4 brain tumor patients. NST correlated with BMI, waist-to-height ratio, and caliper-measured skinfold thickness. NST, BMI and waist-to-height ratio were associated with increased blood pressure. In craniopharyngioma patients with hypothalamic involvement/lesion or gross-total resection, rate and degree of obesity were increased. CONCLUSIONS: NST could serve as a novel useful marker for regional nuchal adipose tissue. NST is highly associated with body mass and waist-to-height ratio, and easily measurable in routine MRI monitoring of brain tumor patients

Outcome after proton beam therapy versus photon-based radiation therapy in childhood-onset craniopharyngioma patients — results of KRANIOPHARYNGEOM 2007

Background: Proton beam therapy (PBT) is being increas16ingly used to treat residual craniopharyngioma (CP) after hypothalamus-sparing surgery. Compared to photon-based radiation therapy (XRT) with PBT, less irradiation in the penumbra reduces the scattered dose to critical organs neighboring but outside the area of treatment, minimizing the risk of sequelae. Patients and methods: Between 2007 and 2019, 99 of 290 (34%) childhood-onset CP patients recruited in KRANIOPHARYNGEOM 2007 received external radiation therapy (RT) (65% PBT, 35% XRT). Outcome was analyzed in terms of survival, endocrinological and anthropometric parameters (BMI and height SDS), quality of life (QoL using PEDQOL), and functional capacity (FMH) with special regard to irradiation technique. Results: PBT became predominant (used in 43% and 72% of all irradiated patients registered within the first and second halves of the recruitment period, between 2008 and 2013 and 2013 and 2018, respectively). Five-year event-free survival rates after PBT or XRT were comparable (92% ± 4% vs. 91% ± 4%, p = 0.42) and higher than for the whole cohort since diagnosis, including non-RT patients (37% ± 4%). Radiation doses to the hypothalamus and pituitary did not differ between PBT and XRT. Endocrine deficits due to disturbances of the hypothalamic-pituitary axis (HPA) were already common before irradiation. During the first 5 years after CP diagnosis/RT, no differences between PBT, XRT, and non-RT CP patients concerning functional capacity and anthropometric parameters have been obtained. Only for the PEDQOL domain “physical function”, parental-assessed QoL was lower 12 months after PBT versus XRT or non-RT patients. Conclusion: QoL, functional capacity, degree of obesity, and endocrinopathy varied over time from diagnosis, but by 5 years, there was no significant difference between PBT and XRT upfront or delayed, nor was there any compromise in historic survival rates, which remained high >90%. RT of any type is extremely effective at stabilizing disease after hypothalamic-sparing surgery. The purported specific benefits of PBT-reducing sequelae are not proven in this study where the organ of critical interest is itself diseased, increasing an urgent need to better address and treat the tumor-induced endocrine harm from diagnosis in dedicated pituitary services. Other hypothesized benefits of PBT versus XRT on vascular events and secondary cancers await longer comparison. Clinical trial registration number: https://clinicaltrials.gov/study/, identifier NCT01272622

Cardiac remodeling in patients with childhood-onset craniopharyngioma—results of HIT-Endo and KRANIOPHARYNGEOM 2000/2007

Hypothalamic obesity caused by childhood-onset craniopharyngioma results in long-term cardiovascular morbidity. Knowledge about clinical markers and risk factors for cardiovascular morbidity is scarce. A cross-sectional study on transthoracic echocardiographic parameters was performed to determine the associations with clinical and anthropometric parameters in 36 craniopharyngioma patients. BMI correlated with the thickness of interventricular septum in diastole (IVSd) (r = 0.604, p < 0.001) and left ventricular posterior wall thickness in diastole (LVPWd) (r = 0.460, p = 0.011). In multivariate analyses on risk factors for cardiac remodeling, sex hormone replacement therapy, BMI, and male gender were positively correlated with increased left ventricular internal diameter in diastole (LVIDd),

Treatment Outcomes of Kasabach-Merritt Syndrome: A Single Hospital Experience

Objective: Although the outcomes of treatment of Kasabach-Merritt syndrome (KMS) have improved over time, some patients succumb to uncontrollable hemorrhage. Additionally, there is no consensus guideline for the management of KMS. Clinical research on KMS might benefit physicians who treat such patients. Methods: A retrospective chart review of patients diagnosed as KMS by Siriraj Hospital from 2006 to 2016 was conducted. Results: Ten patients were diagnosed with KMS. Four patients underwent surgical intervention and obtained pathological results; 3 of them had kaposiform hemangioendothelioma (KHE), while the fourth had infantile hemangioma (IH). The combination of propranolol and prednisolone, with or without vincristine, was the most common first-line regimen, with a complete response of 37.5%. A combination of vincristine, aspirin, and ticlopidine (VAT) was prescribed as the second-line therapy for 5 patients, but there were no responses in this cohort. Another 2 patients attained hematological remission with embolization and prednisolone monotherapy. A further 2 patients with KHE who were refractory to other treatments responded well to sirolimus, while the tenth patient died of abdominal hemorrhage. Conclusion: The combination of propranolol and prednisolone seems to be effective for KMS. Sirolimus may be considered for salvage therapy for those whose disease is recalcitrant to standard treatment, especially in cases of KMS secondary to KHE. However, research on a larger cohort should be conducted to substantiate the efficacy of such treatments