Clinico-Radiological Outcomes in WNT-Subgroup Medulloblastoma.
Shakthivel ManiAbhishek ChatterjeeArchya DasguptaNeelam ShirsatAkash PawarSridhar EpariAyushi SahayArpita Sahu AAliasgar V MoiyadiMaya PrasadGirish ChinnaswamyTejpal GuptaPublished in: Diagnostics (Basel, Switzerland) (2024)
Medulloblastoma (MB) comprises four broad molecular subgroups, namely wingless (WNT), sonic hedgehog (SHH), Group 3, and Group 4, respectively, with subgroup-specific developmental origins, unique genetic profiles, distinct clinico-demographic characteristics, and diverse clinical outcomes. This is a retrospective audit of clinical outcomes in molecularly confirmed WNT-MB patients treated with maximal safe resection followed by postoperative standard-of-care risk-stratified adjuvant radio(chemo)therapy at a tertiary-care comprehensive cancer centre. Of the 74 WNT-MB patients registered in a neuro-oncology unit between 2004 to 2020, 7 patients accrued on a prospective clinical trial of treatment deintensification were excluded, leaving 67 patients that constitute the present study cohort. The median age at presentation was 12 years, with a male preponderance (2:1). The survival analysis was restricted to 61 patients and excluded 6 patients (1 postoperative mortality plus 5 without adequate details of treatment or outcomes). At a median follow-up of 72 months, Kaplan-Meier estimates of 5-year progression-free survival and overall survival were 87.7% and 91.2%, respectively. Traditional high-risk features, large residual tumour (≥1.5 cm 2 ), and leptomeningeal metastases (M+) did not significantly impact upon survival in this molecularly characterized WNT-MB cohort treated with risk-stratified contemporary multimodality therapy. The lack of a prognostic impact of conventional high-risk features suggests the need for refined risk stratification and potential deintensification of therapy.
Keyphrases
- end stage renal disease
- newly diagnosed
- ejection fraction
- clinical trial
- chronic kidney disease
- stem cells
- prognostic factors
- cell proliferation
- peritoneal dialysis
- healthcare
- patient reported outcomes
- squamous cell carcinoma
- blood pressure
- palliative care
- risk assessment
- dna methylation
- adipose tissue
- study protocol
- skeletal muscle
- photodynamic therapy
- patient reported
- risk factors
- climate change
- single molecule
- cardiovascular events
- replacement therapy