Glee-al Club
Pediatric low-grade glioma is now known to be a single pathway disease. This is the name of the pathway. (Full name for double points)
What is Mitogen-activated protein kinase (MAPK) pathway?
Commonly known as the most common primary CNS cancer, this entity is divided into four main molecular subgroup (name of the entity and all 4 subgroups)
What is Medulloblastoma? WNT, SHH, Group 3 and Group 4.
This disease group is the most common malignancy (benign and malignant tumors) in pediatrics (e.g, leukemia, CNS tumors etc). Source: CBTRUS
What is CNS tumors?
A 15 year old teenage boy presents with nystagmus and symptoms of diabetes insipidus. These laboratory tests would be diagnostic.
What is alpha fetoprotein and beta human chorionic gonadotrophin?
What is neurofibromatosis type 1?
DIPG is a devastating diagnosis that affects approximately 300 children each year. DIPG has a median overall survival of 11 months and is characterized by which mutation/alteration?
What is H3K27M mutation?
Trilateral retinoblastoma involves this non-eye structure in the brain. (50 points extra for the name of the cancer)
what is the pineal gland and pineoblastoma?
Constitutional MMRD high-grade glioma are hypermutant, which makes them particularly sensitive to this type of therapy.
What is immune checkpoint inhibitor therapy?
This group of tumors often present with hydrocephalus and are defined by the presence or loss of its papillary architecture.
what is choroid plexus tumors?
This is the most common CNS tumor in patients with NF1 syndrome.
What is optic pathway (nerve) glioma?
Pediatric high-grade glioma (histone WT) have poor outcomes with OS of <30%. Most are MGMT unmethylated. Aside from RT, what is considered the standard-of-care treatment upfront?
What is TMZ and CCNU?
Most older patients with embryonal/neuronal WHO Grade 4 tumors require surgery, craniospinal irradiation, and chemo. Proton CSI is superior to photon because it decreases the dose these organs. (name 3 for full points)
What is heart, thyroid, ovaries, thyroid, lungs, kidney, bowel?
This combination treatment for solid tumors with BRAFV600-mutations, is one of the first tumor agnostic approval by the FDA (following larotrectinib for NTRK tumors).
In contrast to pHGG, infant HGG have more favorable outcomes as they respond better to chemo and often have a targetable fusion. This is an example of a common fusion seen in infant HGG (name 2 for full points)
what is ALK, NTRK, ROS, MET?
A 15 year old presents with acute hearing loss. MRI shows bilateral vestibular schwannoma. This gene is associated with this cancer predisposition syndrome.
What is Merlin or schwannomin?
This is the most effective treatment for pediatric-type low-grade glioma - associated with a cure rate of nearly 90%? (Wisoff et al 2011)
What is a gross-total resection?
Discovered by Dr. Olivier Delattre as the cause, this entity is better known by its four letter acronym and is the most common malignant CNS tumor in infants (entity and gene for full points)
What is atypical teratoid rhabdoid tumor and SMARCB1?
These two regimens are the most commonly used for pediatric low-grade glioma and represents the divide between the Americans and the Canadians.
What is carboplatin / vincristine and vinblastine?
Craniopharyngioma are WHO grade 1 tumors that occurs in both children and adult. On imaging, these tumors are in the suprasellar region and present with calcification in Children. What are the two histological subtypes of craniopharyngioma?
what is adamantinomatous and papillary?
An 11 month old presents with symptoms of hydrocephalus and a large aggressively appearing enhancing ventricular mass. There is a family history of cancer in the family. What is the diagnosis and genetic predisposition syndrome?
What is choroid plexus carcinoma and Li-Fraumeni Syndrome
Tovorafenib is a pan-RAF inhibitor that is FDA approved for both BRAFV600 mutant and KIAA1549-BRAF fusion pLGG. One of the most common side effects is linear growth suppression. Inhibition of what gene is thought to be the cause for this temporary growth suppression?
What is CRAF?
Embryonal Tumor with Multilayered Rosettes (ETMR) is highly aggressive and primarily affects children under 3. Over 90% are driven by an amplification of the C19MC microRNA cluster on chromosome 19. This is the other driver of ETMR?
what is DICER1 mutation?
A young patient with recurrent medullloblastoma is treated with this inhibitor. After 6 months, disease is stable but his height is now in the 1st percentile. XR shows closure of growth plate. What medication is he on?
What is vismodegib or sonidegib? SHH inhibitor is acceptable.
Ependymoma is one the most common malignant CNS tumor in children (under the age of 5). Posterior fossa Eependymoma is divided into PFA and PFB, with PFA having significantly worse outcome. What chromosomal aberration is associated with highest risk for recurrence?
What is 1q gain and 6q loss?
Besides medulloblastoma, patients with Gorlin syndrome (PTCH/SUFU) are at risk for developing this tumor - often after radiation therapy.
What is meningioma?