Peds

Question 1

Morphological subtypes of Medulloblastoma (best to worst prognosis)

Answer 1

1) Desmoplastic, extensive nodularity
2) Classic
3) Large cell, anaplastic

Question 2

MOLECULAR SUBTYPES best to wost prognosis

Answer 2

1) WNT
2) SHH
3) Group 4
4) Group 3 (usually high MYC amplification)

Question 3

Medulloblastoma Risk groups: standard vs high

Answer 3

1) Standard risk:
- ≥3yrs, <1.5cm2 residual on post-op MRI, M0 by craniospinal MRI and CSF, not anaplastic
2) everything else (Note;: <3 is high risk because there is no planned role for RT). Can also consider unfavourable pathology (anaplastic or large cell, or molecular features such as MYC amplification)

Question 4

Treatment MB based on risk group

Answer 4

1) standard risk:
CSI 23.4/13 + 54/30 boost to surgical cavity with concurrent weekly vincristine, then add cisplatin and cyclo x 4

2) high risk:
36Gy/20#, boost cavity to 54Gy with concurrent VCR followed by adjuvant cyclo, cisplatin, VCR x 6

Question 5

Germ cell tumor classifications and markers

Answer 5

Germinomas
- Elevated -hCG (<50-100 mlU/ml), but can be normal  APF not elevated

Non-germinomatous GCT
Choriocarcinoma: hCG elevated
Yolk sac: elevated AFP
Embryonal: AFP + hCG elevated Teratoma

Question 6

Medulloblastoma what are 6 late toxicities to discuss

Answer 6

Hearing loss
Neurocognitive impact
Endocrine dysfunction
Loss of height
Bone marrow loss
Secondary malignancies
Fertility
Renal dysfunction
Small bowel obstruction

Question 7

Wilm’s long term tox to counsel on;
2) 4 things to check in survivorship clinic

Answer 7

Scoliosis, kyphosis
Delayed growth
Soft-tissue hypoplasia
Small bowl obstruction
Iliac wing hypoplasia
Liver/kidney hypoplasia
Hypertension
Renal failure
Pneumonitis
CHF (doxorubicin)
Infertility
Second malig

2) Cardiac function
Renal function
Liver function
Hypertension