ATRT

Question 1

What age groups gets ATRT

Answer 1

95% occur in kids 5 or younger

Question 2

Where do they occur anatomically

Answer 2

More likely to invovle the lateral hemisphere of the cerebellum
>50% invovle the posterior fossa
little under half are supratentorial

Question 3

Where do most failures occur

Answer 3

locally

Question 4

What were ATRTs misdiagnosed as until recently

Answer 4

PNET

Question 5

Describe workup for ATRT

Answer 5

Assess for INI-1 mutations- Loss of this in >85%
Assess for vimentin, EPA, SMA
CT abdomen/pelvis to assess for rhabdoid tumor of the kidney (not a met)

Question 5

What is the treatment paradigm for ATRT

Answer 5

max safe surgical resection–>chemox2–> HD chemoX3
–>PBST–> RT (IFRT)

Question 5

WHat are the RT doses for ATRT

Answer 5

IFRT
posterior fossa RT
If < 3 yrs, IFRT to 50.4
If > 3, IFRT to 55
Try to avoid RT if under 6 months

Question 5

What chemo is used

Answer 5

Induction: vincristine, methotrexate, etoposide, cyclophosphamide, cisplatin
Consolidation: thiotepa, carboplatin with stem cell support

Question 6

What RT doses for M+

Answer 6

Treat M+ like high risk medulloblastoma
CSI- 36 Gy
IFRT boost to 54 Gy
Boost cord disease to 45

Boost spinal disease above cord to 45 Gy, below cord to 50.4-54
If there is diffuse cord disease, treat to 39.6Gy
If <3 years, CSI to 23.4 Gy

Question 6

What is the survival for ATRT

Answer 6

Median survival is 1 year