Treatments

Question 1

Tx of Subependymal giant cellnastrocytoma
SEGAs

Answer 1

mTor inhibitors

Question 2

Low grade glioma

Answer 2

If completely resected: observe
If residual/can’t resect: chemo (carbo+VCR; vinblastine alone; thioguianine+procarbazine+lomustine+VCR =TPCV)
In trials: MEK inhib and BRAF inhib

Question 3

High grade glioma

Answer 3

Resection followed by focal radiotherapy and chemo

Infants <3 yo have somewhat improved outcomes due to unique biology. Overall 5yr OS 15-20%

Question 4

Ependymoma

Answer 4

Resection followed by focal radiation

Metastatic disease typically requires craniospinal radiation with boost to focal area

Could try chemo alone in infants

Question 5

Germ cell tumors

Answer 5

Chemo followed by response base radiation (carbo+etoposide x4 cycles)

2nd look surgery prior to XRT is encouraged to assess residual mass
-risk of growing teratoma syndrome

Question 6

Sodium thiosulfate undated for use in …

Answer 6

Cisplatin related ototoxicity in patients with localized solid tumor (not metastatic!)

Question 7

Epithelioid sarcoma scenario
1cm tumor against the L 5th proximal phalanx
Loss of INI1, SMARCB1 mutation

Answer 7

Ray amputation along with sentinel lymph node bx, then observation

-rare non-rhabdo soft tissue sarcomas, poorly chemo responsive
Tend to spread to lymph nodes
PETs are recommended (node bx required regardless)

Question 8

Order of correction for iron def labs:

Answer 8

Reticulocyte hemoglobin content, hemoglobin, MCV, RDW, serum ferritin

Question 9

Tx of paroxysmal cold hemoglobinuria (PCH)

Answer 9

Transient and self limited
Complement mediated intravascular hemolysis

PCH can be due to presence or Donath-Landsteiner antibody (cold reacting IgG of high thermal amplitude”

IgM is easy to remove by pheresis, not IgG

Question 10

Tx of non-metastatic synovial sarcoma in the leg muscle
< 5cm

Answer 10

Complete resection and observation

Spindle cells arranged in fascicles
SS18 (SYT) gene rearrangement by FISH

If >5cm or pos margins or metastatic, Tx with chemo (ifos/doxo). Radiation limited to primary site post resection

Question 11

Long term side effects of TKI (imatinib)

Answer 11

Endocrinopathies
-short stature
-thyroid abnormalities
-change in bone metabolism

Question 12

Anti phospholipid antibody syndrome

Answer 12

Dx requires repeat testing 12 wks later

+dilute Russell viper venom time
+silca clot test
+high titer anti-cardiolipin antibodies
+high titer beta2 glycoprotein antibodies

Tx warfarin or LMWH, indefinitely