ONCO II - Test 3 (Sarcomas, Pediatric Tumors, Palliative)

What S&S presents in osteosarcoma?

Firm, painful mass adjacent to the bone

What are to common sites of spread for osteosarcoma

Distal femur, proximal tibia, proximal humerus

Most common S&S of STS?

Painless mass
Symptoms vary on characteristics of tumor

What is the most common histopathology for Osteosarcoma

Conventional, intramedullary
High grades include: osteoblastic, chondroblastic, fibroblastic

Most common histopathology for STS

Undifferentiated pleomorphic sarcoma (75%)
Liposarcoma (25%)

Most common mets for osteosarcoma

Lung → bone
skip mets via medullary canal

Most common mets for STS

Lung → bone
Liver → peritoneum (If tumor is abdo/retroperitoneum)

Staging for Osteosarcoma

TNM (size/extent, P/A, P/A)
Enneking (grade, intra/extracompartmental, P/A)

Staging for STS

TNM (Size, P/A, P/A)
SSSMTS (Grade, local extent, P/A) *does not account for size of primary*

Diagnostic tests for osetosarcoma

1. Hx+physical

2. Biopsy

3. Plain film + CT/MRI

Diagnostic tests for STS

1. Hx + Physical

2. Pre-biopsy with imaging

Staging test for Osteosarcoma

1. chest ct

2. biopsy

3. bloodwork (LDH + ALP)

Staging test for STS

Low grade = chest ct

Abdo/peritoneum tumors = abdo ct, creatinine clearance test

High grade = PET
Myxoid = MRI

Primary treatment for osteosarcoma

Sx

Conventional intramedullary low grade osteosarcoma tx

Sx = wide local excision

Conventional intramedullary high grade osteosarcoma tx

neo adj Cx → Sx → adj Cx

2 cycles → WLE → 4 cycles

agents = cisplatin + doxorubicin

Conventional intramedullary Metastatic tx for osteosarcoma

Cx = IfoEtop ± Carbo

Conventional intramedullary palliative tx for osteosarcoma

Advanced = 55Gy

Mets = 50-60Gy/3-5 SBRT or 8-30Gy/1-10

Primary tx for STS

Sx

Early stage neoadj RT for STS

50/25

Early adj RT for STS

60-66/30-33

Advanced non-met RT for STS

70/35

Retroperitoneal STS neoadj RT

50.4/28

Which genetic mutation is associated with LGG

BRAF Mutations, NF1

Which genetic mutation is associated with HGG

H3 K27M

Which genetic mutation is associated with Medulloblastoma`

Li-Fraumeni, Gorlin, Turcot

Which genetic mutation is associated with Neuroblastoma

NF1

Which genetic mutation is associated with Wilms

WT1 Mutations

Which genetic mutation is associated with Ewings

EWS-FLI1 fusion, CD99+ translocation

What are the most common S&S of CNS tumors

1. ICP

2. Neurological deficet

3. Siezures

4. Decreased hormones

S&S of infant neuroblastoma

healthy appearance with abdominal mass

S&S of older child neuroblastoma

Bony pain, abdo mass, malaise, fever

S&S of Wilms

Painless abdominal mass, hematuria, fever

S&S of Ewing

Pain/swelling, b-symptoms (mets present)

Is there a staging system for Paeds CNS tumors

No there is no uniform staging system

What is INSS Staging

Neuroblastoma
extent of tumor + LNs, P/A of mets
Stage 1-4S

What is INRG staging

Neuroblastoma (image based)
defines risk of disease based on what is present in image

What is NWTS staging

Based on the extent of the disease

kindey → LN → peritoneal → mets

TNM staging for Ewings (in bone)

Size+location, extent, p/a

TNM staging for Ewings (extraosseus)

Size, extent, p/a

LGG Treatment modalities

Primary = Sx

If progressive = CarbVin + adj 54/30

HGG Tx modalities

Primary = Sx + adj 59.4/33 or 54/30 (if DIPG)

Medulloblastoma Tx Modalities

Primary = Sx

Conventional = Sx → CSI → Cx

• Standard Risk = 23.4+ 54 boost → adj CisCyloVin

• High Risk = 36 + 54 boost ± CisCycloVin

Ependymoma Tx Modalities

Sx + adj 54 + 59.4 boost

Craniopharyngioma Tx modalities

Sx + Adj 54/30

Gemimomas Tx modalities

4 cycles CisEtop/VinCyclo (Alternating) + adj 23.4/13

Neuroblastoma Tx Modalities

Induction Cx → Sx → remaining Cx (total 6 cycles Cx) = CisDoxVinCycloEtop

Consolidation = Cx → 21.6/12 + 36 boost after 4-6 wks → cis-retinoic acid for 2 yrs

Wilms Tx modalities

Favorable conditions = Sx + adj ActinVin

Unfavorable = Sx + ActinVinDox + adj 10.6/6 (flank) or 12/8 (WLRT)

Ewing Tx modalities

Cx → Sx or RT → Cx

VDC/IE Regimen (VinDoxCycloIfosEtop) for 12 wks

• Phase 1 = Sx or adj 45

• Phase 2 = 55.8