Sarcomas Flashcards Preview

Solid tumors > Sarcomas > Flashcards

Flashcards in Sarcomas Deck (22):
1

Rhabdomyosarcomas: predisposition syndromes (7)

1. Li-Fraumeni
2. Gorlin
3. Beckwith-Wiedemann
4. Costello
5. Noonan
6. NF1
7. DICER-1

2

Immunochemistry findings in soft tissue sarcomas

RMS: Myogenin, muscle specific actin, MyoD, desmin
Synovial: cytokeratin, EMA, bcl2

3

Associated translocations:
a) RMS
b) Synovial sarcoma
c) Undifferenciated sarcoma
d) Infantile fibrosarcoma

a) PAX(3 or 7)/FOXO1
b) t(X;18) -- SYT-SSX fusion
c) t(4;19) -- CIC-DUX4 fusion occasionally
d) t(12;15)

4

Which soft tissue sarcomas are responsive to chemotherapy? (3)

Synovial sarcoma
Undifferenciated sarcoma
Infantile fibrosarcoma

5

Which chemotherapy agents are usually used in RMSTS?

Ifosphamide
Doxorubicin

6

Which soft tissue sarcomas are unresponsive to chemotherapy? (4)

MPNST
Leiomyosarcoma
Alveolar soft part sarcoma
Epithelial sarcoma

7

RMS: define risk stratification

LR:
- ERMS, Stage 1-2, Group I-II
- ERMS, Stage 1, Group III (orbit)
IR: all others
HR:
- EMRS, Stage 4, age > 10 years
- ARMS, Stage 4

8

RMS, prognosis (EFS at 5 years)

LR: 85%
IR: 50-75%
HR: 20%

9

Role or radiation in RMS (initial diagnosis)

Form of local control; for all patients except Group I (localized, fully resected) ERMS
Dose: 36-50.4Gy
Timing: starts between week 3-15 (unless urgently at diagnosis)

10

Name 8 prognostic factors in RMS

1. Age
2. Histological subtypes
3. Translocation status
4. Metastatic disease at diagnosis
5. Lymph node spread
6. Resectability
7. Site of tumor
8. Size of tumor

11

Inherited syndromes associated with OS (5)

Li-Fraumeni
Hereditary retinoblastoma
Rothmund-Thrompson
Bloom
Werner

12

Acquired RF for osteosarcomas (3)

Prior radiation therapy
Prior treatment with alkylating agents
Exposure to radium or beryllium (very rare)
Paget disease, in adults ­after 40 y.o.

13

Osteosarcoma, staging

- Plain X-R of affected limb
- MRI of affecting limb
- Bone scan
- Chest CT-scan

14

Osteosarcoma: list 5 pre-treatment prognostic factors

- Site of primary tumor
- Size of primary tumor
- Presence of metastase
- Site of metastases (lung only vs other, uni vs bilateral)
- Number of mets (better when less than 3)

15

Osteosarcoma: list 2 post-treatment prognostic factors

- Degree of tumor necrosis (better if ­>90%) -- both for primary site and mets
- Resectability

16

How is defined metastatic osteosarcoma?
Most sensitive modality to detect metastasis?

1) 3 pulmonary lesions of at least 5 mm or 1 pulmonary lesion of ast least 1 cm
2) Chest CT-scan

17

Prognosis in osteosarcoma

Localized: 75%
Metastatic: 40-50%

18

Bone lytic lesion: name 3 DDX

1) LCH
2) Aneurysmal bone cyst
3) Giant cell tumor

Osteosarcoma can have a lytic component, usually has some sclerotic component as well

19

Radiologic aspect of OS on X-R

- Epiphyseal lesion
- Mixed sclerotic and lytic pattern (although 1 component is predominant)
- Periosteonal new bone formation: sunburst pattern, Codman triangle
- Areas of calcification in bone and soft tissue component

20

3 most common sites of metastasis for Ewing sarcoma

Lung
Bone
Bone marrow

21

Describe management of relapsed Ewing

- MRI, PET-SCAN
- No standard of care for treatment
1. Cyclo/topotecan
2. Irinotecan/temozolomide

22

Most common site of recurrence in osteosarcoma?

Lungs