Sarcomas

Question 1

Rhabdomyosarcomas: predisposition syndromes (7)

Answer 1

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

Question 2

Immunochemistry findings in soft tissue sarcomas

Answer 2

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

Question 3

Associated translocations:

a) RMS
b) Synovial sarcoma
c) Undifferenciated sarcoma
d) Infantile fibrosarcoma

Answer 3

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)

Question 4

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

Answer 4

Synovial sarcoma
Undifferenciated sarcoma
Infantile fibrosarcoma

Question 5

Which chemotherapy agents are usually used in RMSTS?

Answer 5

Ifosphamide

Doxorubicin

Question 6

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

Answer 6

MPNST
Leiomyosarcoma
Alveolar soft part sarcoma
Epithelial sarcoma

Question 7

RMS: define risk stratification

Answer 7

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

Question 8

RMS, prognosis (EFS at 5 years)

Answer 8

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

Question 9

Role or radiation in RMS (initial diagnosis)

Answer 9

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)

Question 10

Name 8 prognostic factors in RMS

Answer 10

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

Question 11

Inherited syndromes associated with OS (5)

Answer 11

Li-Fraumeni
Hereditary retinoblastoma
Rothmund-Thrompson
Bloom
Werner

Question 12

Acquired RF for osteosarcomas (3)

Answer 12

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

Question 13

Osteosarcoma, staging

Answer 13

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

Question 14

Osteosarcoma: list 5 pre-treatment prognostic factors

Answer 14

• 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)

Question 15

Osteosarcoma: list 2 post-treatment prognostic factors

Answer 15

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

Question 16

How is defined metastatic osteosarcoma?

Most sensitive modality to detect metastasis?

Answer 16

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

Question 17

Prognosis in osteosarcoma

Answer 17

Localized: 75%
Metastatic: 40-50%

Question 18

Bone lytic lesion: name 3 DDX

Answer 18

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

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

Question 19

Radiologic aspect of OS on X-R

Answer 19

• 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

Question 20

3 most common sites of metastasis for Ewing sarcoma

Answer 20

Lung
Bone
Bone marrow

Question 21

Describe management of relapsed Ewing

Answer 21

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

Question 22

Most common site of recurrence in osteosarcoma?

Answer 22

Lungs