Rhabdomyosarcoma > Background and recommendations > Flashcards

Background and recommendations Flashcards

(51 cards)

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1
Q

Labs need for work up?

A
  1. CBC
  2. LFT
  3. BMP
  4. LDH
  5. Pregnancy test for girls at risk
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2
Q

What imaging is needed for work up?

A
  1. Diagnostic CT scan of primary
  2. MR of primary
  3. CT scan of chest/abdomen
  4. Bone scan
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3
Q

When is an LP needed?

A

When the primary tumor is parameningeal or paraspinal

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4
Q

What are the parameningeal head and neck sites?

A
  1. Middle ear
  2. Nasopharynx
  3. Paranasal sinuses
  4. Parapharyngeal space
  5. Mastoid
  6. Nasal cavity
  7. Infratemporal fossa
  8. Pterygopalantine fossa
  9. Parapharyngeal space
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5
Q

Stage I

A

Any favorable site.

Any T and Any N but M0

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6
Q

Stage II

A

Unfavorable site

T1a-T2a (<5 cm)

N0

M0

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7
Q

What are the unfavorable sites? 3 year OS?

A
  1. Bladder/prostate
  2. Extremity
  3. Parameningeal
  4. Trunk (excludes biliary)
  5. Retroperitoneum
  6. Cranial

3 year OS for patients with unfavorable sites is 70%

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8
Q

Stage III

A

Unfavorable site

T1b to T2b (> 5cm)

or

N1

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9
Q

Stage IV

A

Metastatic disease is present

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10
Q

Group I (POST-OP)

A

Localized disease, Completely resected

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11
Q

Group II (POST-OP)

A
  1. R1 and/or node positive
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12
Q

Group III (POST-OP)

A
  1. Bx only or gross residual after resection
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13
Q

Group IV (POST-OP)

A

Stage IV with metastases

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14
Q

When do you treat patients with group I? What dose?

A
  1. Alveolar only and not amputated 2. 36 Gy at 1.8 Gy per fraction
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15
Q

When do you treat group II? What dose do you use?

A
  1. Always 2. LN negative: 36 Gy at 1.8 Gy per fraction 3. LN positive: 41.4 Gy at 1.8 Gy per fraction
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16
Q

When do you treat group 3? What dose do you use?

A
  1. Always 2. Orbital: 45 Gy at 1.8 Gy per fraction 3. All others: 50.4 Gy at 1.8 Gy per fraction
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17
Q

How do you develop the GTV? Cone down GTV?

A
  1. Fuse the pretreatment MR and CT of the primary 2. GTV1= pretreatment visible disease Use GTV2 when induction chemotherapy worked. Start after 36 Gy. 1. Fuse postchemo MR and CT 2, GTV2= post-chemo visible disease
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18
Q

How do you develop CTV1 and CTV2 and PTV?

A
  1. CTV 1= GTV1 + node positive regions+ 1 cm EFAB 2. CTV 2= GTV2 + node positive regions +0.5 cm but not smaller than prechemo GTV and EFAB 2. PTV = CTV + 3 mm
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19
Q

What are dose constraints for the optic nerve?

A

46.8 Gy to .1 cc

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20
Q

What are dose constraints for the lens?

A

14.4 Gy to .1 cc

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21
Q

What are dose constraints for the lacrimal gland?

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A

41.4 Gy to .1 cc

22
Q

What are dose constraints for the whole brain?

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A

30.6 Gy to .1 cc

23
Q

When do you deliver RT?

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A
  1. Intracranial extension: Week 2
  2. Alveolar: Week 4
  3. Parameningeal site: Week 4
  4. Everyone else: Week 13
24
Q

What is the chemotherapy? How ofen is it given?

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A
  1. Vincristine
  2. Actinomycin
  3. Cytoxan It is given every 4 weeks
25
Is concurrent chemotherapy given during radiation?
Yes, Vincrisitne and Cytoxan q 4 weeks
26
What do you do if a patients has a residual mass after treatment?
Nothing but close monitoring. 50% actually still have tumor cells, but surgical resection does not improve OS.
27
What are the 4 major histologies?
1. Embryonal (classic, spindle, botryoid) 2. Alveolar 3. Pleomorphic 4. Undifferentiated
28
From best prognosis to worst, how do the histologies rank?
29
What are the 5 year OS rates associated with each histology?
30
For what sites are LNDs required?
1. Paratesticular (if older than 10 years) 2. Bladder requires pelvic LND 3. Lower extremity requires inguinal LND 4. Upper extremity requires axillary LND
31
What are the non-parameningeal head and neck site?
1. Scalp 2. Cheek 3. Parotid 4. Oral cavity 5. Oropharynx 6. Larynx
32
How is a tissue diagnosis acquired?
33
When is a MRI of the neuroaxis needed?
If the LP is positive
34
What are the favorable organ sites? 3 year OS?
1. Orbit 2. Nonparameningeal head and neck 3. Non-prostate/bladder GU 4. Biliary 3 year OS for patients with these sites is 94%
35
What studies are needed for a tissue diagnosis?
1. Core biopsy or incisional biopsy of the primary 2. Bone marrow biopsy 3. LP for parameningeal sites
36
What work up studies are needed for bladder tumors?
EUA and cystoscopy
37
What are the most common sites of metastasis?
Bone, BM and lung
38
What histologies are common at each age group?
1. Infants: botryoid 2. Young children: embryonal 3. Adolescents: alveolar 4. Pleomorphic
39
What is the most common group of disease?
Group III: 50%
40
What is the general treatment paradigm for RMS?
1. Biopsy only or surgical resection 2. Chemo 3. +/- Radiation (timing depends on risk grouping)
41
What is considered high risk disease?
All metastatic patients
42
T staging?
T1a: tumor limited to site of origin and 5 cm or less T1b: tumor limited to site of origin \> 5 cm in size T2a: tumor extends to adjacent tissue and 5 cm or less T2b: tumor extends to adjacent tissue and \> 5 cm
43
N Stage?
N1: regional nodal involvement
44
When is biopsy only appropriate?
Parameningeal Orbit
45
When is amputation of an extremity RMS not appropriate?
Stage IV disease
46
When is second look surgery recommended if initially unresectable disease becomes resectable?
At week 13
47
When is a parameningeal tumor considered high risk?
1. CN palsy 2. Intracranial extension 3. Subarachnoid space involvement with skull base erosion
48
Which patients are low risk?
1. Favorable sites, all groups 2. Unfavorable sites, groups I and II
49
What are the 5 year OS rates for different histologies?
Botryoid: 95% Spindle cell: 88% Embryonal: 66% Alveolar: 54% Undifferentiated: 40%
50
What is the timing of RT?
1. Low risk: week 13 2. Intermediate risk: week 4 3. High risk: week 20
51
Dose constraint for kidney?
Whole kidney 19.8 Gy
Rhabdomyosarcoma
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