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Rhabdomyosarcoma > Background and recommendations > Flashcards

Background and recommendations Flashcards

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?

A

41.4 Gy to .1 cc

22
Q

What are dose constraints for the whole brain?

A

30.6 Gy to .1 cc

23
Q

When do you deliver RT?

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?

A
  1. Vincristine
  2. Actinomycin
  3. Cytoxan It is given every 4 weeks
25
Q

Is concurrent chemotherapy given during radiation?

A

Yes, Vincrisitne and Cytoxan q 4 weeks

26
Q

What do you do if a patients has a residual mass after treatment?

A

Nothing but close monitoring. 50% actually still have tumor cells, but surgical resection does not improve OS.

27
Q

What are the 4 major histologies?

A
  1. Embryonal (classic, spindle, botryoid)
  2. Alveolar
  3. Pleomorphic
  4. Undifferentiated
28
Q

From best prognosis to worst, how do the histologies rank?

A
29
Q

What are the 5 year OS rates associated with each histology?

A
30
Q

For what sites are LNDs required?

A
  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
Q

What are the non-parameningeal head and neck site?

A
  1. Scalp
  2. Cheek
  3. Parotid
  4. Oral cavity
  5. Oropharynx
  6. Larynx
32
Q

How is a tissue diagnosis acquired?

A
33
Q

When is a MRI of the neuroaxis needed?

A

If the LP is positive

34
Q

What are the favorable organ sites? 3 year OS?

A
  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
Q

What studies are needed for a tissue diagnosis?

A
  1. Core biopsy or incisional biopsy of the primary
  2. Bone marrow biopsy
  3. LP for parameningeal sites
36
Q

What work up studies are needed for bladder tumors?

A

EUA and cystoscopy

37
Q

What are the most common sites of metastasis?

A

Bone, BM and lung

38
Q

What histologies are common at each age group?

A
  1. Infants: botryoid
  2. Young children: embryonal
  3. Adolescents: alveolar
  4. Pleomorphic
39
Q

What is the most common group of disease?

A

Group III: 50%

40
Q

What is the general treatment paradigm for RMS?

A
  1. Biopsy only or surgical resection
  2. Chemo
  3. +/- Radiation (timing depends on risk grouping)
41
Q

What is considered high risk disease?

A

All metastatic patients

42
Q

T staging?

A

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
Q

N Stage?

A

N1: regional nodal involvement

44
Q

When is biopsy only appropriate?

A

Parameningeal

Orbit

45
Q

When is amputation of an extremity RMS not appropriate?

A

Stage IV disease

46
Q

When is second look surgery recommended if initially unresectable disease becomes resectable?

A

At week 13

47
Q

When is a parameningeal tumor considered high risk?

A
  1. CN palsy
  2. Intracranial extension
  3. Subarachnoid space involvement with skull base erosion
48
Q

Which patients are low risk?

A
  1. Favorable sites, all groups
  2. Unfavorable sites, groups I and II
49
Q

What are the 5 year OS rates for different histologies?

A

Botryoid: 95%

Spindle cell: 88%

Embryonal: 66%

Alveolar: 54%

Undifferentiated: 40%

50
Q

What is the timing of RT?

A
  1. Low risk: week 13
  2. Intermediate risk: week 4
  3. High risk: week 20
51
Q

Dose constraint for kidney?

A

Whole kidney 19.8 Gy

Rhabdomyosarcoma (1 decks)

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