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Pediatric: Rhabdomyosarcoma > Overview > Flashcards

Overview Flashcards

1
Q

What are the two incidence age peaks of RMS and their associated histologies?

A

2–6 yo (embryonal) and 15–19 yo (alveolar)

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2
Q

What is the estimated overall annual incidence of RMS in the United States?

A

350 cases/yr of RMS in the United States, 3% of all childhood cancers (#1 STS)

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3
Q

What are the most common sites of RMS? List them in order of approximate frequency in %.

A

Most common sites of RMS:

  1. H&N 40% (PM 25%, orbit 9%, non-PM 6%)
  2. GU 30%
  3. Extremity 15%
  4. Trunk 15%
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4
Q

What are the most common sites of mets?

A

Bone, BM, and lung

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5
Q

What % of pts present with mets? What types are prone to have hematogenous mets?

A

15% of pts present with mets. The prostate, trunk, and extremities are prone to hematogenous mets

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6
Q

What is the most common origin of RMS?

A

Mesenchymal stem cells. Sporadic RMS is the most common.

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7
Q

What genetic syndromes are associated with RMS?

A

Beckwith–Wiedemann syndrome (BWS), Li Fraumeni, NF-1, Costello syndrome, and Noonan syndrome

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8
Q

What are the 4 major histologies of RMS and their associated subtypes (if any)? Which is most common?

A

Major histologies of RMS and subtypes:

  1. Embryonal (classic, spindle cell, and botryoid) (Most common: 60%)
  2. Alveolar
  3. Pleomorphic
  4. Undifferentiated
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9
Q

What genetic change is associated with embryonal RMS?

A

LOH 11p15.5 (embryonal) is associated with IGF2 gene deletion, seen in BWS

Also, abnormalities in chromosomes 2, 8, 12, and 13 are associated with MYCN, MDM2, CDK4, CDKN2A (p16), CDKN2B(p15INK4b), and TP53 genes.

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10
Q

What translocations are associated with alveolar RMS? What are the genes involved in the fusion?

A

Alveolar RMS is associated with t(2;13) (70%) and t(1;13) (20%).

Genes involved are PAX3 or PAX7 with FOXO1 (aka FKHR).

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11
Q

Which is the most common histology of RMS in infants? Young children? Adolescents? Adults?

A

Most common RMS histology (by age group):

Infants: botryoid

Young children: embryonal

Adolescents: alveolar

Adults: pleomorphic

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12
Q

Which histologies are most commonly associated with each organ site (H&N, GU, extremities/trunk)?

A

Most common RMS histologies (by site):

H&N: embryonal

GU: botryoid

Extremities/Trunk: alveolar

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13
Q

What is the most important histologic tumor marker for RMS?

A

MyoD (and other myogenic proteins: actin, myosin, desmin, myoglobin)

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14
Q

What is the DDx for small round blue cell tumors of childhood?

A

Small round blue cell tumors of childhood:

Lymphoma

EWS

Acute lymphoblastic leukemia

RMS

NB

Neuroepithelioma

MB

Retinoblastoma

(Mnemonic: LEARN NMR)

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15
Q

List the histologies of RMS in terms of prognosis from best to worst.

A
  1. Spindle cell and botryoid
  2. Classic embryonal
  3. Alveolar
  4. Undifferentiated
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16
Q

What are the 5-yr OS rates for each of the histologic subtypes?

A

5-yr OS (by histology):

Botryoid: 95%

Spindle cell: 88%

Embryonal: 66%

Alveolar: 54%

Undifferentiated: 40%

17
Q

Which sites require LND b/c of a high propensity for LN mets? What is the risk of LN mets for these sites?

A

The following sites are associated with >20% LN mets rate and thus require LND:

PrT: (only if >10 yo)

Bladder: pelvic

H&N: NPX, LND typically not done for NPX

Extremities: UE (axillary) and LE (inguinal/femoral) (La TH et al., IJROBP 2011)

18
Q

Which sarcoma histologies are at high risk for LN mets?

A

Sarcomas with significant risk of LN mets are:

Synovial cell sarcoma

Clear cell sarcoma

Angiosarcoma

RMS

Epithelioid sarcoma

(Mnemonic: SCARE)

19
Q

Which International Rhabdomyosarcoma Study (IRS) called for routine LN sampling in RMS of the extremity?

A

IRS-IV (Neville HL et al., J Pediatr Surg 2000): 139 extremity pts, 76 pts had surgical LN evaluation; of the 10% who were clinically node positive (cN+), 50% were Pathologically node positive (pN+); of those cN0, 17% were pN+.

20
Q

What are considered nonregional mets/LNs for various sites (UE, LE, pelvic organs [PrT, vagina, uterus])?

A

Nonregional LN stations by primary site:

UE: scalene node

Pelvic (PrT/vagina/uterus): inguinal

Retroperitoneal (RP): P-A (except if immediately adjacent)

LE: iliacs/P-A

21
Q

What are the 4 favorable organ sites and their estimated 3-yr OS rate?

A

Favorable organ sites:

  1. Orbit
  2. Non-PM H&N
  3. Nonprostate/bladder GU
  4. Biliary

The estimated 3-yr OS is 94%.

22
Q

What is the estimated 3-yr OS for RMS arising from unfavorable sites (PM H&N, prostate, bladder, extremities/trunk)?

A

For unfavorable sites, estimated 3-yr OS is 70%.

23
Q

What are the PM H&N sites?

A

PM H&N sites:

Middle ear

Mastoid

Nasal cavity

NPX

Infratemporal fOssa

Pterygopalatine fOssa

PNS

Parapharyngeal space

(Mnemonic: MMNNOOPP)

24
Q

What are the non-PM H&N sites?

A

Scalp, cheek, parotid, oral cavity, oropharynx, and larynx

25
Q
A

Pediatric: Rhabdomyosarcoma (5 decks)

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