Rhabdomyosarcoma

Question 1

Small blue round cell tumors?

Answer 1

Rhabdo
Neuroblastoma
lymphoma
PNET

Question 2

Genetic syndromes associated with Rhabdo?

Answer 2

Li Fraumeni

NF-1

Question 3

RMS staging based on pre-op work up?

Answer 3

Stage 1: any orbit, H&N(excludes parameningeal), GU (excludes bladder/prostate)tumor with no mets

Stage 2: Any other location less than 5 cm

Stage 3: Any other location with tumor greater than 5 cm or node positive

Stage 4: Any mets including distant nodes.

Question 4

Clinical grouping based on Post-op status?

Answer 4

1) complete resection with negative margins
2) Gross resection with positive micro margins, no nodes
3) gross residual disease
4) Stage 4

Question 5

Staging work up for RMS?

Answer 5

Labs: CBC, lytes, Cr, LFTs, urine

Imaging: CT or MRI for primary site + CT chest,liver and head, bone scan.

Bone marrow and LP

Question 6

Surgery for biliary tract RMS?

Answer 6

NO….biopsy only!

Excellent outcomes with chemo rads

Question 7

Surgical management of pelvic and retroperitoneal disease?

Answer 7

Aggressive resection to achieve RO with 5 mm margins if possible.

Debulking only has some benefit for patients <12 yo and stage 4.

Question 8

Gyne lesions surgical management?

Answer 8

Typically very chemosensitive so avoid aggressive resection unless persistent disease post neo-adj

Question 9

Standard Chemo for RMS?

Answer 9

VAC:

Vincristine
Actinomycin-D
Cyclophosphamide

Question 10

Principles of resection for RMS?

Answer 10

• circumferential margin of 5mm
• accept positive margins to avoid loss of function, organ compromise, poor cosmesis
• mark margins with titanium clips to guide radiation or re-excision
• intra-op frozen sections for margins

Question 11

indication for pulmonary metasectomy?

Answer 11

Only after chemo rads if no additional disease i.e. for cure

Question 12

What is pre-treatment re-excision and when is it indicated?

Answer 12

If non-oncologic operation was performed for diagnosis or margins were unclear in otherwise resectable mass.

Take previous surgical site with margin.

Question 13

When should node dissection be done for RMS?

Answer 13

Never.
Nodal status is for prognostication only.

Node sampling should always be done for extremity tumors or paratesticular tumors (age>10) because of high incidence of positive nodes.

Question 14

Indications for neo-adj in chest wall RMS?

Answer 14

• > 5 cm
• involve 3 ribs (require resection of 5 or more)
• abutting scapula, sternum
• intraspinal extension
• significant neurovascular involvement.

Question 15

When to assess respectability of RMS after neo-adj?

Answer 15

• Assess post 2-4 cycles

- if gross total resection is not possible rads can be given alone but preference is rad after GTR.

Question 16

Is radiation indicated in all cases of chest wall RMS post-resection?

Answer 16

No, RO resection may avoid radiation.

Question 17

Outcomes for chest wall RMS based on post op status?

Answer 17

5 year survival:

RO - 65%
R1- 65%
Biopsy only 59%
Mets 7%