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RHABDOMYOSARCOMA Hematuria

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RHABDOMYOSARCOMA Hematuria Powered By Docstoc
					RHABDOMYOSARCOMA

    Dr. Michelle Ghert
    Osama Alshaya(PGY2)
        2008/08/06
Outline
Introduction
Epidemiology
Associated syndromes
Clinical presentation
Pathological classification
Diagnosis and DDx
Clinical staging and prognosis
treatment
Soft tissue sarcoma in pediatric divided to:
- Rhabdomyosarcoma(40-50%)
- Non-rhabdomyosarcoma
  (synovial sarcoma, fibrosarcoma, PNET)
Soft tissue sarcoma common in adult
Rhabdomyosarcoma: malignant mesenchymal
tumor of striated skeletal muscle
Epidemiology

3-4% of all child cancer
4cases per million in Canada
Commonest STT in patient <20 Y.O
M:F(1,5:1)
Two third of cases diagnosed in first 5years
Specific risk factor------unknown and most
cases are sporadic
20% have lung metastases at time of
presentation
Inherited syndromes

Li-Fraumeni syndrome:
  -germ line mutation cause inactivation of
tumor suppressor gene (P53)
  -many STT(tx?)

Beckwith-Wiedemann syndrome:
associate with overgrowth tumors
NF-1: 30 folds risk to develop RMS


Costello syndrome:
-other name FCS syndrome
-has growth and mental retardation
Clinical presentation

Depend on( site, age, present of metastases )
Rapid growing painless mass
Head and neck:
 -commonest site orbit>parameningeal sites
>other sites
 - proptosis, S&S of obstruction
Genitourinary tract:
-2nd common site
-hematuria and S&S of obstruction

Extremities:
-mainly proximal lower limbs and in adult
-L.N involved in 50% and have alveolar
subtype
Pathological classification

1)Embryonic : -common in age less than 10
  Y.O(2/3)
  - Site: head and neck>genitourinary>lower
  extremities
  - Molecular genetic: loss of heterozygosity
   at 11p5(IGF-11)
  - Subtypes: a)Spindle cell type
                -mainly paratesticular
                -good prognosis
b)Botryoid type
  -grape like nodular tumor
  -less common(10%)
  -mainly submucosal in vagina and
 bladder
  -good prognosis
c)Not-otherwise specific:
  -intermediate prognosis
Illustrated picture
show:
  Grape like structure
from genital tract of
child
2)Alveolar type:
   -adolescent type(10-25Y.O)
   -M=F
   -site: trunk, extremities and paraspinal
   -subtypes:
    a)classic: has typical alveolar pattern
    b)solid
    c)mixed
   -molecular genetic: t(2,13)(q35,q14)
    present in 60%
   -prognosis poor
3)Pleomorphic = undifferentiated
  -adult type(50-70Y.O)
  -site: proximal L.L
  -prognosis poor
 (25% 5 years survival)
Diagnosis

Extend blood works
Bone marrow aspiration in children
L.N biopsy if RMS involve extremities
CSF sampling in head and neck RMS
Radiographic evaluation
   1)X-ray and C.T scan:
    -nothing suggestive about RMS (soft
tissue)
    -bone involvement rare
30 Y.O , male
presented
with RT arm
painless
mass
PLEOMORPHIC
RMS INVOLVE BICEPS
MUSCLE:


 Only you can see soft tissue
        unclear mass
 MRI
T1 MRI show
mild
hyperintense
with isointense
soft tissue
tumor involving
left gluteus
medius muscle
51-year-old man with pleomorphic
rhabdomyosarcoma of right thigh
Local and systemic
staging

 -Important to have biopsy by either needle or
incision biopsy
 -Apply different IHC
(actin, desmin and myoglopin)
 -Determine the pretreatment stage and
surgicopathological clinical group to know the
prognosis and the treatment regimens
 Term                                Definition
Favorable   Orbit; non-parameningeal head and neck; genitourinary excluding
   site                 kidney, bladder, and prostate; biliary tract.


Unfavora                  Any site not considered favorable.
 ble site

   T1                     Confined to anatomic site of origin.


   T2               Extension and/or fixative to surrounding tissue.


    a                    Tumor ≤5 cm in maximum diameter.


   b                     Tumor >5 cm in maximum diameter.


   N0                No clinical regional lymph node involvement.


   N1                  Clinical regional lymph node involvement.


   M0                           No metastatic disease.
   M1                             Metastatic disease.
        Sites of Primary      Tumor        Regional Lymph      Distant
Stage
            Tumor              Size            Nodes          Metastasis




  1      Favorable sites      Any size     N0, or N1, or NX
                                                                 M0

  2     Unfavorable sites   T1a or T2a        N0 or NX
                                                                 M0

  3     Unfavorable sites   T1a, T2a, or         N1
                             T1b, T2b      N0 or N1 or NX        M0


  4         Any site          Any size        N0 or N1
                                                                 M1
           Group                                        Definition



 I [Note: Approximately 13%]     A localized tumor completely removed with pathologically
                                  clear margins and no regional lymph node involvement.



II [Note: Approximately 20% ]      A localized tumor that is grossly removed with: (A)
                                  microscopic disease at the margin, (B) involved, grossly
                                  removed regional lymph nodes, or (C) both A and B.


III [Note: Approximately 48% ]      A localized tumor with gross residual disease after
                                            incomplete removal or biopsy only.



IV [Note: Approximately 18% ]           Distant metastases are present at diagnosis.
  Risk Group            Histology           Stage     Group




   Low Risk              Embryonal            1       I, II, III

                         Embryonal           2, 3       I, II




Intermediate Risk        Embryonal           2, 3        III

                          Alveolar          1, 2, 3   I, II, III




   High Risk        Embryonal or Alveolar     4          IV
Spindle cell subtype
 has small round blue cells and separated by myxoid like matrix, cross striation
seen under special stain .
Poor Differentiated Embryonal
RMS
difficult to distinguish from other small round blue cell tumors(Ewing, PNET)
Botryoid cell subtype
dense rhabdomyoplast subepithelial layer
Alveolar subtype(classic type)
small round cell floating in pseudo-alveolar space represent fibrovascular
septae
Pleomorphic RMS
spindle shape cells (wrist watches)
Treatment

  -need multidisciplinary team
  -aim for local and systemic control
 1)Surgical treatment: wide local resection

 2)Chemotherapy:
   *always respond to neoadjuvant and
adjuvant chemotherapy
   *2-3 years duration of treatment
   *common regimen(VAC)
   *S/E:myelosuppresion(90%)
3)Radiotherapy:
 *if +ve margin and help decrease risk of
recurrent
 *S/E: either acute or chronic and post
radiation sarcoma one of the complication

				
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