Difference between revisions of "Rhabdomyosarcoma"

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Notes:
Notes:
*Translocation-negative alveolar RMS shares characteristics with ''embryonal RMS''.
*Translocation-negative alveolar RMS shares gene expression prolifing characteristics with embryonal RMS -- suggesting these can be grouped together.


==Microscopic==
==Microscopic==
Alveolar rhabdomyosarcoma:
===Alveolar rhabdomyosarcoma===
*Alveolus-like pattern:
Features:<ref name=PST14feb11>PST. 14 February 2011.</ref>
*Alveolus-like pattern -- '''key low-power feature'''.
**Fibrous septae lined by tumour cells.
**Fibrous septae lined by tumour cells.
***Space between fibrous sepate may be filled with tumour: ''solid variant of alveolar rhabdomyosarcoma''.
***Cells may "fall-off" the septa, i.e. be detached/scattered in the alveolus-like space.
*Eccentric nucleus (???).
***Space between fibrous sepate may be filled with tumour = ''solid variant of alveolar rhabdomyosarcoma''.
*Cytoplasm - dense pink staining on H&E (if well differentiated).
*Rhabdomyoblasts - '''essentially diagnostic'''.
*Usu. nuclear pleomorphism +++.
**Eccentric nucleus.
*Mitoses common.
**Moderate amount of intensly eosinophilic cytoplasm.
**Striations -- if you're really lucky; these are not common.
 
Other features:
*Nuclear pleomorphism - common.
*Mitoses - common.
 
Notes:
*Well-differentiated rhabdomyoblasts are uncommon in alveolar RMS.
 
===Embryonal rhabdomyosarcoma===
Features:<ref name=PST14feb11>PST. 14 February 2011.</ref>
*Randomly arranged small cells.
*Myxoid matrix.
*Strap cells:
**Tadpole-like morphology.
*Rhabdomyoblasts - '''essentially diagnostic'''.
**Eccentric nucleus.
**Moderate amount of intensly eosinophilic cytoplasm.
**Striations -- if you're really lucky; these are not common.
 
====Subtypes of embryonal RMS====
There are two common subtypes of embryonal RMS.  Both of them have a better prognosis that embryonal RMS not otherwise specified (NOS).
 
Common subtypes:
#Botryoid subtype:
#*Gross: Grape-like morphology.
#*Microscopic: Non-proliferating layer deep to the surface ("Cambrian layer").
#Spindle cell subtype.
#*General: may mimic [[leiomyosarcoma]] -- which is not common in the pediatric population.
#*Microscopic: vesicular growth pattern, spindle cells.
 
==IHC==
Panel of muscle markers -- ''DAM'':
*Desmin (best marker).
*Actin.
*Myogenin.
 
==[[Electronmicroscopy]]==
Features:
*Sarcomeric like structures - usu. in "bent" cells; cells that are U-shaped.


==Molecular diagnostics==
==Molecular diagnostics==
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Notes:
Notes:
*t(1,13) vs. t(2,13) -- t(1,13) usually: younger age, extremity lesion, localized disease, better survival.
*Several uncommon [[translocations]] exist.
*Several uncommon [[translocations]] exist.
==IHC==
*Desmin (best marker).
*Actin.


==See also==
==See also==
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