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Difference between revisions of "Rhabdomyosarcoma"
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Alveolar_rhabdomyosarcoma_-_very_high_mag.jpg | |||
| Width = | |||
| Caption = Alveolar rhabdomyosarcoma. [[H&E stain]]. | |||
| Synonyms = | |||
| Micro = +/-rhabdomyoblasts (eccentric nucleus, moderate amount of intensly eosinophilic cytoplasm, striations - not common); alveolar RMS: alveolus-like pattern (classic); embryonal RMS: [[small round cell tumour]] | |||
| Subtypes = embryonal (spindle cell subtype, botryoid), alveolar (translocation-positive, translocation-negative), undifferentiated | |||
| LMDDx = [[small round cell tumours]] - esp. [[small cell carcinoma]] and (large cell) [[lymphoma]]s | |||
| Stains = | |||
| IHC = desmin (best marker) +ve, actin +ve, myogenin +ve, CD56 +ve (common), synaptophysin -ve/+ve, chromogranin -ve/+ve, cytokeratins -ve/+ve | |||
| EM = sarcomeric like structures - typically in U-shaped cells | |||
| Molecular = alveolar RMS (~85% of cases): t(2,13) PAX3/FKHR fusion gene ''or'' t(1,13) PAX7/FKHR fusion gene | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[soft tissue]] - skeletal muscle (alveolar RMS), non-muscle site (embryonal RMS) | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = | |||
| Other = | |||
| ClinDDx = other soft tissue tumours | |||
| Tx = | |||
}} | |||
'''Rhabdomyosarcoma''', often abbreviated '''RMS''', is a [[malignant]] tumour of skeletal muscle. | '''Rhabdomyosarcoma''', often abbreviated '''RMS''', is a [[malignant]] tumour of skeletal muscle. | ||
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#*Usually young adults/adolescents. | #*Usually young adults/adolescents. | ||
#*Early mets common. | #*Early mets common. | ||
#* | #*Usually arises in regions with skeletal muscle. | ||
#Embryonal rhabdomyosarcoma. | #Embryonal rhabdomyosarcoma. | ||
#*Usual <10 years old. | #*Usual <10 years old. | ||
#*Typically locally invasive. | #*Typically locally invasive. | ||
#* | #*Usually arises in regions '''without''' skeletal muscle. | ||
Less common types:<ref name=pmid12110339>{{Cite journal | last1 = Hicks | first1 = J. | last2 = Flaitz | first2 = C. | title = Rhabdomyosarcoma of the head and neck in children. | journal = Oral Oncol | volume = 38 | issue = 5 | pages = 450-9 | month = Jul | year = 2002 | doi = | PMID = 12110339 }}</ref> | Less common types:<ref name=pmid12110339>{{Cite journal | last1 = Hicks | first1 = J. | last2 = Flaitz | first2 = C. | title = Rhabdomyosarcoma of the head and neck in children. | journal = Oral Oncol | volume = 38 | issue = 5 | pages = 450-9 | month = Jul | year = 2002 | doi = | PMID = 12110339 }}</ref> | ||
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Other features: | Other features: | ||
*Nuclear pleomorphism - common. | *[[Nuclear pleomorphism]] - common. | ||
*Mitoses - common. | *Mitoses - common. | ||
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==[[Electron microscopy]]== | ==[[Electron microscopy]]== | ||
Features: | Features: | ||
*Sarcomeric like structures - | *Sarcomeric like structures - usually in "bent" cells; cells that are U-shaped. | ||
==Molecular diagnostics== | ==Molecular diagnostics== | ||