Difference between revisions of "Pleomorphic undifferentiated sarcoma"

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#redirect [[Soft_tissue_lesions#Pleomorphic_undifferentiated_sarcoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Pleomorphic_undifferentiated_sarcoma_-_very_high_mag.jpg
| Width      =
| Caption    = Pleomorphic undifferentiated sarcoma. [[H&E stain]].
| Synonyms  = undifferentiated pleomorphic sarcoma (UPS), malignant fibrous histiocytoma (MFH)
| Micro      = storiform pattern ([[AKA]] ''patternless pattern''), marked [[nuclear pleomorphism]], mitoses, necrosis (common), mix of spindle cells and epithelioid cells, deep to skin
| Subtypes  =
| LMDDx      = [[atypical fibroxanthoma]] (superficial), [[dedifferentiated liposarcoma]], [[leiomyosarcoma]],[[metaplastic carcinoma]], [[malignant melanoma]], [[rhabdomyosarcoma]], [[synovial sarcoma]],[[undifferentiated endometrial sarcoma]], others
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = deep [[soft tissue lesion|soft tissue]] - trunk and extremities
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = uncommon overall
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = poor
| Other      =
| ClinDDx    =
| Tx        = surgery if feasible
}}
'''Pleomorphic undifferentiated sarcoma''', abbreviated '''PUS''', is an undifferentiated malignant [[soft tissue lesion]].
 
It is also known as '''undifferentiated pleomorphic sarcoma''' (abbreviated '''UPS'''). Previously, it was known as '''malignant fibrous histiocytoma''', commonly abbreviated '''MFH'''.<ref>URL: [http://sarcomahelp.org/learning_center/mfh.html http://sarcomahelp.org/learning_center/mfh.html]. Accessed on: 8 April 2011.</ref>
 
==General==
*Common sarcoma.
*Usually deep tissue of the trunk and extremities.
*A diagnosis of exclusion<ref name=pmid19671033>{{cite journal |author=Matushansky I, Charytonowicz E, Mills J, Siddiqi S, Hricik T, Cordon-Cardo C |title=MFH classification: differentiating undifferentiated pleomorphic sarcoma in the 21st Century |journal=Expert Rev Anticancer Ther |volume=9 |issue=8 |pages=1135–44 |year=2009 |month=August |pmid=19671033 |pmc=3000413 |doi=10.1586/era.09.76 |url=}}</ref> / wastebasket for unclassifiable high grade sarcomas.
 
==Microscopic==
Features:<ref name=Ref_WMSP_613>{{Ref WMSP|613}}</ref>
*Storiform pattern ([[AKA]] ''patternless pattern'') - '''key feature'''.
*Marked [[nuclear pleomorphism]] '''key feature'''.
**Variation is nuclear size, nuclear shape and nuclear staining (esp. hyperchromasia).
*Mitoses - abundant; atypical mitoses common.
*Necrosis (common).
*Mix of spindle cells and epithelioid cells.
*Deep to skin  - '''important'''.
 
Other findings:
*+/-Giant cells (see subclassification).
*+/-Inflammation (see subclassification).
**Neutrophils.
**Eosinophils.
 
Notes:
*Superficial lesions with the morphology of ''PUS'' are called by some ''[[atypical fibroxanthoma]]s'' (AFXs).
 
DDx:
*[[Atypical fibroxanthoma]] (AFX) - superficial skin.
*[[Dedifferentiated liposarcoma]].
*[[Leiomyosarcoma]].
*[[Metaplastic carcinoma]].
*[[Malignant melanoma]].
*[[Rhabdomyosarcoma]].
*[[Synovial sarcoma]].
*[[Undifferentiated endometrial sarcoma]] - uterus.
*Others.
 
===Images===
<gallery>
Image:Pleomorphic_undifferentiated_sarcoma_-_very_high_mag.jpg | PUS - high mag. (WC/Nephron)
Image:Pleomorphic_undifferentiated_sarcoma_-_intermed_mag.jpg | PUS - intermed. mag. (WC/Nephron)
</gallery>
[[File:3 13646052598462 sl 1.png|Pleomorphic sarcoma]]
[[File:3 13646052598462 sl 2.png|Pleomorphic sarcoma]]
[[File:3 13646052598462 sl 3.png|Pleomorphic sarcoma]]
[[File:3 13646052598462 sl 4.png|Pleomorphic sarcoma]]<br>
Pleomorphic sarcoma in thigh of late middle aged man. A. Tumor haphazardly spreads and invades and surrounds degenerated skeletal muscle fibers. B. Bizarre, sometimes multinucleated cells are seen. C. In other areas, spindle cells predominate. D. Invasion of adipose tissue by tumor should not be interpreted as proof of dedifferentiated liposarcoma; lipoblasts should be seen.  Vital is remembering this is a diagnosis of exclusion, with negative keratin, CD34, CD30,  S100, and CD31 stains being suggested.
 
===Subclassification===
Pleomorphic sarcoma (PS) is subclassified the following way:<ref name=Ref_WMSP_613-4>{{Ref WMSP|613-4}}</ref>
*PS with giant cells.
*PS with inflammation.
*PUS (not otherwise specified) - wastebasket diagnosis; if neither of the above two apply.
 
==IHC==
Exclusionary stains - should be negative:
*AE1/AE3.
*p63.
*Myogenin.
*S-100.
*HMB-45.
 
Usually negative, may be positive:<ref name=Ref_WMSP613>{{Ref WMSP|613}}</ref>
*Desmin.
*SMA.
 
Commonly positive:
*CD68.<ref name=Ref_WMSP613>{{Ref WMSP|613}}</ref>
*Vimentin.
 
==See also==
*[[Sarcoma]].
 
==References==
{{Reflist|2}}
 
[[Category:Diagnosis]]
[[Category:Soft tissue lesions]]

Latest revision as of 18:04, 2 January 2017

Pleomorphic undifferentiated sarcoma
Diagnosis in short

Pleomorphic undifferentiated sarcoma. H&E stain.
Synonyms undifferentiated pleomorphic sarcoma (UPS), malignant fibrous histiocytoma (MFH)
LM storiform pattern (AKA patternless pattern), marked nuclear pleomorphism, mitoses, necrosis (common), mix of spindle cells and epithelioid cells, deep to skin
LM DDx atypical fibroxanthoma (superficial), dedifferentiated liposarcoma, leiomyosarcoma,metaplastic carcinoma, malignant melanoma, rhabdomyosarcoma, synovial sarcoma,undifferentiated endometrial sarcoma, others
Site deep soft tissue - trunk and extremities
Prevalence uncommon overall
Prognosis poor
Treatment surgery if feasible

Pleomorphic undifferentiated sarcoma, abbreviated PUS, is an undifferentiated malignant soft tissue lesion.

It is also known as undifferentiated pleomorphic sarcoma (abbreviated UPS). Previously, it was known as malignant fibrous histiocytoma, commonly abbreviated MFH.[1]

General

  • Common sarcoma.
  • Usually deep tissue of the trunk and extremities.
  • A diagnosis of exclusion[2] / wastebasket for unclassifiable high grade sarcomas.

Microscopic

Features:[3]

  • Storiform pattern (AKA patternless pattern) - key feature.
  • Marked nuclear pleomorphism key feature.
    • Variation is nuclear size, nuclear shape and nuclear staining (esp. hyperchromasia).
  • Mitoses - abundant; atypical mitoses common.
  • Necrosis (common).
  • Mix of spindle cells and epithelioid cells.
  • Deep to skin - important.

Other findings:

  • +/-Giant cells (see subclassification).
  • +/-Inflammation (see subclassification).
    • Neutrophils.
    • Eosinophils.

Notes:

DDx:

Images

  • PUS - high mag. (WC/Nephron)

  • PUS - intermed. mag. (WC/Nephron)

Pleomorphic sarcoma Pleomorphic sarcoma Pleomorphic sarcoma Pleomorphic sarcoma
Pleomorphic sarcoma in thigh of late middle aged man. A. Tumor haphazardly spreads and invades and surrounds degenerated skeletal muscle fibers. B. Bizarre, sometimes multinucleated cells are seen. C. In other areas, spindle cells predominate. D. Invasion of adipose tissue by tumor should not be interpreted as proof of dedifferentiated liposarcoma; lipoblasts should be seen. Vital is remembering this is a diagnosis of exclusion, with negative keratin, CD34, CD30, S100, and CD31 stains being suggested.

Subclassification

Pleomorphic sarcoma (PS) is subclassified the following way:[4]

  • PS with giant cells.
  • PS with inflammation.
  • PUS (not otherwise specified) - wastebasket diagnosis; if neither of the above two apply.

IHC

Exclusionary stains - should be negative:

  • AE1/AE3.
  • p63.
  • Myogenin.
  • S-100.
  • HMB-45.

Usually negative, may be positive:[5]

  • Desmin.
  • SMA.

Commonly positive:

  • CD68.[5]
  • Vimentin.

See also

References

  1. ↑ URL: http://sarcomahelp.org/learning_center/mfh.html. Accessed on: 8 April 2011.
  2. ↑ Matushansky I, Charytonowicz E, Mills J, Siddiqi S, Hricik T, Cordon-Cardo C (August 2009). "MFH classification: differentiating undifferentiated pleomorphic sarcoma in the 21st Century". Expert Rev Anticancer Ther 9 (8): 1135–44. doi:10.1586/era.09.76. PMC 3000413. PMID 19671033. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3000413/.
  3. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 613. ISBN 978-0781765275.
  4. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 613-4. ISBN 978-0781765275.
  5. ↑ 5.0 5.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 613. ISBN 978-0781765275.
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