Difference between revisions of "Alveolar soft part sarcoma"

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#redirect [[Soft_tissue_lesions#Alveolar_soft_part_sarcoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Alveolar_soft_part_sarcoma_-2-_very_high_mag.jpg
| Width      =
| Caption    = ASPS. [[H&E stain]].
| Micro      = large cells (~30-50 μm) with abundant eosinophilic cytoplasm and an eccentric nucleus +/-nucleolus, arranged in nests/separated by thin septa - vaguely resembles alveoli (at low power)
| Subtypes  =
| LMDDx      = [[paraganglioma]], [[clear cell renal cell carcinoma]], [[alveolar rhabdomyosarcoma]]
| Stains    = PAS +ve (cytoplasm)
| IHC        = TFE3 +ve (suggestive of translocation)
| EM        = intracytoplasmic crystal lattice with ~5 nm fibres spaced 10 nm apart
| Molecular  = t(X;17)
| IF        =
| Gross      =
| Grossing  =
| Site      = [[soft tissue lesions|soft tissue]] - usu. [[head and neck pathology|head and neck]]
| Assdx      =
| Syndromes  =
| Clinicalhx = usu. slow growing mass
| Signs      =
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = ultimately poor
| Other      =
| ClinDDx    =
}}
'''Alveolar soft part sarcoma''', abbreviated '''ASPS''', is a rare [[malignant]] soft tissue lesion typically seen in younger individuals.
 
==General==
*Adolescents/young adults.
*Children -- classically location: orbit and base of [[tongue]].<ref name=pmid21934227>{{Cite journal  | last1 = Anbarasi | first1 = K. | last2 = Sathasivasubramanian | first2 = S. | last3 = Kuruvilla | first3 = S. | title = Alveolar soft-part sarcoma of tongue. | journal = Indian J Pathol Microbiol | volume = 54 | issue = 3 | pages = 581-3 | month =  | year =  | doi = 10.4103/0377-4929.85099 | PMID = 21934227 }}</ref>
*Typical indolent initially - ultimately a poor prognosis.<ref name=pmid17071801>{{Cite journal  | last1 = Folpe | first1 = AL. | last2 = Deyrup | first2 = AT. | title = Alveolar soft-part sarcoma: a review and update. | journal = J Clin Pathol | volume = 59 | issue = 11 | pages = 1127-32 | month = Nov | year = 2006 | doi = 10.1136/jcp.2005.031120 | PMID = 17071801 |PMC = 1860509 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860509/?tool=pubmed }}</ref>
 
==Microscopic==
Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
*Arranged in nests/separated by thin septa; vaguely resembles alveoli (at low power).
*Large cells (~30-50 μm) with abundant eosinophilic cytoplasm.
**May be focally cleared.
*An eccentric nucleus.
*+/-Nucleolus, prominent.
*+/-Multi-nucleation (common).
 
DDx:
*[[Paraganglioma]].
*[[Clear cell renal cell carcinoma]] - areas with cytoplasmic clearing.
*[[Rhabdomyosarcoma]], alveolar - usu. does not have sheets of rhabdoid cells, no cross striations.
 
===Images===
<gallery>
Image:Alveolar soft part sarcoma - very low mag.jpg | ASPS - very low mag. (WC/Nephron)
Image:Alveolar_soft_part_sarcoma_-_low_mag.jpg | ASPS - low mag. (WC/Nephron)
Image:Alveolar_soft_part_sarcoma_-_intermed_mag.jpg | ASPS - intermed. mag. (WC/Nephron)
Image:Alveolar soft part sarcoma - high mag.jpg | ASPS - high mag. (WC/Nephron)
Image:Alveolar soft part sarcoma - very high mag.jpg | ASPS - very high mag. (WC/Nephron)
Image:Alveolar_soft_part_sarcoma_-2-_very_high_mag.jpg | ASPS - very high mag. (WC/Nephron)
</gallery>
www:
*[http://www.webpathology.com/image.asp?case=508&n=10 ASPS - PAS (webpathology.com)].
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860509/figure/fig1/ ASPS - low mag. (nih.gov)].<ref name=pmid17071801/>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860509/figure/fig5/ ASPS - high mag. (nih.gov)].<ref name=pmid17071801/>
 
==Stains==
*PAS +ve (cytoplasmic) - considered the most useful.<ref name=pmid17516754>{{Cite journal  | last1 = Zarrin-Khameh | first1 = N. | last2 = Kaye | first2 = KS. | title = Alveolar soft part sarcoma. | journal = Arch Pathol Lab Med | volume = 131 | issue = 3 | pages = 488-91 | month = Mar | year = 2007 | doi = 10.1043/1543-2165(2007)131[488:ASPS]2.0.CO;2 | PMID = 17516754 }}</ref>
*PASD +ve (cytoplasmic).
 
==IHC==
*TFE3 +ve -- suggestive of characteristic translocation.
*Desmin ~ 50% of cases.<ref name=pmid17071801/>
 
Others:<ref name=pmid17071801/>
*EMA -ve.
*Cytokeratins -ve.
*HMB45 -ve.
*Melan‐A -ve.
*Chromogranin A -ve.
*Synaptophysin -ve.
 
==Molecular==
*t(X;17)(p11.2;q25).<ref>{{OMIM|606243}}</ref>
 
Note:
*Same translocation may be seen in ''[[renal tumour with Xp11.2 translocation]]''.
 
==EM==
*Distinctive membrane-bound intracytoplasmic crystal lattice with ~5 nm fibres spaced 10 nm apart.<ref name=pmid17071801/>
 
Image:
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860509/figure/fig8/ Distinctive crystal lattice in ASPS (nlm.nih.gov)].<ref name=pmid17071801/>
 
==See also==
*[[Soft tissue lesions]].
*[[Head and neck pathology]].
 
==References==
{{Reflist|2}}
 
[[Category:Soft tissue lesions]]
[[Category:Diagnosis]]

Latest revision as of 16:40, 25 December 2014

Alveolar soft part sarcoma
Diagnosis in short

ASPS. H&E stain.
LM large cells (~30-50 μm) with abundant eosinophilic cytoplasm and an eccentric nucleus +/-nucleolus, arranged in nests/separated by thin septa - vaguely resembles alveoli (at low power)
LM DDx paraganglioma, clear cell renal cell carcinoma, alveolar rhabdomyosarcoma
Stains PAS +ve (cytoplasm)
IHC TFE3 +ve (suggestive of translocation)
EM intracytoplasmic crystal lattice with ~5 nm fibres spaced 10 nm apart
Molecular t(X;17)
Site soft tissue - usu. head and neck
Clinical history usu. slow growing mass
Prevalence rare
Prognosis ultimately poor

Alveolar soft part sarcoma, abbreviated ASPS, is a rare malignant soft tissue lesion typically seen in younger individuals.

General

  • Adolescents/young adults.
  • Children -- classically location: orbit and base of tongue.[1]
  • Typical indolent initially - ultimately a poor prognosis.[2]

Microscopic

Features:[3]

  • Arranged in nests/separated by thin septa; vaguely resembles alveoli (at low power).
  • Large cells (~30-50 μm) with abundant eosinophilic cytoplasm.
    • May be focally cleared.
  • An eccentric nucleus.
  • +/-Nucleolus, prominent.
  • +/-Multi-nucleation (common).

DDx:

Images

  • ASPS - very low mag. (WC/Nephron)

  • ASPS - low mag. (WC/Nephron)

  • ASPS - intermed. mag. (WC/Nephron)

  • ASPS - high mag. (WC/Nephron)

  • ASPS - very high mag. (WC/Nephron)

  • ASPS - very high mag. (WC/Nephron)

www:

Stains

  • PAS +ve (cytoplasmic) - considered the most useful.[4]
  • PASD +ve (cytoplasmic).

IHC

  • TFE3 +ve -- suggestive of characteristic translocation.
  • Desmin ~ 50% of cases.[2]

Others:[2]

  • EMA -ve.
  • Cytokeratins -ve.
  • HMB45 -ve.
  • Melan‐A -ve.
  • Chromogranin A -ve.
  • Synaptophysin -ve.

Molecular

  • t(X;17)(p11.2;q25).[5]

Note:

EM

  • Distinctive membrane-bound intracytoplasmic crystal lattice with ~5 nm fibres spaced 10 nm apart.[2]

Image:

See also

References

  1. ↑ Anbarasi, K.; Sathasivasubramanian, S.; Kuruvilla, S.. "Alveolar soft-part sarcoma of tongue.". Indian J Pathol Microbiol 54 (3): 581-3. doi:10.4103/0377-4929.85099. PMID 21934227.
  2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Folpe, AL.; Deyrup, AT. (Nov 2006). "Alveolar soft-part sarcoma: a review and update.". J Clin Pathol 59 (11): 1127-32. doi:10.1136/jcp.2005.031120. PMC 1860509. PMID 17071801. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860509/.
  3. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 627. ISBN 978-0781765275.
  4. ↑ Zarrin-Khameh, N.; Kaye, KS. (Mar 2007). "Alveolar soft part sarcoma.". Arch Pathol Lab Med 131 (3): 488-91. doi:10.1043/1543-2165(2007)131[488:ASPS]2.0.CO;2. PMID 17516754.
  5. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 606243
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