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Alveolar soft part sarcoma (view source)
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{{ 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]] | [[Category:Diagnosis]] | ||