Difference between revisions of "Alveolar soft part sarcoma"

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*[http://www.webpathology.com/image.asp?case=508&n=10 ASPS - PAS (webpathology.com)].
*[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==
==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>
*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>

Revision as of 03:40, 29 September 2013

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 nest/separated by thin septa - vaguely resembles alveoli (at low power)
LM DDx alveolar rhabdomyosarcoma
Stains PAS +ve (cytoplasm)
IHC TFE3 +ve (suggestive of translocation)
Molecular t(X;17)
Site soft tissue - usu. head and neck
Prognosis 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: base of tongue and orbit.
  • Typical indolent initially - ultimately a poor prognosis.[1]

Microscopic

Features:[2]

  • Arranged in nest/separated by thin septa; vaguely resembles alveoli (at low power).
  • Large cells (~30-50 μm) with abundant eosinophilic cytoplasm.
  • An eccentric nucleus.
  • +/-Nucleolus.

DDx:

Images

  • ASPS - low mag. (WC/Nephron)

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

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

www:

Stains

  • PAS +ve (cytoplasmic) - considered the most useful.[3]
  • PASD +ve (cytoplasmic).

IHC

  • TFE3 +ve -- suggestive of characteristic translocation.
  • Desmin ~ 50% of cases.[1]

Others:[1]

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

Molecular

  • t(X;17)(p11.2;q25).[4]

Note:

EM

  • Distinctive membrane-bound intracytoplasmic crystal lattice with ~5 nm fibres spaced 10 nm apart.[1]

Image:

See also

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 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/.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 606243
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