Difference between revisions of "Angiomatoid fibrous histiocytoma"

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#redirect [[Soft_tissue_lesions#Angiomatoid_fibrous_histiocytoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Angiomatoid_fibrous_histiocytoma_-_intermed_mag.jpg
| Width      =
| Caption    = Angiomatoid fibrous histiocytoma. [[H&E stain]].
| Synonyms  =
| Micro      = cystic spaces with blood +/- histiocytic appearance; inflammation (lymphocytes around periphery of lesion); hemorrhage
| Subtypes  =
| LMDDx      =
| Stains    =
| IHC        = CD68 +ve, CD57 +ve, desmin +ve (focal), vimentin +ve
| EM        =
| Molecular  = t(12;16) FUS/ATF1, t(12;22) EWS/ATF1, others
| IF        =
| Gross      =
| Grossing  =
| Site      = [[soft tissue lesions|soft tissue]] - usu. extremities
| Assdx      =
| Syndromes  =
| Clinicalhx = children, young adults
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = usually good
| Other      =
| ClinDDx    =
| Tx        = complete excision
}}
'''Angiomatoid fibrous histiocytoma''', abbreviated '''AFH''', is a rare [[soft tissue lesion]] that is typically seen in children and young adults.
 
==General==
*Rarely metastasizes.
*Children & young adults.
*Should be completely excised.
 
==Gross==
*Usu. soft tissue of the extremities.{{fact}}
 
==Microscopic==
Features:<ref name=Ref_WMSP624-5>{{Ref WMSP|624-5}}</ref>
*Cystic spaces with blood - simulates a vascular neoplasm.<ref name=pmid228836>{{Cite journal  | last1 = Enzinger | first1 = FM. | title = Angiomatoid malignant fibrous histiocytoma: a distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm. | journal = Cancer | volume = 44 | issue = 6 | pages = 2147-57 | month = Dec | year = 1979 | doi =  | PMID = 228836 }}</ref>
*Epithelioid to spindle cells.
**May have a histiocytic appearance.<ref>URL: [http://dermatology.cdlib.org/1605/1_case_reports/4_09-00041/patrizi.html http://dermatology.cdlib.org/1605/1_case_reports/4_09-00041/patrizi.html]. Accessed on: 15 November 2011.</ref>
*Inflammation.
**Lymphoid cuff<ref name=pmid20154033/> - lymphocytes around periphery of lesion.
*Hemorrhage.
 
Note:
*The first impression may be that it is [[granuloma|granulomatous inflammation]]; however, the cytoplasm doesn't fit (it isn't bubbly and it isn't sheet-like), and the nuclei aren't quite right (few footprint shaped nuclei).
 
===Images===
<gallery>
Image: Angiomatoid fibrous histiocytoma - low mag.jpg | AFH - low mag.
Image: Angiomatoid fibrous histiocytoma - intermed mag.jpg | AFH - intermed. mag.
Image: Angiomatoid fibrous histiocytoma - high mag.jpg | AFH - high mag.
Image: Angiomatoid fibrous histiocytoma - very high mag.jpg | AFH - very high mag.
 
Image: Angiomatoid fibrous histiocytoma - intermed mag - 2.jpg | AFH - intermed. mag.
Image: Angiomatoid fibrous histiocytoma - high mag - 2.jpg | AFH - high mag.
Image: Angiomatoid fibrous histiocytoma - very high mag - 2.jpg | AFH - very high mag.
</gallery>
www:
*[http://www.sarcomaimages.com/index.php?v=Angiomatoid-Fibrous-Histiocytoma AFH (sarcomaimages.com)].
*[http://jcp.bmj.com/content/63/2/124/F1.large.jpg AFH (bmj.com)].<ref name=pmid20154033>{{Cite journal  | last1 = Matsumura | first1 = T. | last2 = Yamaguchi | first2 = T. | last3 = Tochigi | first3 = N. | last4 = Wada | first4 = T. | last5 = Yamashita | first5 = T. | last6 = Hasegawa | first6 = T. | title = Angiomatoid fibrous histiocytoma including cases with pleomorphic features analysed by fluorescence in situ hybridisation. | journal = J Clin Pathol | volume = 63 | issue = 2 | pages = 124-8 | month = Feb | year = 2010 | doi = 10.1136/jcp.2009.072256 | PMID = 20154033 | url = http://jcp.bmj.com/content/63/2/124.full }}
</ref>
*[http://path.upmc.edu/cases/case512.html AFH - several images (upmc.edu)].
 
==IHC==
Features:<ref name=Ref_WMSP624-5>{{Ref WMSP|624-5}}</ref>
*CD68 +ve.
*CD57 +ve.
*Desmin +ve (focal).
*Vimentin +ve.
 
==Molecular==
AFH has recurrent [[translocations]]:
*t(12;16) FUS/ATF1.
*t(12;22) EWS/ATF1.
 
==See also==
*[[Soft tissue lesions]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Soft tissue lesions]]
[[Category:Pediatric pathology]]

Latest revision as of 02:53, 22 June 2014

Angiomatoid fibrous histiocytoma
Diagnosis in short

Angiomatoid fibrous histiocytoma. H&E stain.

LM cystic spaces with blood +/- histiocytic appearance; inflammation (lymphocytes around periphery of lesion); hemorrhage
IHC CD68 +ve, CD57 +ve, desmin +ve (focal), vimentin +ve
Molecular t(12;16) FUS/ATF1, t(12;22) EWS/ATF1, others
Site soft tissue - usu. extremities

Clinical history children, young adults
Prevalence uncommon
Prognosis usually good
Treatment complete excision

Angiomatoid fibrous histiocytoma, abbreviated AFH, is a rare soft tissue lesion that is typically seen in children and young adults.

General

  • Rarely metastasizes.
  • Children & young adults.
  • Should be completely excised.

Gross

Microscopic

Features:[1]

  • Cystic spaces with blood - simulates a vascular neoplasm.[2]
  • Epithelioid to spindle cells.
    • May have a histiocytic appearance.[3]
  • Inflammation.
    • Lymphoid cuff[4] - lymphocytes around periphery of lesion.
  • Hemorrhage.

Note:

  • The first impression may be that it is granulomatous inflammation; however, the cytoplasm doesn't fit (it isn't bubbly and it isn't sheet-like), and the nuclei aren't quite right (few footprint shaped nuclei).

Images

www:

IHC

Features:[1]

  • CD68 +ve.
  • CD57 +ve.
  • Desmin +ve (focal).
  • Vimentin +ve.

Molecular

AFH has recurrent translocations:

  • t(12;16) FUS/ATF1.
  • t(12;22) EWS/ATF1.

See also

References

  1. 1.0 1.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 624-5. ISBN 978-0781765275.
  2. Enzinger, FM. (Dec 1979). "Angiomatoid malignant fibrous histiocytoma: a distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm.". Cancer 44 (6): 2147-57. PMID 228836.
  3. URL: http://dermatology.cdlib.org/1605/1_case_reports/4_09-00041/patrizi.html. Accessed on: 15 November 2011.
  4. 4.0 4.1 Matsumura, T.; Yamaguchi, T.; Tochigi, N.; Wada, T.; Yamashita, T.; Hasegawa, T. (Feb 2010). "Angiomatoid fibrous histiocytoma including cases with pleomorphic features analysed by fluorescence in situ hybridisation.". J Clin Pathol 63 (2): 124-8. doi:10.1136/jcp.2009.072256. PMID 20154033. http://jcp.bmj.com/content/63/2/124.full.