Angiomatoid fibrous histiocytoma
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Angiomatoid fibrous histiocytoma, abbreviated AFH, is a rare soft tissue lesion that is typically seen in children and young adults.
| 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 |
General
- Rarely metastasizes.
- Children & young adults.
- Should be completely excised.
Gross
- Usu. soft tissue of the extremities.[citation needed]
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
AFH - intermed. mag. (WC/Nephron)
AFH - high mag. (WC/Nephron)
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
- ↑ 1.0 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.
- ↑ 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.
- ↑ URL: http://dermatology.cdlib.org/1605/1_case_reports/4_09-00041/patrizi.html. Accessed on: 15 November 2011.
- ↑ 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.