Angiomatoid fibrous histiocytoma
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|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|
Angiomatoid fibrous histiocytoma, abbreviated AFH, is a rare soft tissue lesion that is typically seen in children and young adults.
- Rarely metastasizes.
- Children & young adults.
- Should be completely excised.
- Usu. soft tissue of the extremities.
- Cystic spaces with blood - simulates a vascular neoplasm.
- Epithelioid to spindle cells.
- May have a histiocytic appearance.
- Lymphoid cuff - lymphocytes around periphery of lesion.
- 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).
- CD68 +ve.
- CD57 +ve.
- Desmin +ve (focal).
- Vimentin +ve.
AFH has recurrent translocations:
- t(12;16) FUS/ATF1.
- t(12;22) EWS/ATF1.
- 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.
- 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.