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
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.