Soft tissue
Fibrohistiocytic tumors
Angiomatoid fibrous histiocytoma

Author: Vijay Shankar, M.D. (see Authors page)

Revised: 13 September 2016, last major update August 2012

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Angiomatoid fibrous histiocytoma [title]

Cite this page: Angiomatoid fibrous histiocytoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/softtissuefhangiomatoid.html. Accessed December 7th, 2016.
Definition / General
  • Uncommon variant with thick pseudocapsule, marked chronic inflammatory infiltrate and cystic areas of hemorrhage
  • Also called fibrohistiocytic tumor of intermediate malignancy
Terminology
  • First described in 1979 (Cancer 1979;44:2147)
  • Formerly called angiomatoid malignant fibrous histiocytoma
  • May not actually be fibrohistiocytic - Rosai believes origin is vessel related myoid cells with inflammatory features
Clinical Features
  • Teens / young adults, often on extremities in areas of lymphoid tissue (popliteal fossa, decubital fossa) or neck
  • Rare in elderly
  • Often fever, malaise, anorexia, anemia, paraproteinemia
  • Unusual sites: lung, mediastinum, ovary, retroperitoneum, vulva (Mod Pathol 2011;24:1560)
Case Reports
Treatment
  • Excision; may recur locally (10%), distant metastases are rare (1%, Hum Pathol 1999;30:1336)
  • Radiotherapy if wide excision margins are not feasible
Clinical Images

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Axillary mass at surgery

Gross Description
  • Circumscribed, multinodular or multicystic hemorrhagic mass
  • Median 2 cm, usually subcutaneous
Micro Description
  • hick fibrous pseudocapsule surrounds nodules, sheets, short fascicles or whorls of monomorphic bland spindle to ovoid eosinophilic cells
  • Often highly cellular with bland histiocytoid cells and hemorrhagic cyst-like spaces, large aggregates of chronic inflammatory cells at edge of tumor in lymphoid follicles
  • May have moderate pleomorphism and mitotic activity
  • Rare clear cells, rhabdomyoblast-like cells, myxoid change in stroma (Mod Pathol 2011;24:1560)
Micro Images

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Resembles out of place lymph node

Chronic inflammatory cells and nodules of cells with round / oval nuclei surround hemorrhagic cystic spaces

Cells surrounding cystic spaces are uniform with round / oval nuclei

Some tumors have moderate pleomorphism



Perls iron, courtesy of Mark R. Wick, M.D.

Courtesy of Mark R. Wick, M.D.



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Morphologic features

Thick fibrous pseudocapsule is present

Dense lymphoplasmacytic infiltrate

H&E and desmin

Cytology Description
  • Histiocyte-like cells in clusters or dispersed
  • Eosinophilic mesenchymal fragments in bloody background with lymphocytes
  • Tumor cells have moderate pleomorphism with abundant fragile cytoplasm and prominent nucleoli (Diagn Cytopathol 2005;33:116)
Positive Stains
Negative Stains
Molecular / Cytogenetics Description
Molecular / Cytogenetics Images

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FISH

t(12;16) karyotype

Diagram

Differential Diagnosis