Stanford School of Medicine
Surgical Pathology Criteria
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Angiomatoid Fibrous Histiocytoma

Definition

  • Predominantly superficial tumor usually containing cystic blood filled spaces and having a prominent peripheral lymphoid infiltrate

Alternate / Historical Names

  • Angiomatoid Malignant Fibrous Histiocytoma

Diagnostic Criteria

  • Circumscribed subcutaneous mass
    • May have a fibrous capsule
    • Rare cases may involve skeletal muscle or periosteum
    • Rare cases centered in dermis
  • Sheets and short fascicles of bland round to short spindled cells
    • Usually arranged in nodules
    • May have epithelioid, histiocytoid or myoid appearance
    • Occasionally contains areas of moderately atypical cells
    • Faintly eosinophilic cytoplasm
    • Infrequent mitotic figures
    • May have occasional foam cells
  • Large blood filled cystic spaces in most cases
    • Lined by lesional round to spindled cells, not by endothelial cells
    • May also have dilated endothelial lined vascular spaces
    • May thrombose
  • Marked peripheral lympho-plasmacytic infiltrate
    • May form germinal centers
    • Present in 80-90% of cases
    • May resemble a lymph node, often in a location where there should not be one
      • Lacks subcapsular sinus and hilar lymphatics
  • 90% <30 years of age
  • Infrequent/rare findings
    • Multinucleated cells
    • Calcifications
    • Marked pleomorphism (no clinical significance)
    • Higher mitotic rate >5/10 hpf (no clinical significance)
  • Richard L Kempson MD
    Robert V Rouse MD
    Department of Pathology
    Stanford University School of Medicine
    Stanford CA 94305-5342

    Original posting/updates: 8/10/08, 2/3/10

 

Supplemental studies

Immunohistology

Calponin 85%
Desmin 50%
EMA 40%
CD99 50%
Smooth muscle actin 15%
Muscle specific actin 15%
H-caldesmon (smooth muscle) 3%
Myoglobin (skeletal muscle) Negative
Myogenin (skeletal muscle) Negative
Keratin Negative
S100 Negative
CD34, CD31 Negative
CD45, CD21, CD35 Negative
CD68 0-67% variable reports
B,T,Kappa,Lambda Mixed in lymphoid component

Genetic analysis

  • Translocations involving EWSR1 or FUS and ATF1 or CREB1 reported in most cases tested
    • FISH for EWSR1 has been demonstrated to be positive in 74% of cases

Differential diagnosis

Metastatic Mesenchymal Neoplasm in a Lymph Node Angiomatoid Fibrous Histiocytoma
Typical nodal architecture Lacks subcapsular sinus and efferent hilar lymphatics
Typical nodal location Frequently in a site where a lymph node would be unusual
Usually has cytologic atypia Usually quite bland
Blood filled cystic spaces unusual for most neoplasms Frequent blood filled cystic spaces

 

Angiosarcoma Angiomatoid Fibrous Histiocytoma
Usually dermal Usually subcutaneous
Usually demonstrates endothelial tufting Cystic spaces lack endothelial lining
CD31 and CD34 positive in most cases CD31 and CD34 negative
Desmin negative Desmin positive in 50% of cases

 

Kaposi Sarcoma Angiomatoid Fibrous Histiocytoma
Usually dermal Usually subcutaneous
Slit-like blood filled spaces Cystic blood filled spaces
CD31 and CD34 positive in most cases CD31 and CD34 negative
Desmin negative Desmin positive in 50% of cases

 

Aneurysmal (Angiectoid)Benign Dermal Fibrous Histiocytoma Angiomatoid Fibrous Histiocytoma
Usually dermal Usually subcutaneous
Lacks fibrous capsule Usually has fibrous capsule
Lacks surrounding lympho-plasmacytic infiltrate Usually has prominent lympho-plasmacytic infiltrate
Frequent overlying epidermal hyperplasia Lacks epidermal hyperplasia
Lacks prominent round cell component Usually has prominent round cell component
Foam cells and giant cells frequent Foam cells and giant cells infrequent
Desmin negative Desmin positive in 50% of cases

 

Rhabdomyosarcoma Angiomatoid Fibrous Histiocytoma
Infiltrative Circumscribed
Lacks surrounding lympho-plasmacytic infiltrate Usually has prominent lympho-plasmacytic infiltrate
Strap cells and/or rhabdoid cytoplasm Scant cytoplasm
Blood filled cystic spaces unusual Frequent blood filled cystic spaces
MyoD1, myoglobin, myogenin positive MyoD1, myoglobulin, myogenin negative

 

Diffuse Tenosynovial Giant Cell Tumor Angiomatoid Fibrous Histiocytoma
Cystic spaces lack blood Cystic blood filled spaces
Numerous giant cells Infrequent giant cells
Scant desmin reactivity May have extensive, strong desmin expression

 

Vascular Malformation / Hemangioma Angiomatoid Fibrous Histiocytoma
Cystic spaces lined by endothelium Cystic spaces not lined by endothelium
Lacks round/spindle cell component Prominent round/spindle cell component

 

Inflammatory Myofibroblastic Tumor Angiomatoid Fibrous Histiocytoma
Blood filled cystic spaces unusual Frequent blood filled cystic spaces
Inflammatory infiltrate usually contains neutrophils Neutrophils unusual in infiltrate

 

Hemangiopericytoma Angiomatoid Fibrous Histiocytoma
Stag horn vascular spaces lined by endothelium Cystic spaces not lined by endothelium
CD34 positive 50% CD34 negative
Desmin negative Desmin positive 50%

Clinical

  • 80% localized to subcutaneous tissue
    • Up to 20% may involve skeletal muscle or periosteum
    • Rare cases in dermis
  • Mean age 15-20 years
    • 90% <30 years
  • Most common on extremities
  • Occasional patients with systemic findings
    • Anemia, fever, weight loss, lymphadenopathy
    • Hypergammaglobulinemia, elevated erythrocyte sedimentation rate
  • Recurrence rate 10-15%
    • Usually only if margins positive
  • Metastatic rate 1-5%
    • Most to local nodes
    • Usually curable by excision of involved nodes
    • Distant metastases may lead to death

Bibliography

  • Kempson RL, Fletcher CDM, Evans HL, Henrickson MR, Sibley RS. Tumors of the Soft Tissues, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 30, 2001
  • Fletcher CDM, Unni KK, Mertens F. Pathology and Genetics of Tumours of Soft Tissue and Bone, World Health Organization Classification of Tumours 2002
  • Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors, 5th edition, 2008
  • Fanburg-Smith JC, Miettinen M. Angiomatoid "malignant" fibrous histiocytoma: a clinicopathologic study of 158 cases and further exploration of the myoid phenotype. Hum Pathol. 1999 Nov;30(11):1336-43.
  • Thway K. Angiomatoid fibrous histiocytoma: a review with recent genetic findings. Arch Pathol Lab Med. 2008 Feb;132(2):273-7.
  • Rossi S, Szuhai K, Ijszenga M, Tanke HJ, Zanatta L, Sciot R, Fletcher CD, Dei Tos AP, Hogendoorn PC. EWSR1-CREB1 and EWSR1-ATF1 fusion genes in angiomatoid fibrous histiocytoma. Clin Cancer Res. 2007 Dec 15;13(24):7322-8.
  • Billings SD, Folpe AL. Cutaneous and subcutaneous fibrohistiocytic tumors of intermediate malignancy: an update. Am J Dermatopathol. 2004 Apr;26(2):141-55.
  • Tanas MR, Rubin BP, Montgomery EA, Turner SL, Cook JR, Tubbs RR, Billings SD, Goldblum JR. Utility of FISH in the diagnosis of angiomatoid fibrous histiocytoma: a series of 18 cases. Mod Pathol. 2010 Jan;23(1):93-7.
 
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