Stanford School of Medicine
Surgical Pathology Criteria
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Inflammatory Myxohyaline Tumor of Distal Extremities

Definition

  • Neoplasm containing myxoid and hyalinized areas with a prominent inflammatory infiltrate and cells with large bizarre nuclei

Alternate / Historical Names

  • Acral myxoinflammatory fibroblastic sarcoma

Diagnostic Criteria

  • Multinodular
    • Infiltrates subcutaneous fat
    • Rarely may infiltrate into synovium, dermis or underlying muscle
  • Myxoid nodules with a surrounding cellular to hyalinized stroma
    • Hemosiderin may be present
  • Scattered large atypical cells may resemble virocytes or Reed-Sternberg cells
    • Short spindled to epithelioid cells
    • Large vesicular nuclei
      • May be markedly pleomorphic
      • Prominent inclusion-like nucleoli
      • May be binucleate or multinucleated
    • Abundant eosinophilic cytoplasm
      • Homogeneous to vacuolated
      • Vacuoles may indent nuclei
      • May have cells resembling lipoblasts or ganglion cells
    • May show emperipolesis of inflammatory cells
  • Few mitotic figures
    • May be atypical
  • Extensive inflammatory infiltrate
    • Lymphocytes, eosinophils, plasma cells, histiocytes primarily in solid areas
    • Neutrophils primarily in myxoid areas
  • Located on extremities, usually distal

    • Richard L Kempson MD
    Robert V Rouse MD
    Department of Pathology
    Stanford University School of Medicine
    Stanford CA 94305-5342

    Original posting: August 10, 2008

 

Supplemental studies

Immunohistology

Vimentin positive
Keratin focal, weak
CD68 variable, focal
CD34 variable, focal
S100, desmin, actin, HMB45 negative
EMA, NSE, CMV negative
CD15, CD30, CD45 negative
Ki67 <1% of large cells

Genetic Analysis

PCR shows no active EBV infection

Differential diagnosis

Infection or Nonspecific Inflammation Inflammatory Myxohyaline Tumor of Distal Extremities
Lacks large atypical cells Large atypical cells often with prominent nucleoli
Culture, stains may be positive Culture, stains negative for organisms

 

Ganglion Cyst Inflammatory Myxohyaline Tumor of Distal Extremities
Lacks large atypical cells Large atypical cells
Inflammation variable Prominent inflammation

 

Superficial Acral Fibromyxoma Inflammatory Myxohyaline Tumor of Distal Extremities
Lacks atypical Reed Sternberg like cells Atypical Reed Sternberg like cells
Lacks hyaline stroma Hyaline stroma present
Lacks inflammatory infiltrate Inflammatory infitrate present including neutrophils in myxoid areas

 

Juxta-articular Myxoma Inflammatory Myxohyaline Tumor of Distal Extremities
Lacks large atypical cells Large atypical cells
Large joints involved Distal extremities
Paucicellular Cellular in areas

 

Proliferative Fasciitis Inflammatory Myxohyaline Tumor of Distal Extremities
Ganglion-like cells not atypical Large atypical cells (may also have ganglion-like cells)
Lacks inflammation Prominent inflammation

 

Inflammatory Myofibroblastic Tumor Inflammatory Myxohyaline Tumor of Distal Extremities
Spindle cells arranged in a fascicular pattern Spindle cells randomly distributed
Rare in extremities Distal extremities

 

Hodgkin Lymphoma Inflammatory Myxohyaline Tumor of Distal Extremities
Myxoid stroma rare Myxoid stroma present
No cytoplasmic vacuolation Cytoplasmic vacuolation
CD15, CD30 positive CD15, CD30 negative

 

Myxofibrosarcoma Inflammatory Myxohyaline Tumor of Distal Extremities
Rare in distal extremities Distal extremities
May have frequent mitotic figures Infrequent mitotic figures
Infrequent inflammation Prominent inflammation

 

Atypical Lipomatous Tumor Inflammatory Myxohyaline Tumor of Distal Extremities
Mature fat component No mature fat
Lipoblasts No true lipoblasts

 

Myxoid Liposarcoma Inflammatory Myxohyaline Tumor of Distal Extremities
Lacks large atypical cells Large atypical cells
Plexiform vascular pattern No plexiform vascular pattern
Rare in distal extremities Distal extremities
May have lipoblasts No true lipoblasts

 

Pleomorphic Liposarcoma Inflammatory Myxohyaline Tumor of Distal Extremities
Very rare on distal extremities Distal extremities
Lipoblasts No true lipoblasts
Lacks inflammation Prominent inflammation

 

Epithelioid Sarcoma Inflammatory Myxohyaline Tumor of Distal Extremities
Lacks large Reed-Sternberg like atypical cells Large Reed-Sternberg like atypical cells
Keratin positive Keratin variable, weak, focal
Frequent necrosis No necrosis
Myxoid stroma rare Myxoid stroma

 

Tenosynovial Giant Cell Tumor Inflammatory Myxohyaline Tumor of Distal Extremities
Lacks large atypical cells Large atypical cells
Inflammation variable Prominent inflammation

Clinical

  • Age range 4-91 years, most 40-60
  • Located in subcutaneous fat
  • Most on hands, feet, wrists, ankles
    • Rarely on proximal leg or arm
  • Recurrences may be aggressive
    • One recurrence ascended arm
  • One documented lymph node metastasis
    • One non-documented case of lung metastases

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
  • Lambert I, Debiec-Rychter M, Guelinckx P, Hagemeijer A, Sciot R.  Acral myxoinflammatory fibroblastic sarcoma with unique clonal chromosomal changes.  Virchows Arch 2001 May;438(5):509-12.
  • Ebhardt H, Kosmehl H, Katenkamp D.  [Acral myxoinflammatory fibroblastic sarcoma. Six cases of a tumor entity]  Pathologe 2001 Mar;22(2):157-61.
  • Meis-Kindblom JM, Kindblom LG.  Acral myxoinflammatory fibroblastic sarcoma: a low-grade tumor of the hands and feet.  Am J Surg Pathol 1998 Aug;22(8):911-24.
  • Montgomery EA, Devaney KO, Giordano TJ, Weiss SW.  Inflammatory myxohyaline tumor of distal extremities with virocyte or Reed-Sternberg-like cells: a distinctive lesion with features simulating inflammatory conditions, Hodgkin's disease, and various sarcomas.  Mod Pathol 1998 Apr;11(4):384-91.
  • Sakaki M, Hirokawa M, Wakatsuki S, Sano T, Endo K, Fujii Y, Ikeda T, Kawaguchi S, Hirose T, Hasegawa T. Acral myxoinflammatory fibroblastic sarcoma: a report of five cases and review of the literature. Virchows Arch. 2003 Jan;442(1):25-30.
  • Jurcić V, Zidar A, Montiel MD, Frković-Grazio S, Nayler SJ, Cooper K, Suster S, Lamovec J. Myxoinflammatory fibroblastic sarcoma: a tumor not restricted to acral sites. Ann Diagn Pathol. 2002 Oct;6(5):272-80.
 
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