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Surgical Pathology Criteria
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Superficial Angiomyxoma

Definition

  • Bland, superficial myxoid lesion with prominent thin walled vessels

Alternate/Historical Names

  • Cutaneous myxoma

Diagnostic Criteria

  • Predominantly involves dermis and subcutis
    • May involve skeletal muscle on face
    • Multilobulated, poorly circumscribed
  • Myxoid stroma
    • Alcian blue positive, hyaluronidase sensitive
    • May have acellular mucin pools
    • Scattered bland stellate and spindled cells
      • Occasional multinucleated cells
      • Pleomorphism, mitotic figures rare
      • Occasional intranuclear pseudoinclusions
  • Many thin-walled small blood vessels
  • Frequent neutrophils
  • Entrapped epithelial component in 20-30% of cases
    • Keratinous cyst
    • Thin strands of squamous epithelium
    • Basaloid buds
  • If multiple or involving external ear, suggest Carney complex

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

Original posting/updates:: 5/2707, 2/5/10

  • Superficial angiomyxoma may be indistinguishable from the cutaneous lesions of the Carney complex
  • Carney complex should be considered if superficial angiomyxoma is multiple or involves the external ear

Carney complex

  • Myxomas
    • Cutaneous
    • External ear
    • Heart
    • Breast myxoid fibroadenoma
  • Cutaneous melanocytic lesions
    • Lentigines
    • Blue nevus
  • Endocrine hyperplasia and neoplasia
    • Pituitary
    • Thyroid
    • Adrenal cortex
    • Testis large cell calcifying Sertoli cell tumor
  • Psammomatous melanotic schwannoma

Supplemental studies

Immunohistology

CD34 100%
Smooth muscle actin 0-90%
Muscle specific actin 67%
S100 0-40%
Factor XIIIa 60%
  • Negative: keratin, desmin, GFAP, ER, PR

Differential Diagnosis

 

Superficial angiomyxoma may be located in the perineum and external genitalia and thus confused with the first four lesions below.

Superficial Angiomyxoma Aggressive Angiomyxoma
Usually <5 cm Nearly always >5 cm
Superficial Deep seated
Long thin walled vessels Medium to large thick walled vessels
Desmin negative Desmin positive
Stromal neutrophils No stromal neutrophils

 

Superficial Angiomyxoma Angiomyofibroblastoma
Long thin walled vessels Numerous capillaries and venules
Desmin negative Desmin positive
Stromal neutrophils No stromal neutrophils

 

Superficial Angiomyxoma Cellular Angiofibroma
Long thin walled vessels Hyalinized round vessels
Stromal neutrophils Usually stromal lymphocytes

 

Superficial Angiomyxoma Superficial Myofibroblastoma
Mean age 20 years for perineal lesions Most 40-70's
Long thin walled vessels Inconspicuous vessels
Desmin, ER, PR negative Desmin, ER, PR positive
S100 variable S100 negative
Stromal neutrophils No stromal neutrophils
Frequently multinodular Single nodule
Frequently involves subcutis Superficial
Paucicellular Cellular areas

 

Focal Cutaneous Mucinosis Superficial Angiomyxoma
Poorly circumscribed Circumscribed
Few blood vessels Prominent vascular component
Neutrophils uncommon Neutrophils common
Epithelial component rare Frequent epithelial component

 

Myxoid Neurofibroma Superfical Angiomyxoma
Poorly circumscribed Circumscribed
Uniformly S100 positive S100 variable
Intralesional axons No intralesoinal nerve
No epithelial component May have epithelial component

 

Neurothekeoma Superfical Angiomyxoma
Vessels not prominent Prominent vessels
S100 negative S100 variable
Neutrophils infrequent Frequent neutrophils
No epithelial component May have epithelial component
May contain nerve twigs No intralesional nerve fibers
Frequent epithelioid component Lacks epithelioid component

 

Superficial Acral Fibromyxoma Superficial Angiomyxoma
Neutrophils uncommon Neutrophils common
Epithelial component rare Frequent epithelial component
Prominent fibrous component mixed/alternating with moderately cellular myxoid areas Predominant hypocellular myxoid component, frequently in pools
EMA variably positive EMA negative

Clinical

  • Mean age 40 years
    • Female genital cases mean 20 year
    • Range birth to 82
  • Nearly even male:female incidence
    • Genital cases 13:4 female:male
  • Wide range of sites: trunk, limbs, head and neck and perineal
  • 30% recurrence rate
    • No aggressive recurrences
    • No metastases
  • May be associated with Carney complex
    • Especially if multiple or involving external ear

Grading / Staging / Report

Grading and staging not applicable

The surgical pathology report should contain or address the following:

  • Location
  • Type of resection or biopsy
  • Histologic diagnosis
  • Managerial category Ib (Recurrences do occur but are not destructive; never metastasizes)
  • Size
  • Depth (dermis, subcutis, below fascia, body cavity)
  • Margins
    • Involved
    • Not involved
  • Results of supplementary studies if performed
  • Relationship to other specimens from the same patient

Lists

Vulvovaginal stromal and spindled tumors

Soft tissue lesions that frequently are prominently myxoid

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, 4th edition, 2001
  • Calonje E, Guerin D, McCormick D, Fletcher CD.Superficial angiomyxoma: clinicopathologic analysis of a series of distinctive but poorly recognized cutaneous tumors with tendency for recurrence. Am J Surg Pathol. 1999 Aug;23(8):910-7.
  • Fetsch JF, Laskin WB, Tavassoli FA. Superficial angiomyxoma (cutaneous myxoma): a clinicopathologic study of 17 cases arising in the genital region. Int J Gynecol Pathol. 1997 Oct;16(4):325-34.
  • Allen PW, Dymock RB, MacCormac LB. Superficial angiomyxomas with and without epithelial components. Report of 30 tumors in 28 patients. Am J Surg Pathol. 1988 Jul;12(7):519-30.
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