Stanford School of Medicine
Surgical Pathology Criteria
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Superficial Myofibroblastoma

Definition

  • Superficial, circumscribed bland lesion of external female genital tract

Alternate/historical names

  • Superficial cervicovaginal myofibroblastoma

Diagnostic Criteria

  • Restricted to external female genital tract
    • Vagina, cervix, vulva
  • Superficial circumscribed
    • No capsule
    • Frequent grenz zone between lesion and epithelium
    • No entrapped fat
  • Uniform bland stellate and spindled cells
    • Oval, elongate nucleus, frequently wavy
    • Even chromatin
    • Small nucleolus
    • 2/26 cases with a few multinucleated cells
    • 4/26 cases with mild pleomorphism
  • Variable patterns of cellularity
    • Moderately to highly cellular overall
    • Densely collagenous foci with vague fascicles
    • Myxoid foci
    • Lace-like or sieve-like pattern
      • Cells surround nodules of collagen or myxoid stroma
  • Mean age 55 years
    • Only 2 of 26 cases under age 38

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

Original posting:: May 27, 2007

Supplemental studies

Immunohistology

Desmin 88%
Smooth muscle actin 0-50%
CD34 50-80%
Estrogen receptor 90%
Progesterone receptor 100%
  • Negative: S100, keratin, EMA, Caldesmon, CD31, HMB45

Differential Diagnosis

The first six entities are predominantly perineal in location and may be confused with superficial myofibroblastoma

Superficial Myofibroblastoma Angiomyofibroblastoma
Inconspicuous vascularity Prominent vascularity
Stellate and spindled cells Perivascular epithelioid cells

 

Fibroepithelial Stromal Polyp Superficial Myofibroblastoma
Wide age range Most 40-70's
Superficial lesional cells, hypocellular core Lesional cells throughout
No grenz zone Usually grenz zone
May have bizzare stromal cells No bizzare stromal cells
No architectural pattern Frequent lace-like pattern

 

Cellular Fibroepithelial Stromal Polyp Superficial Myofibroblastoma
Young, frequently pregnant Most 40-70's
Cellularity predominanlty superficial Cellularity varies throughout
No grenz zone Usually grenz zone
Scattered bizarre or multinucleated cells Uniform bland cells
Mitotic figures may be frequent Mitotic figures <1/10 hpf

 

Superficial Myofibroblastoma Cellular Angiofibroma
Desmin positive Desmin 10% positive
Inconspicuous thin walled vessels Prominent thick walled vessels
No fat Frequently contains fat
ER and PR >90% ER and PR 35-55%
Both are superficial and may be CD34 postive. Cellular angofibroma may occur in males.

 

Superficial Myofibroblastoma Aggressive Angiomyxoma
Age 40-70's Young patients
Small, superficial Large, deep
Circumscribed Frequently infiltrative
Inconspicuous vessels Prominent thick walled hyalinized vessels
Both are frequently positive for desmin, CD34, ER, PR

 

Vaginal Spindle Cell Epithelioma Superficial Myofibroblastoma
Squamous or glandular component No epithelial component
Keratin postive Keratin negative
Desmin negative Desmin positive

 

Superficial myofibroblastoma is restricted to perineal and genital regions, while the entities considered below may occur in a wide variety of sites.

 

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

 

Nerve Sheath Tumor Superficial Myofibroblastoma
S100 positive S100 negative
Desmin negative Desmin positive
Both may be CD34 positive

 

Solitary Fibrous Tumor Superficial Myofibroblastoma
Frequent stag horn vessels Inconspicuous vessels
Desmin negative Desmin positive
CD34 positive CD34 variable

Clinical

  • Restricted to external female genital tract
    • Vagina 22 cases, Cervix 2 cases, Vulva 2 cases
  • Presents as mass or polyp
  • Size 2mm to 6.5 cm
    • Only 1/26 cases >5 cm
  • Mean age 55 years
    • Only 2/26 cases <38 years
  • No recurrences or metastases

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 Ia (Local excision is almost always curative; metastasis never occurs)
  • 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

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
  • Laskin WB, Fetsch JF, Tavassoli FA. Superficial cervicovaginal myofibroblastoma: fourteen cases of a distinctive mesenchymal tumor arising from the specialized subepithelial stroma of the lower female genital tract. Hum Pathol. 2001 Jul;32(7):715-25.
  • Ganesan R, McCluggage WG, Hirschowitz L, Rollason TP. Superficial myofibroblastoma of the lower female genital tract: report of a series including tumours with a vulval location. Histopathology. 2005 Feb;46(2):137-43.
Printed from Surgical Pathology Criteria: http://surgpathcriteria.stanford.edu/
© 2005  Stanford University School of Medicine