Stanford School of Medicine

Surgical Pathology Criteria
http://surgpathcriteria.stanford.edu/

 use browser back button to return

Aggressive Angiomyxoma

Definition

  • Infiltrative, hypocellular, bland myxoid lesion of perineum and pelvis

Diagnostic Criteria

  • Virtually restricted to deep soft tissue of perineum, external genitalia, inguinal region in both sexes
    • Rare in males
    • Rarely in vagina or pelvis in females
  • Grossly lobulated, infiltrative
    • May surround nerves, fat and adjacent tissues
  • Usually over 10 cm
    • Rarely under 5 cm
  • Practically never extends to overlying epithelium
  • Abundant myxoid stroma
    • Prominent round, medium to large vessels
      • Vessel walls frequently thick, hyalinized
      • Smooth muscle bundles may surround vessels with “pinwheel” appearance
  • Hypocellular
    • Small bland uniform spindle or stellate cells
    • Rare cases with nests of rounder cells similar to angiomyofibroblastoma
      • The two lesions are probably different parts of a spectrum

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

Vimentin Positive
Desmin Positive
Actin Infrequent to variable
CD34 Positive, focal and weak
Factor XIIIa Positive dendritic cells
Estrogen receptor Positive
Progesterone receptor Positive

Cytogenetics

  • t(5;12) and t(8;12) involving the HMGIC region involved in lipomas and leiomyomas have been identified

Differential Diagnosis

The first four entities are predominantly perineal in location and may be confused with aggressive angiomyxoma

Angiomyofibroblastoma Aggressive Angiomyxoma
Most <5 cm Nearly always >5 cm
Well circumscribed Not circumscribed
Alternating hypercellular and hypocellular areas Hypocellular
Clusters of rounded, epithelioid, frequently plasmacytoid cells Sparse spindled or stellate cells
Numerous small vessels Large thick walled vessels
Actin frequently positive Actin infrequently positive

  • Cases with features of both occur raising the possibility that they form a spectrum.
  • Size may be a critical distinguishing feature.
  • Immunohistochemistry reports of actin staining are variable and thus it may not be reliable for separation; both are desmin positive.
  • Aggressive Angiomyxoma Cellular Angiofibroma
    Usually large, deep lesion Subcutaneous, mean size 6 cm
    Hypocellular Moderately cellular
    Infiltrative margin Circumscribed
    Usually desmin positive Desmin 10% positive
    May infiltrate fat May have fat integral to lesion
    Both have prominent medium sized vessels, both may be CD34+.

    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

    Fibroepithelial stromal polyp Aggressive Angiomyxoma
    Exophytic Sessile, deep lesion
    Often extends to overlying epithelium Practically never extends to overlying epithelium
    Vaginal or cervical Usually in soft tissue
    Usually <5 cm Nearly always >5 cm
    May have scattered atypical stromal cells No atypical cells
    Both contain desmin positive cells

    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

     

    Aggressive angiomyxoma is virtually restricted to perineal, genital, inguinal regions, while the myxoid entities considered below may occur in a wide variety of sites.

    Intramuscular Myxoma Aggressive Angiomyxoma
    Frequently on extremities Perineal or pelvic
    Hypovascular Prominent medium to large vessels

    Myxoid neurofibroma Aggressive angiomyxoma
    S100 positive S100 negative
    Desmin negative Desmin positive

    Myxofibrosarcoma Aggressive angiomyxoma
    Large cytologically atypical cells Small bland cells
    May have frequent mitotic figures Rare mitotic figures

    Myxoid Liposarcoma Aggressive Angiomyxoma
    Arborizing vessels Large, disorganized vessels
    Signet ring lipoblasts Lacks lipoblasts

    Clinical

    • Nearly all age 20-50 years
      • Reports of 13, 8 and 1 year old males
      • One report in an 11 year old female
    • Most occur in females
      • About 5% in males
    • Virtually restricted to soft tissue of perineum, external genitalia, inguinal region in both sexes
      • Rarely in vagina or pelvis in females
    • Usually over 10 cm
      • Rarely under 5 cm
    • Can recur
      • Current recurrence rate is significantly lower than reported in early series
      • Two cases of metastasis reported, one with death

    Grading / Staging / Report

    Grading and staging are not applicable

    The surgical pathology report should contain or address the following:

    • Location
    • Type of resection or biopsy
    • Histologic diagnosis
    • Managerial category II (Local recurrence can occur but is rarely destructive; only two cases with metastasis reported)
      • Almost all recurrent cases are cured by second excision
    • 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
    • Amezcua CA, Begley SJ, Mata N, Felix JC, Ballard CA. Aggressive angiomyxoma of the female genital tract: a clinicopathologic and immunohistochemical study of 12 cases. Int J Gynecol Cancer. 2005 Jan-Feb;15(1):140-5.
    • Begin LR, Clement PB, Kirk ME, Jothy S, McCaughey WT, Ferenczy A. Aggressive angiomyxoma of pelvic soft parts: a clinicopathologic study of nine cases. Hum Pathol 1985 Jun;16(6):621-8.
    • Bigotti G, Coli A, Gasbarri A, Castagnola D, Madonna V, Bartolazzi A. Angiomyofibroblastoma and aggressive angiomyxoma: two benign mesenchymal neoplasms of the female genital tract. An immunohistochemical study. Pathol Res Pract 1999;195(1):39-44.
    • Blandamura S, Cruz J, Faure Vergara L, Machado Puerto I, Ninfo V. Aggressive angiomyxoma: a second case of metastasis with patient's death. Hum Pathol. 2003 Oct;34(10):1072-4.
    • Carlinfante G, De Marco L, Mori M, Ferretti S, Crafa P. Aggressive angiomyxoma of the spermatic cord. Two unusual cases occurring in childhood. Pathol Res Pract 2001;197(2):139-44.
    • Clatch RJ, Drake WK, Gonzalez JG. Aggressive angiomyxoma in men. A report of two cases associated with inguinal hernias. Arch Pathol Lab Med 1993 Sep;117(9):911-3.
    • Fetsch JF, Laskin WB, Lefkowitz M, Kindblom LG, Meis-Kindblom JM. Aggressive angiomyxoma: a clinicopathologic study of 29 female patients. Cancer 1996 Jul 1;78(1):79-90.
    • Granter SR, Nucci MR, Fletcher CD. Aggressive angiomyxoma: reappraisal of its relationship to angiomyofibroblastoma in a series of 16 cases. Histopathology 1997 Jan;30(1):3-10.
    • Iezzoni JC, Fechner RE, Wong LS, Rosai J. Aggressive angiomyxoma in males. A report of four cases. Am J Clin Pathol 1995 Oct;104(4):391-6.
    • Kazmierczak B, Dal Cin P, Wanschura S, Bartnitzke S, Van den Berghe H, Bullerdiek J. Cloning and molecular characterization of part of a new gene fused to HMGIC in mesenchymal tumors. Am J Pathol 1998 Feb;152(2):431-5.
    • Kim HS, Park SH, Chi JG. Aggressive angiomyxoma of childhood: two unusual cases developed in the scrotum. Pediatr Dev Pathol. 2003 Mar-Apr;6(2):187-91. 8 and 1 yr old boys
    • McCluggage WG, Patterson A, Maxwell P. Aggressive angiomyxoma of pelvic parts exhibits oestrogen and progesterone receptor positivity. J Clin Pathol 2000 Aug;53(8):603-5.
    • Nucci MR, Weremowicz S, Neskey DM, Sornberger K, Tallini G, Morton CC, Quade BJ. Chromosomal translocation t(8;12) induces aberrant HMGIC expression in aggressive angiomyxoma of the vulva. Genes Chromosomes Cancer 2001 Oct;32(2):172-6.
    • Ockner DM, Sayadi H, Swanson PE, Ritter JH, Wick MR. Genital angiomyofibroblastoma. Comparison with aggressive angiomyxoma and other myxoid neoplasms of skin and soft tissue. Am J Clin Pathol 1997 Jan;107(1):36-44.
    • Siassi RM, Papadopoulos T, Matzel KE. Metastasizing aggressive angiomyxoma. N Engl J Med 1999 Dec 2;341(23):1772 Letter
    • Silverman JS, Albukerk J, Tamsen A. Comparison of angiomyofibroblastoma and aggressive angiomyxoma in both sexes: four cases composed of bimodal CD34 and factor XIIIa positive dendritic cell subsets. Pathol Res Pract 1997;193(10):673-82.
    • Skalova A, Michal M, Husek K, Zamecnik M, Leivo I. Aggressive angiomyxoma of the pelvioperineal region. Immunohistological and ultrastructural study of seven cases. Am J Dermatopathol 1993 Oct;15(5):446-51.
    • Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm. Am J Surg Pathol 1983 Jul;7(5):463-75.
    • Tsang WY, Chan JK, Lee KC, Fisher C, Fletcher CD. Aggressive angiomyxoma. A report of four cases occurring in men. Am J Surg Pathol 1992 Nov;16(11):1059-65.
    • White J, Chan YF. Aggressive angiomyxoma of the vulva in an 11-year-old girl. Pediatr Pathol 1994 Jan-Feb;14(1):27-37.
    Printed from Surgical Pathology Criteria: http://surgpathcriteria.stanford.edu/
    © 2005  Stanford University School of Medicine