Skip to main content

Advertisement

SpringerLink
Log in

Aggressive angiomyxoma of the thigh

  • Case Report
  • Published:
Skeletal Radiology Aims and scope Submit manuscript

Abstract

Aggressive angiomyxoma is a rare tumour that typically occurs in the perineum in women of reproductive age. A small number of cases occurring in men have been reported, all of which were located in the low pelvis, perineum or scrotum. While benign, the tumour is locally infiltrative and consequently has a high rate of local recurrence following surgery; therefore, accurate pre-operative diagnosis is important. The characteristic location of these tumours in the low pelvis or perineum has led to speculation that aggressive angiomyxomas arise from a mesenchymal cell that is unique to the perineum. We describe a case of aggressive angiomyxoma arising in the thigh of a 54-year-old man, which we believe is the first reported instance of this rare neoplasm occurring remote from the pelvis or perineum in a male patient. Cross-sectional imaging demonstrated a well-defined mass that had low density on CT and high intensity on fluid-sensitive MR sequences. Biopsy was non-diagnostic and excision was performed. At histological analysis, the tumour exhibited the characteristic features of aggressive angiomyxoma, with bland spindle cells and large, hyalinised blood vessels in a hypocellular myxoid matrix. Extensive immunohistochemical staining further supported the diagnosis. While the imaging features of these tumours are non-specific and suggestive of myxoid neoplasms, the diagnosis should be considered whenever biopsy of a myxoid-appearing mass yields hypocellular, non-diagnostic material, despite adequate sampling.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Yamashita Y, Tokunaga O, Goto M. Aggressive angiomyxoma of the oral floor: report of a case. J Oral Maxillofac Surg 2004; 62: 1429–1431.

    Article  Google Scholar 

  2. De La Ossa M, Castellano-Sanchez A, Alvarez E, Smoak W, Robinson M. Sonographic appearance of aggressive myxoma of the scrotum. J Clin Ultrasound 2001; 29(8): 476–478.

    Article  Google Scholar 

  3. Nakamura T, Miura K, Maruo Y et al. Aggressive angiomyxoma of the perineum originating from the rectal wall. J Gastroenterol 2002; 37: 303–308.

    Article  Google Scholar 

  4. Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum: report of nine cases of a distinctive type of gynecologic and soft-tissue neoplasm. Am J Surg Pathol 1983; 7: 463–475.

    Article  CAS  Google Scholar 

  5. Lepisto A, Heiskanen I, Bohling T, Raade M, Stefanovic V, Jarvinen H. Aggressive angiomyxoma—report of three cases. Int J Colorectal Dis 2007; 22(12): 1545–1546.

    Article  CAS  Google Scholar 

  6. Begin L, Clement P, Kirk M, Jothy S, McCaughey W, Ferenczy A. Aggressive angiomyxoma of soft parts: a clinicopathological study of nine cases. Hum Pathol 1985; 16: 621–628.

    Article  CAS  Google Scholar 

  7. Jeyadevan N, Sohaib, Thomas J, Jeyarajah A, Shepherd J, Fisher C. Imaging features of aggressive angiomyxoma. Clin Radiol 2002; 58: 157–162.

    Article  Google Scholar 

  8. Siassi R, Papadopoulos T, Matzel K. Metastasizing aggressive angiomyxoma. N Engl J Med 1999; 341(23): 1772.

    Article  CAS  Google Scholar 

  9. Blandamura S, Cruz J, Vergara L, Puerto I, Ninfo V. Aggressive angiomyxoma: a second case of metastasis with patient’s death. Hum Pathol 2003; 34(10): 1072–1074.

    Article  Google Scholar 

  10. Magtibay P, Salmon Z, Keeney G, Podratz K. Aggressive angiomyxoma of the female pelvis and perineum: a case series. Int J Gynecol Cancer 2006; 16: 396–401.

    Article  CAS  Google Scholar 

  11. Behranwala K, Thomas J. ‘Aggressive’ angiomyxoma: a distinct clinical entity. Eur J Surg Oncol 2003; 29: 559–563.

    Article  CAS  Google Scholar 

  12. Sakata K, Ishikawa S, Tokue A, Hirota N. Aggressive angiomyxoma of the scrotum. Urol Int 1997; 58(4): 247–249.

    Article  CAS  Google Scholar 

  13. Outwater E, Marchetto B, Wagner B, Siegelman E. Aggressive angiomyxoma: findings on CT and MR imaging. AJR Am J Roentgenol 1999; 172: 435–438.

    Article  CAS  Google Scholar 

  14. Chien A, Freeby J, Win T, Gadwood K. Aggressive angiomyxoma of the female pelvis; sonographic, CT and MR findings. AJR Am J Roentgenol 1998; 171: 530–531.

    Article  CAS  Google Scholar 

  15. Sinha R, Verma R. Case 106: aggressive angiomyxoma. Radiology 2007; 242(2): 625–627.

    Article  Google Scholar 

  16. Wiser A, Korach J, Gotlieb W, Fridman E, Apter S, Ben-Baruch G. Importance of accurate preoperative diagnosis in the management of aggressive angiomyxoma: report of three cases and review of the literature. Abdom Imaging 2006; 31: 383–386.

    Article  CAS  Google Scholar 

  17. Suleiman M, Duc C, Ritz S, Bieri S. Pelvic excision of large aggressive angiomyxoma in a woman: irradiation for recurrent disease. Int J Gynecol Cancer 2006; 16(Suppl 1): 356–360.

    Article  Google Scholar 

  18. Fetsch J, Laskin W, Lefkowitz M, Kindblom L-G, Meis-Kindblom J. Aggressive angiomyxoma. A clinicopathological study of 29 female patients. Cancer 1996; 78(1): 79–90.

    Article  CAS  Google Scholar 

  19. Granter SR, Nucci MR, Fletcher CD. Aggressive angiomyxoma: reappraisal of its relationship to angiomyofibroblastoma in a series of 16 cases. Histopathology 1997; 30(1): 3–10.

    Article  CAS  Google Scholar 

  20. Fukunaga M, Nomura K, Matsumoto K, Doi K, Endo Y, Ushigome S. Vulval angiomyofibroblastoma. Clinicopathologic analysis of six cases. Am J Clin Pathol 1997; 107: 45–51.

    Article  CAS  Google Scholar 

  21. 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; 15(1): 140–145.

    Article  CAS  Google Scholar 

  22. Idrees MT, Hoch BL, Wang BY, Unger PD. Aggressive angiomyxoma of male genital region. Report of 4 cases with immunohistochemical evaluation including hormone receptor status. Ann Diagn Pathol 2006; 10(4): 197–204.

    Article  Google Scholar 

  23. McCluggage W, Patterson A, Maxwell P. Aggressive angiomyxoma of pelvic parts exhibits oestrogen and progesterone receptor positivity. J Clin Pathol 2000; 53: 603–605.

    Article  CAS  Google Scholar 

  24. Shinohara N, Nonmura K, Ishikawa S, Seki H, Koyanagi T. Medical management of recurrent aggressive angiomyxoma with gonadotropin-releasing hormone agonist. Int J Urol 2004; 11: 432–435.

    Article  Google Scholar 

  25. Alameda F, Munne A, Baro T, et al. Vulvar angiomyxoma, aggressive angiomyxoma, and angiomyofibroblastoma: an immunohistochemical and ultrastructural study. Ultrastruct Pathol 2006; 30(3): 193–205.

    Article  Google Scholar 

  26. Kempson RL, Fletcher CD. Tumors of the soft tissues. In: Atlas of tumor pathology, 3rd edn, fascicle 30. Armed Forces Institute of Pathology, 2001.

Download references

Author information

Authors and Affiliations

  1. Department of Radiology, Vancouver General Hospital, Jim Patisson Pavilion South, 899 West 12th Avenue, Vancouver, BC, V5Z1M9, Canada

    E. J. Heffernan, F. O. Alkubaidan & P. L. Munk

  2. University of British Columbia, Vancouver, BC, Canada

    E. J. Heffernan, F. O. Alkubaidan & P. L. Munk

  3. Department of Pathology, BC Cancer Agency, Vancouver, BC, Canada

    M. M. Hayes

  4. Department of Surgery, Radiation Oncology and Developmental Radiotherapeutics, BC Cancer Agency, Vancouver, BC, Canada

    P. W. Clarkson

Authors
  1. E. J. Heffernan
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. M. M. Hayes
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. F. O. Alkubaidan
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. P. W. Clarkson
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. P. L. Munk
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to E. J. Heffernan.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Heffernan, E.J., Hayes, M.M., Alkubaidan, F.O. et al. Aggressive angiomyxoma of the thigh. Skeletal Radiol 37, 673–678 (2008). https://doi.org/10.1007/s00256-008-0465-0

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00256-008-0465-0

Keywords

Search

Navigation