Stewart-Treves Syndrome Follow-up

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD   more...
 
Updated: Apr 25, 2011
 

Further Inpatient Care

Complications from metastatic disease, such as pleural effusions, may require hospitalization of the patient. A CT scan may identify bilateral pulmonary involvment. Stewart-Treves syndrome patients may need further inpatient care for pain control.

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Deterrence/Prevention

The most efficient way to avoid Stewart-Treves syndrome, a highly lethal disease, is by preventing or treating long-standing lymphedema, which predisposes individuals to this lymphangiosarcoma years later.

Other complications commonly associated with chronic lymphedema, such as erysipelas and deep venous thromboses, must be prevented by regularly examining these areas and then treating them.

Early biopsy of any suggestive lesion should be performed because early diagnosis and surgical treatment offers the highest rate of long-term survival.

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Complications

Patients can present with recurrent episodes of erysipelas and deep venous thromboses in areas of chronic lymphedema. Other complications include recurrent infections and malignancies.

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Prognosis

The prognosis is dismal. The 5-year survival rate reported by Sordillo et al in 1981 was 13.6%,[13] In 1987, Hultberg found that patients with Stewart-Treves syndrome had a mean survival of 20 months after tumor onset.[32] Untreated patients have an average survival of 5-8 months. A more recent analysis showed the overall 5-year survival was 16%.[17]

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Patient Education

Patients should be informed about the significance of prolonged chronic lymphedema and about how to reduce and control it. Patients should be encouraged to seek early medical attention if they notice unexplained skin changes or unresolved lymphedema. Patients should be educated about complications, such as recurrent infections, deep venous thrombosis, and malignancies, that can occur with lymphedema.

For excellent patient education resources, visit eMedicine's Women's Health Center and Cancer and Tumors Center. Also, see eMedicine's patient education articles Mastectomy and Breast Cancer.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Geover Fernandez, MD, FAAD  Staff Physician, Department of Dermatology, University of Medicine and Dentistry New Jersey, New Jersey Medical School

Geover Fernandez, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and American Society for MOHS Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Abby S Van Voorhees, MD  Assistant Professor, Director of Psoriasis Services and Phototherapy Units, Department of Dermatology, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania

Abby S Van Voorhees, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, National Psoriasis Foundation, Phi Beta Kappa, Sigma Xi, and Women's Dermatologic Society

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Merck Salary Management position; Abbott Honoraria Speaking and teaching; Amgen Honoraria Review panel membership; Centocor Honoraria Consulting; Leo Consulting; Merck None Other

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Christen M Mowad, MD  Associate Professor, Department of Dermatology, Geisinger Medical Center

Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Glen H Crawford, MD  Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital

Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

References

  1. Gonne E, Collignon J, Kurth W, et al. [Angiosarcoma consecutive to chronic lymphoedema: a Stewart-Treves syndrome]. Rev Med Liege. Jul-Aug 2009;64(7-8):409-13. [Medline].

  2. Dürr HR, Pellengahr C, Nerlich A, Baur A, Maier M, Jansson V. Stewart-Treves syndrome as a rare complication of a hereditary lymphedema. Vasa. Feb 2004;33(1):42-5. [Medline].

  3. Lowenstein S. Der atiologische zusammenhang zwischen akutem ein-maligem trauma und sarkon. Beitr Z klin Clir. 1906;48:780.

  4. Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema: A report of six cases in elephantiasis chirurgica. Cancer. 1948;1:64-81.

  5. Hallel-Halevy D, Yerushalmi J, Grunwald MH, Avinoach I, Halevy S. Stewart-Treves syndrome in a patient with elephantiasis. J Am Acad Dermatol. Aug 1999;41(2 Pt 2):349-50. [Medline].

  6. Offori TW, Platt CC, Stephens M, Hopkinson GB. Angiosarcoma in congenital hereditary lymphoedema (Milroy's disease)--diagnostic beacons and a review of the literature. Clin Exp Dermatol. Mar 1993;18(2):174-7. [Medline].

  7. Schreiber H, Barry FM, Russell WC, Macon WL 4th, Ponsky JL, Pories WJ. Stewart-Treves syndrome. A lethal complication of postmastectomy lymphedema and regional immune deficiency. Arch Surg. Jan 1979;114(1):82-5. [Medline].

  8. Stark RB, Dwyer EM, De Forest M. Effect of surgical ablation of regional lymph nodes on survival of homografts. Ann NY Acad Sci. 1960;87:140-148.

  9. Sternby NH, Gynning I, Hogeman KE. Postmastectomy angiosarcoma. Acta Chir Scand. 1961;121:420-432.

  10. Styring E, Fernebro J, Jonsson PE, et al. Changing clinical presentation of angiosarcomas after breast cancer: from late tumors in edematous arms to earlier tumors on the thoracic wall. Breast Cancer Res Treat. Jan 20 2010;[Medline].

  11. Schirger A. Postoperative lymphedema: etiologic and diagnostic factors. Med Clin North Am. Jul 1962;46:1045-50. [Medline].

  12. Wierzbicka-Hainaut E, Guillet G. [Stewart-Treves syndrome (angiosarcoma on lyphoedema): A rare complication of lymphoedema]. Presse Med. Dec 2010;39(12):1305-8. [Medline].

  13. Sordillo PP, Chapman R, Hajdu SI, Magill GB, Golbey RB. Lymphangiosarcoma. Cancer. Oct 1 1981;48(7):1674-9. [Medline].

  14. Woodward AH, Ivins JC, Soule EH. Lymphangiosarcoma arising in chronic lymphedematous extremities. Cancer. Aug 1972;30(2):562-72. [Medline].

  15. Tomita K, Yokogawa A, Oda Y, Terahata S. Lymphangiosarcoma in postmastectomy lymphedema (Stewart-Treves syndrome): ultrastructural and immunohistologic characteristics. J Surg Oncol. Aug 1988;38(4):275-82. [Medline].

  16. Yap BS, Yap HY, McBride CM, Bodey GP. Chemotherapy for postmastectomy lymphangiosarcoma. Cancer. Mar 1 1981;47(5):853-6. [Medline].

  17. Styring E, Fernebro J, Jönsson PE, Ehinger A, Engellau J, Rissler P, et al. Changing clinical presentation of angiosarcomas after breast cancer: from late tumors in edematous arms to earlier tumors on the thoracic wall. Breast Cancer Res Treat. Aug 2010;122(3):883-7. [Medline].

  18. Bassotti A, Driban N, Huczak L, Zaccarria S. Angiosarcoma en linfedema cronico posmastectomia (Sindrome de Stewart-Treves). Med Rev. 2008;4:1-20.

  19. Schwartz RA, Kardashian JF, McNutt NS, Crain WR, Welch KL, Choy SH. Cutaneous angiosarcoma resembling anaplastic Kaposi's sarcoma in a homosexual man. Cancer. Feb 15 1983;51(4):721-6. [Medline].

  20. Salameire D, Templier I, Charles J, et al. An "anaplastic" Kaposi's sarcoma mimicking a Stewart-Treves syndrome. A case report and a review of literature. Am J Dermatopathol. Jun 2008;30(3):265-8. [Medline].

  21. Kunkel T, Mylonas I, Mayr D, Friese K, Sommer HL. Recurrence of secondary angiosarcoma in a patient with post-radiated breast for breast cancer. Arch Gynecol Obstet. Nov 2008;278(5):497-501. [Medline].

  22. Nakazono T, Kudo S, Matsuo Y, et al. Angiosarcoma associated with chronic lymphedema (Stewart-Treves syndrome) of the leg: MR imaging. Skeletal Radiol. Jul 2000;29(7):413-6. [Medline].

  23. Schindera ST, Streit M, Kaelin U, Stauffer E, Steinbach L, Anderson SE. Stewart-Treves syndrome: MR imaging of a postmastectomy upper-limb chronic lymphedema with angiosarcoma. Skeletal Radiol. Mar 2005;34(3):156-60. [Medline].

  24. McConnell AH, Haslam P. Angiosarcoma in post-mastectomy lymphedema: report of five cases and review of the literature. Br J Surg. 1959;46:322-32.

  25. Grobmyer SR, Daly JM, Glotzbach RE, Grobmyer AJ 3rd. Role of surgery in the management of postmastectomy extremity angiosarcoma (Stewart-Treves syndrome). J Surg Oncol. Mar 2000;73(3):182-8. [Medline].

  26. Breidenbach M, Rein D, Schmidt T, et al. Intra-arterial mitoxantrone and paclitaxel in a patient with Stewart-Treves syndrome: selection of chemotherapy by an ex vivo ATP-based chemosensitivity assay. Anticancer Drugs. Apr 2000;11(4):269-73. [Medline].

  27. Goetze S, Schmook T, Audring H, Ziegenbein C, Worm M, Schulze P. [Successful treatment of Stewart-Treves syndrome with liposomal doxorubicin]. J Dtsch Dermatol Ges. Jan 2004;2(1):49-52. [Medline].

  28. Paydas S, Yapar Z, Gonlusen G. Stewart-Treves syndrome: questionable response to weekly paclitaxel. Chemotherapy. 2010;56(1):66-8. [Medline].

  29. Fujisawa Y, Ito M, Mori K, Okada S, Nakamura Y, Kawachi Y, et al. Intra-arterial mitoxantrone/paclitaxel in angiosarcoma of the lower limb associated with chronic lymphedema (Stewart-Treves syndrome) in a patient with cervical cancer. Eur J Dermatol. Jan 14 2011;[Medline].

  30. Furue M, Yamada N, Takahashi T, et al. Immunotherapy for Stewart-Treves syndrome. Usefulness of intrapleural administration of tumor-infiltrating lymphocytes against massive pleural effusion caused by metastatic angiosarcoma. J Am Acad Dermatol. May 1994;30(5 Pt 2):899-903. [Medline].

  31. Roy P, Clark MA, Thomas JM. Stewart-Treves syndrome--treatment and outcome in six patients from a single centre. Eur J Surg Oncol. Nov 2004;30(9):982-6. [Medline].

  32. Hultberg BM. Angiosarcomas in chronically lymphedematous extremities. Two cases of Stewart-Treves syndrome. Am J Dermatopathol. Oct 1987;9(5):406-12. [Medline].

  33. Schwartz RA. Kaposi's sarcoma. In: Schwartz RA, ed. Skin Cancer: Recognition and Management. 1988:80-8.

 
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