eMedicine Specialties > Dermatology > Malignant Neoplasms

Stewart-Treves Syndrome: Treatment & Medication

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Geover Fernandez, MD, FAAD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry New Jersey, New Jersey Medical School
Contributor Information and Disclosures

Updated: Nov 13, 2009

Treatment

Medical Care

  • Chemotherapy and irradiation continue to be evaluated as adjuvants to surgery for the treatment of Stewart-Treves syndrome.
    • Currently, these treatment options offer little benefit.
    • In 2000, Grobmyer and associates found no statistical significant difference in the survival rates of patients treated with chemotherapy compared with those treated with irradiation.21
    • Although long-term survivors after either radiation therapy or systemic chemotherapy have been reported,22,23 the overall results have been discouraging.
    • As a result of these findings, these treatment options are reserved for patients with inoperable, advanced disease or those who refuse surgery.
  • In 1994, Furue et al demonstrated that immunotherapy may be beneficial as palliative treatment for pleural effusions caused by metastatic angiosarcoma.24

Surgical Care

  • Early amputation or wide local excision provides the best chance of long-term survival in patients with Stewart-Treves syndrome. Some authorities favor radical ablative surgery with an early diagnosis, in order to confer a reasonable prognosis with this rare but aggressive disease.25 A nihilistic approach is undesirable.
    • The most common approach in patients with lymphangiosarcoma is amputation of the limb or forequarter rather than wide local surgical excision.
    • Even in cases with early surgical treatment, the prognosis is disappointing, with a high rate of local recurrence and metastasis.
    • Metastatic disease should exclude surgical treatment unless surgery is useful for symptomatic improvement.
  • Chemotherapy, immunotherapy, and/or radiation therapy can be used as adjuvants to surgery (see Medical Care).

More on Stewart-Treves Syndrome

Overview: Stewart-Treves Syndrome
Differential Diagnoses & Workup: Stewart-Treves Syndrome
Treatment & Medication: Stewart-Treves Syndrome
Follow-up: Stewart-Treves Syndrome
References
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References

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  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].

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  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].

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

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

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

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

  13. 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].

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

  15. 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].

  16. 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].

  17. 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].

  18. 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].

  19. 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].

  20. 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.

  21. 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].

  22. 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].

  23. 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].

  24. 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].

  25. 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].

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

  27. Caldwell JB, Ryan MT, Benson PM, James WD. Cutaneous angiosarcoma arising in the radiation site of a congenital hemangioma. J Am Acad Dermatol. Nov 1995;33(5 Pt 2):865-70. [Medline].

  28. Cozen W, Bernstein L, Wang F, Press MF, Mack TM. The risk of angiosarcoma following primary breast cancer. Br J Cancer. Oct 1999;81(3):532-6. [Medline].

  29. Heitmann C, Ingianni G. Stewart-Treves syndrome: lymphangiosarcoma following mastectomy. Ann Plast Surg. Jan 2000;44(1):72-5. [Medline].

  30. Krause KI, Hebert AA, Sanchez RL, Solomon AR Jr. Anterior abdominal wall angiosarcoma in a morbidly obese woman. J Am Acad Dermatol. Aug 1986;15(2 Pt 2):327-30. [Medline].

  31. Laguerre B, Lefeuvre C, Kerbrat P, Hassel M. [Stewart-Treves syndrome arising in post-traumatic lymphedema]. Bull Cancer. Mar 1999;86(3):279-82. [Medline].

  32. Muller R, Hajdu SI, Brennan MF. Lymphangiosarcoma associated with chronic filarial lymphedema. Cancer. Jan 1 1987;59(1):179-83. [Medline].

  33. Ruocco V, Schwartz RA, Ruocco E. Lymphedema: an immunologically vulnerable site for development of neoplasms. J Am Acad Dermatol. Jul 2002;47(1):124-7. [Medline].

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

  35. Sinclair SA, Sviland L, Natarajan S. Angiosarcoma arising in a chronically lymphoedematous leg. Br J Dermatol. Apr 1998;138(4):692-4. [Medline].

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Further Reading

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Keywords

Stewart-Treves syndrome, cutaneous angiosarcoma, postmastectomy angiosarcoma, lymphangiosarcoma in postmastectomy lymphedema, postlymphedema angiosarcoma, hemangiosarcoma in chronic lymphedema, hemangiosarcoma in postmastectomy lymphedema, postmastectomy lymphedema, lymphangiosarcoma, Milroy disease, Milroy's disease, idiopathic lymphedema, congenital lymphedema, traumatic lymphedema, filarial lymphedema

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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, UMDNJ-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.

Medical Editor

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; Genentech Honoraria Consulting; Incyte Grant/research funds Other; Warner Chilcott Honoraria Consulting; Merck Salary Management position; Abbott  Speaking and teaching

Pharmacy Editor

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.

Managing Editor

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.

CME Editor

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

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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