History

Lymphangiosarcoma typically develops in patients many years after mastectomy, more commonly 5-15 years after surgery. In their original series, Stewart and Treves reported that the earliest angiosarcoma appeared 6 years after radical mastectomy, the longest interval being 24 years (average, 12.5 y).

In 1988, Tomita and associates demonstrated that the most common interval between radical mastectomy and the onset of lymphangiosarcoma was 5-14 years.^[26]In 1981, Yap et al reported a series of 22 patients in whom the median interval from mastectomy to the onset of angiosarcoma was 11 years (range, 5-16 y).^[27]

Clinical data from Sweden suggests 2 distinct groups: women who were treated for breast cancer with radical mastectomy and radiotherapy developed angiosarcomas in edematous arms (Stewart-Treves syndrome) after a median of 11 years, and those who were treated by segmental resection, anti-hormonal treatment and radiotherapy developed angiosarcomas in the irradiated field on the thoracic wall after a median of 7.3 years.^[23]

It has also been described developing 15 years after a total hysterectomy and radiation therapy for cervical cancer.^[28]Chronic lymphedema from other causes may also be associated with Stewart-Treves syndrome.^{[29, 30]}It usually appears in atrophic, pachydermatous, hyperkeratotic skin in longstanding lymphedema.^[24]

Physical Examination

As Stewart and Treves described, edema occurs first. Severe chronic edema of an upper extremity usually occurs in patients with Stewart-Treves syndrome. In patients who undergo radical mastectomy, edema first appears on the arm on the side operated on.^[31]

The occurrence of edema is not associated with complications such as postoperative infection or thrombosis. The edematous area gradually extends from arm to forearm and dorsal aspect of the hand and fingers. Initially, pain is absent, although skin distention may produce local discomfort. In the areas of long-standing chronic edema, recurrent erysipelas may occur, just as they do in patients with congenital lymphedema.

The skin in patients with Stewart-Treves syndrome tends to become atrophic and eventually pachydermatous, with prominent wrinkle lines. At times, hyperkeratoses and telangiectasias can be observed.

After an interval of 1-30 years, a purplish patch appears that then develops into a plaque or nodule in the area of chronic lymphedema. Other initial lesions of Stewart-Treves syndrome may include a palpable subcutaneous mass or a poorly healing eschar with recurrent bleeding and oozing.

The lesions of Stewart-Treves syndrome typically appear as multiple reddish blue macules or nodules that may become polypoid. Around these nodules, small satellite areas can develop and become confluent, forming an enlarging lesion. Sometimes, a bullous component may be seen. Thus, the morphology may be heterogeneous, with hematomalike lesions, multiple bluish-reddish nodules, and asymptomatic nodules.^[23]

As the angiosarcoma continues to grow and expand, the overlying atrophic epidermis may ulcerate, producing recurrent episodes of bleeding and infection.^[32]Necrosis may be evident in advanced cutaneous tumors.

Ultimately, extensive cutaneous nodules and systemic metastases appear. These nodules most commonly occur in the lungs and cause the patient's death. Not every tumor in an area of lymphedema is an angiosarcoma.

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.

Differential Diagnoses

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Media Gallery

Classic example of angiosarcoma associated with chronic lymphedema after lymphadenectomy and radiation therapy.

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Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Coauthor(s)

Geover Fernandez, MD, FAAD Staff Physician, Department of Dermatology, Rutgers New Jersey Medical School

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

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

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

Disclosure: Nothing to disclose.

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

Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, Noah Worcester Dermatological Society, Pennsylvania Academy of Dermatology, American Academy of Dermatology, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

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

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

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, Women's Dermatologic Society, National Psoriasis Foundation, American Medical Association, Phi Beta Kappa, Sigma Xi, The Scientific Research Honor Society

Disclosure: Received honoraria from Amgen for consulting; Received honoraria from Abbott for consulting; Partner received salary from Merck for management position; Received honoraria from Abbott for speaking and teaching; Received honoraria from Amgen for review panel membership; Received honoraria from Centocor for consulting; Received honoraria from Leo for consulting; Received none from Merck for other.

Stewart-Treves Syndrome Clinical Presentation

History

Physical Examination

Complications

References

Tables

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