Stewart-Treves Syndrome Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD   more...
 
Updated: Mar 22, 2012
 

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.[16] 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).[17]

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.[18]

It has also been described developing 15 years after a total hysterectomy and radiation therapy for cervical cancer.[19] Chronic lymphedema from other causes may also be associated with Stewart-Treves syndrome.[20]

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Physical

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.[21]

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.[18]

As the angiosarcoma continues to grow and expand, the overlying atrophic epidermis may ulcerate, producing recurrent episodes of bleeding and infection. 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.

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Causes

The most important single causative agent in Stewart-Treves syndrome is prolonged chronic lymphedema. Although Stewart-Treves syndrome develops after radical mastectomy in most patients, lymphangiosarcoma also develops in other forms of acquired lymphedema and in congenital lymphedema. Causes for such secondary lymphedema may include trauma, surgical invasion of the groin for the treatment of penile or cervical cancer, filariasis, idiopathic acquired lymphedema, vascular stasis, and morbid obesity.

Edema secondary to cardiac or renal disease is not associated with this malignancy. Thus, edema alone is not sufficient to cause lymphangiosarcoma. Perhaps additional factors such as a genetic predisposition are required.

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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, Paul L Foster School of Medicine

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, 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 and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

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