Stewart-Treves syndrome: Pathogenesis and management

Sharma, Amit; Schwartz, Robert A.

doi:10.1016/j.jaad.2012.04.028

Cited by 142 publications

(204 citation statements)

References 45 publications

(47 reference statements)

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“…In rare cases, chronic lymphedema is associated with increased incidence of malignancies such as lymphangiosarcoma (Stewart-Treves syndrome), Kaposi sarcoma, and lymphoma. 23,24 Surgical procedures to treat lymphedema have existed for over a century. The Charles procedure was first reported in 1912.…”

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confidence: 99%

Review of Current Surgical Treatments for Lymphedema

et al. 2014

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Background. The current mainstay of lymphedema therapy has been conservative nonsurgical treatment. However, surgical options for lymphedema have been reported for over a century. Early surgical procedures were often invasive and disfiguring, and they often had only limited long-term success. In contrast, contemporary surgical techniques are much less invasive and have been shown to be effective in reducing excess limb volume, the risk of cellulitis, and the need for compression garment use and lymphedema therapy. Microsurgical procedures such as lymphaticovenous anastomosis and vascularized lymph node transfer lymphaticolymphatic bypass can treat the excess fluid component of lymphedema swelling that presents as pitting edema. Suction-assisted protein lipectomy is a minimally invasive procedure that addresses the solid component of lymphedema swelling that typically occurs later in the disease process and presents as chronic nonpitting lymphedema. These surgical techniques are becoming increasingly popular and their success continues to be documented in the medical literature. We review the efficacy and limitations of these contemporary surgical procedures for lymphedema. Methods. A Medline literature review was performed of lymphedema surgery, vascularized lymph node transfer, lymphaticovenous anastomosis, lymphatic liposuction, and lymphaticolymphatic bypass with particular emphasis on developments within the past 10 years. A literature review of technique, indications, and outcomes of the surgical treatments for lymphedema was undertaken.Results. Surgical treatments have evolved to become less invasive and more effective. Conclusions. With proper diagnosis and the appropriate selection of procedure, surgical techniques can be used to treat lymphedema safely and effectively in many patients when combined with integrated lymphedema therapy.Lymphedema is a progressive and debilitating condition associated with dysfunction of the lymphatic system. While a small percentage of cases are congenital, most patients in developed countries present with lymphedema resulting from treatment of malignancy. The true incidence of lymphedema is difficult to determine as a result of significant differences in diagnostic criteria. However, lymphedema is reported to occur in up to 49 % of breast, 20 % of gynecologic, 16 % of melanoma, 10 % of genitourinary, and 6 % head and neck cancer patients after lymph node dissection and/or radiotherapy. Even among patients who undergo isolated axillary sentinel lymph node biopsy, up to 7 % have measurable arm differences, and up to 10 % have subjective symptoms of lymphedema.

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confidence: 99%

Review of Current Surgical Treatments for Lymphedema

et al. 2014

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“…Lymphedema associated to STS can be congenital, idiopathic, traumatic, filarial, postsurgical and obesity-related (5-7). To the best of our knowledge, only a few cases of CAS have occurred in lower limbs lymphedema (8) as in the case here reported.…”

Section: Introductionmentioning

confidence: 53%

“…The lymphedema-associated angiosarcoma (StewartTreves-Syndrome) is extre mely rare (0,45% of lymphoedemata), and develops late up to 27 years after lymphoedema-onset (9, 10). The most of patients affected by STS are women (peak age 65-70 years) with a history of radical mastectomy due to breast cancer (8). STS of the lower limbs is rare and the duration of the lymphoedema before the development of CAS is about 5 to 15 years (7,8).…”

Section: Discussionmentioning

confidence: 99%

Stewart-Treves syndrome: cutaneous angiosarcoma developing in chronic lymphedema

Savoia¹

2015

cderm

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SummaryCutaneous Angiosarcoma (CAS) is an uncommon, highly malignant, endotelial-derived tumor with a poor prognosis that may develop on chronic lymphedema as in Stewart-Treves Syndrome (STS). We report the case of a 82-year-old female affected by chronic lymphedema that referred to us because of a 2-month history of purpura of the lower limbs, followed by the onset of red-violaceus ulcerated nodules, finally measuring about 13 x 7 centimeters. Histological features and immunohistochemistry confirmed the diagnosis of STS. Considering the size of the tumor, age, comorbidities and general condition of the patient, wide surgery was not performed.Chemotherapy with liposomal doxorubicin and local radiotherapy were started; however, after 5 months, computer tomography revealed diffuse metastases involving inguinal and pelvic lymph nodes, liver and lungs. The patient died shortly after. Our case highlights the poor prognosis of STS, the difficult management in old patients and confirm that chronic lymphedema is an "immunocompromised district" and a predisposing factor for the development of cutaneous neoplasias.

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“…Chemotherapy and radiation therapy have not improved survivorship significantly (16). Nevertheless, even aggressive therapy has resulted in a mean survival rate of 24 months and a 5-year survival rate of only 10%.…”

Section: Discussionmentioning

confidence: 99%

Steward-Treves syndrome in a male patient

Marina

Lozanova

Broshtilova

et al. 2014

SSM

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Lymphangiosarcoma is an uncommon vascular tumor that usually develops in chronic lymphedema. The etiology of lymphangiosarcoma remains unknown. It develops mainly after mastectomy, in association with chronic lymphedema (Stewart-Treves syndrome), or after radiotherapy. The early diagnosis of this aggressive tumor is important, as it has a high risk of local recurrence and metastasis. Stewart-Treves syndrome occurs in 0.5% of patients who survive mastectomy for more than 5 years. The mean age at appearance of the angiosarcoma is 62 years, and the mean interval between mastectomy and the appearance of the tumour is 10.5 years. Only two cases have been reported in men following mastectomy. We present a 70-year old male patient with lymphangiosarcoma which developed three and a half years after the diagnosis of breast carcinoma. The patient underwent mastectomy of the right mammary gland with ipsilateral axillary lymph node dissection and had postoperative chemo-and radiotherapy.

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Stewart-Treves syndrome: Pathogenesis and management

Cited by 142 publications

References 45 publications

Review of Current Surgical Treatments for Lymphedema

Review of Current Surgical Treatments for Lymphedema

Stewart-Treves syndrome: cutaneous angiosarcoma developing in chronic lymphedema

Steward-Treves syndrome in a male patient

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