Stewart-Treves Syndrome of the Lower Extremity

Veiga, Rossana Ruth Garcia da; Nascimento, Bianca Angelina Macêdo do; Carvalho, Alessandra Haber; Brito, Arival Cardoso de; Bittencourt, Maraya de Jesus Semblano

doi:10.1590/abd1806-4841.20153926

Cited by 12 publications

(11 citation statements)

References 5 publications

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“…Cases with severe cutaneous involvement can exhibit chronic ulceration and angiosarcoma (Stewart–Treves syndrome). 52 In addition, 35% of KLA patients have subcutaneous masses that are soft, are not tender or painful, and do not grow. 8 These patients undergo resection, but care should be taken regarding the possibility of local lymphorrhea in the resected area.…”

Section: Discussion Of Findings and Relevant Literaturementioning

confidence: 99%

Generalized Lymphatic Anomaly and Gorham–Stout Disease: Overview and Recent Insights

Ozeki

Fukao

2019

Advances in Wound Care

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Significance: Generalized lymphatic anomaly and Gorham–Stout disease are extremely rare diseases with severe symptoms and poor prognosis. The etiology and clinical presentation of the patients remain poorly defined, but recent research has attempted to determine the pathogenesis of these diseases. Recent Advances: In recent years, the characteristics of complex lymphatic anomalies have been revealed. Kaposiform lymphangiomatosis is recognized as a new entity that has an aggressive course and poor prognosis. Genetic analysis revealed somatic mutations in genes associated with the phosphoinositide 3-kinase (PI3K) pathway in lymphatic malformation lesions. Somatic NRAS mutation in lymphatic endothelium from a generalized lymphatic anomaly patient was also detected as a potential cause of disease. Furthermore, studies demonstrated the efficacy of the mammalian target of rapamycin (mTOR) inhibitor sirolimus for these lymphatic diseases. Critical Issues: These diseases have overlapping symptoms, imaging features, and complications, leading to difficulty in their differential diagnosis. In addition, there are no standard therapies. Therefore, we need to determine the differences among these diseases to not only diagnose but also treat them appropriately. Future Directions: Further investigations should reveal differences in the clinical features and findings of radiological, pathological, and genetic examinations to manage each disease appropriately. Somatic mutation in genes encoding RAS/PI3K/mTOR signaling pathway components could be associated with the pathogenesis of these diseases and may be novel targets for drug therapies.

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Section: Discussion Of Findings and Relevant Literaturementioning

confidence: 99%

Generalized Lymphatic Anomaly and Gorham–Stout Disease: Overview and Recent Insights

Ozeki

Fukao

2019

Advances in Wound Care

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“…While the incidence of Stewart-Treves syndrome is not well known, it has been reported to be about 1/10 to 1/20 of all CAS ( 19 – 22 ). Similarly, the cumulative incidence of radiation-associated CAS 15 years after radiotherapy for breast carcinoma was reported to be 0.9 per 1,000 patients ( 23 ), meaning less than 1 occurrence per 10,000 irradiated patients per year.…”

Section: Clinical Presentationmentioning

confidence: 99%

Cutaneous Angiosarcoma: The Possibility of New Treatment Options Especially for Patients with Large Primary Tumor

et al. 2018

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The most widely accepted treatment for cutaneous angiosarcoma (CAS) is wide local excision and postoperative radiation to decrease the risk of recurrence. Positive surgical margins and large tumors (T2, >5 cm) are known to be associated with poor prognosis. Moreover, T2 tumors are known to be associated with positive surgical margins. According to previous reports, the majority of CAS patients in Japan had T2 tumors, whereas less than half of the patients in the studies from western countries did so. Consequently, the reported 5-year overall survival of Japanese CAS patients without distant metastasis was only 9%, lower than that for stage-IV melanoma. For patients with T2 tumors, management of subclinical metastasis should be considered when planning the initial treatment. Several attempts to control subclinical metastasis have been reported, such as using adjuvant/neoadjuvant chemotherapy in addition to conventional surgery plus radiation. Unfortunately, those attempts did not show any clinical benefit. Besides surgery, new chemotherapeutic approaches for advanced CAS have been introduced in the past couple of decades, such as paclitaxel and docetaxel. We proposed the use of chemoradiotherapy (CRT) using taxanes instead of surgery plus radiation for patients with T2 tumors without distant metastasis and showed a high response ratio with prolonged survival. However, this prolonged survival was seen only in patients who received maintenance chemotherapy after CRT, indicating that continuous chemotherapy is mandatory to control subclinical residual tumors. With the recent development of targeted drugs for cancer, many potential drugs for CAS are now available. Given that CAS expresses a high level of vascular endothelial growth factor (VEGF) receptor, drugs that target VEGF signaling pathways such as anti-VEGF monoclonal antibody and tyrosine kinase inhibitors are also promising, and several successful treatments have been reported. Besides targeted drugs, several new cytotoxic anticancer drugs such as eribulin or trabectedin have also been shown to be effective for advanced sarcoma. However, most of the clinical trials did not include a sufficient number of CAS patients. Therefore, clinical trials focusing only on CAS should be performed to evaluate the effectiveness of these new drugs.

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“…Classically, STS presents after radical mastectomy in the upper extremities but can present in other areas like the lower extremities about 10% of the time [4]. The lesions are small and typically purple at onset and then develop into a nodule.…”

Section: Discussionmentioning

confidence: 99%

Stewart-Treves Syndrome in Obesity-Associated Chronic Lymphedema: A Case Report

Chaney

Piao

Tahir³

et al. 2021

J Med Cases

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Stewart-Treves syndrome (STS) is defined as the development of cutaneous angiosarcoma in the presence of long-standing lymphedema and is a rare disease with only about 400 cases reported in world literature. We report a case of a 63-year-old morbidly obese woman with a long-standing history of lymphedema who developed angiosarcoma of the right lower extremity with metastasis and presented with acute respiratory distress. The patient underwent a thorough laboratory workup with a chest X-ray showing bilateral effusions. The hematology-oncology service was consulted and found the patient to have significant progression of angiosarcoma causing respiratory failure and cardiac instability. A decision to transition to hospice care was made and the patient eventually passed away in the intensive care unit. We present this case to raise awareness of STS in medical literature to understand its clinical manifestations better. Early detection is imperative as angiosarcoma is commonly an aggressive disease.

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Stewart-Treves Syndrome of the Lower Extremity

Cited by 12 publications

References 5 publications

Generalized Lymphatic Anomaly and Gorham–Stout Disease: Overview and Recent Insights

Generalized Lymphatic Anomaly and Gorham–Stout Disease: Overview and Recent Insights

Cutaneous Angiosarcoma: The Possibility of New Treatment Options Especially for Patients with Large Primary Tumor

Stewart-Treves Syndrome in Obesity-Associated Chronic Lymphedema: A Case Report

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