OverviewCutaneous Angiosarcoma Secondary to Lymphoedema or Radiation Therapy — A Systematic Review
Introduction
Angiosarcoma is a rare but fatal tumour, comprising less than 1% of all sarcomas. It arises from the vascular endothelium, with aggressive behaviour and tends to infiltrate locally and metastasise. It is, however, difficult to diagnose on initial presentation and, hence, carries a poor prognosis. These tumours can occur anywhere in the body, but usually take preference on the skin or deep tissues.
In general, angiosarcomas of this region can be categorised into two groups – primary and secondary. Primary angiosarcomas occur spontaneously in younger patients without any known risk factors. Secondary angiosarcomas are known to develop in elder individuals who have previously received surgical treatment for primary breast cancer with adjuvant radiotherapy. About 1000 in 100,000 breast cancer survivors suffer from the development of angiosarcoma 10–15 years after primary breast cancer therapy including surgery and radiotherapy [1], [2]. These tumours can occur at the primary site of the breast or develop on the ipsilateral upper limb (Stewart–Treves syndrome), which is often plagued by lymphoedema [3].
Lymphoedema is a recognised risk factor for the development of angiosarcoma. This condition, also known as Stewart–Treves syndrome, describes a cutaneous angiosarcoma arising from chronic lymphoedema after mastectomy and lymphadenectomy. Another known risk factor is breast irradiation. In fact, radiotherapy-induced fibrosis in itself can also cause chronic lymphatic obstruction. The latency period from radiotherapy to angiosarcomatous development is quoted to be on average 6–7 years in most studies.
Although uncommon, the incidence of secondary angiosarcomas is rising, due to the increased incidence of breast cancer and improved long-term breast cancer survival. As a result, long-term consequences of radiotherapy and lymphoedema were more commonly seen among breast cancer survivors.
This systematic review aims to evaluate the clinical features and outcomes of secondary angiosarcoma, commonly arising after breast cancer treatment.
Section snippets
Materials and Methods
The systematic review was conducted in accordance with the PRISMA statement and current methodological literature. As this was a systematic review/meta-analysis, institutional review board approval was not required (see Figure 1 for the PRISMA flowchart).
Results
In total, 148 studies or reports were identified using the preset key words defined in the study protocol. After excluding irrelevant papers (such as editorials or commentaries without case presentation, or conference abstracts), 39 studies or reports were included in the review [1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18], [19], [20], [21], [22], [23], [24], [25], [26], [27], [28], [29], [30], [31], [32], [33], [34], [35], [36], [37], [38],
Discussion
With increased breast cancer awareness globally, breast cancer patients are being diagnosed and successfully treated at early stages. These long-term breast cancer survivors are at risk of angiosarcoma secondary to the breast cancer treatment. Angiosarcoma is rare but is nearly always fatal unless diagnosed and treated early. Unfortunately, they are easily missed on presentation and difficult to diagnose, even with proper imaging. Thus, it is important to understand this disease and to have a
Conclusion
Angiosarcoma secondary to lymphoedema/radiotherapy remains a deadly disease with a long latent period (see Table 2 for a summary).
Conflict of interest
The authors declare no conflict of interest.
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2022, Journal of Plastic, Reconstructive and Aesthetic SurgeryCitation Excerpt :Although the prevention of lymphedema can be the best management and treatment for AS, a high index of suspicion is the major hope to detect it early. Importantly, lymphedema can be prevented, controlled, and managed by various means, without affecting breast cancer survivors’ quality of life.2 Lymph flow evaluation is warranted not only in the upper extremity but also in the breast lymphedema after breast cancer treatments to allow early diagnosis and appropriate therapeutic interventions to breast lymphedema.
Radiation-Induced Sarcoma: A Retrospective Population-Based Study Over 34 Years in a Single Institution
2021, Clinical OncologyCitation Excerpt :In our study, 83% of RIS patients were women. A similar finding has been reported in other studies [8–11] and explained by the frequency of radiotherapy for the management of breast cancer, a common disease among women that is frequently cured with standard therapeutic approaches [19]. Accordingly, breast cancer was the most frequent original cancer in our study as well as in literature data [5,10,11,16,17].