Angiosarcoma Of Breast Research Articles

Clinical Impact of Systemic Staging Studies in Secondary Breast Angiosarcoma

Our goal was to evaluate the clinical impact of local and systemic imaging in secondary breast angiosarcoma (SBAS) at diagnosis (DX) and in follow-up (FU). Singe-center retrospective review of SBAS treated from 1/2007-7/2020. Clinicopathologic data was collected, including local and systemic imaging at DX and FU. The clinical impact of imaging was evaluated. Twenty patients had SBAS diagnosed 10.6±6.7 years following their index BC. All were diagnosed on exam. Mammogram, ultrasound, and breast MRI were commonly performed concurrently with DX (2.4 studies per patient) but did not impact clinical management. In-breast imaging extent of disease did not correlate with pathologic extent of disease (p=0.49). Systemic staging uncommonly identified asymptomatic metastatic disease (5%). During FU, 90% of recurrences were identified by exam or new symptoms, not routine imaging. SBAS extent is not reliably estimated on targeted breast imaging. Asymptomatic metastatic disease is uncommon but targeted evaluation of new symptoms is warranted given high likelihood of identifying metastasis. Additional data is needed to define optimum imaging timing and modalities for SBAS.

Metastatic breast angiosarcoma: A case report

Introduction: Angiosarcoma of the breast is a rare finding that accounts for less than 1% of breast malignancies and less than 5% of all sarcomas. It is categorized according to the etiology: de novo (primary) and therapy related (secondary). Case Report: This case report discusses the management of a patient (a 62-year-old female) who had primary breast angiosarcoma. Management of such a patient always merits a multidisciplinary team approach that involves the breast surgeons, oncologists, breast radiologists, and breast care nurses. Conclusion: The main conclusion from this case report is that frequent breast screening would be beneficial in order to diagnose malignancy at an early stage, especially in patients who have a family history as survival depends on the stage at which the disease is diagnosed. However, this disease has a very poor prognosis overall.

Primary Angiosarcoma of Breast in a Young Female- A Case Report

Introduction: Primary angiosarcoma of the breast is exceedingly rare, and represents around 0.04% of malignant breast neoplasm. Radiographically, breast angiosarcomas exhibit no pathognomonic features. They often appear as ill-defined masses on mammograms. The diagnosis is essentially based on the histological assessment of the excised sample. The treatment is based on simple mastectomy. This neoplasm carries a very poor prognosis, with a five- year survival of 8–50%. Case Report: We report a case of a 20- year-old woman with a highly vascular mass in her left breast which is suggestive of malignancy at radiology. The patient underwent a mastectomy. The histology of tumour showed dilated vascular spaces lined by atpical endothelial cells infiltratimg throughout the breast parenchyma. There are solid areas comprising highly atypical spindle or epitheloid cells with hyperchromatic round, oval or spindle nuclei with marked anisonucleosis. On immunohistochemistry the tumour was found positive for CD31, the diagnosis of Angiosarcoma Grade III was made. The patient is now receiving chemotherapy as known on two-month follow-up of the patient. Conclusion: Young women with solid-appearing breast tumors that are highly vascular at the time of biopsy should be considered malignant until proven otherwise.

Reverse abdominoplasty advancement flap: An effective and low morbidity reconstruction option for the radical mastectomy patient

Introduction: Radical mastectomy (RM) is indicated for patients with locally uncontrolled breast carcinoma or angiosarcoma of the breast, for either local control or with curative intent. Flap coverage is routinely offered using pedicled or free flap reconstruction, committing the patient to a long post-operative recovery and risks significant donor site morbidity long-term. We suggest reverse abdominoplasty advancement (RAA) flap a viable option for these high-risk patients, providing a lower risk intra-operative course, earlier return to normal activities of daily living and preserved oncological outcomes.

Clinical and Molecular Insights of Radiation-Induced Breast Sarcomas: Is There Hope on the Horizon for Effective Treatment of This Aggressive Disease?

Radiation-induced breast sarcomas (RIBS) are rare entities representing <1% of all primary breast malignancies, limiting most reports to small retrospective case series. They constitute a heterogeneous group of neoplasms, with high-grade angiosarcoma being the most common subtype. Other sarcoma histotypes, such as undifferentiated pleomorphic sarcoma and leiomyosarcoma, can also be identified. Radiation-induced breast angiosarcoma (RIBA) has an incidence of approximately 0.1% after breast-conserving therapy and arises mainly from the dermis of the irradiated breast. MYC gene amplification is highly indicative of secondary breast angiosarcomas. Their clinical presentation often mimics benign port-radiation lesions, leading to a delay in diagnosis and a lost window of opportunity for cure. Surgery with negative margins is the mainstay of treatment of localized RIBS. In the case of angiosarcoma, technical difficulties, including multifocality, infiltrative margins, and difficulty in assessing tumor margins, render surgical treatment quite challenging. A limited number of studies showed that adjuvant radiation therapy reduces local recurrences; therefore, it is proposed by many groups for large, high-grade tumors. Chemotherapy has been evaluated retrospectively in a small subset of patients, with some evidence supporting its use in angiosarcoma patients. Approximately half of patients with RIBA will show local recurrence. In the advanced setting, different therapeutic options are discussed in the review, including chemotherapy, antiangiogenic therapy, and immunotherapy, whereas the need for further research on molecular therapeutic targets is pointed out.

Differences in potential key genes and pathways between primary and radiation-associated angiosarcoma of the breast

BackgroundAngiosarcoma of the breast is a high-grade malignant soft tissue tumor, it can be divided into primary and radiation-associated angiosarcoma(secondary). However, the differences between primary and secondary angiosarcomas in terms of pathogenesis, clinical behavior, early diagnosis biomarkers, genetic abnormalities, and therapeutic targets remain to be fully elucidated. At the same time, due to its rarity, most of current information relating to angiosarcoma is provided by case reports. Therefore, exploring the mechanisms of primary and secondary breast angiosarcoma have important value for the discovery of new biomarkers and research into potential therapeutic targets. MethodsThe differentially expressed genes (DEGs) between 36 cases of primary angiosarcoma and 54 cases of secondary angiosarcoma were screened. Then, the DEGs were used to gene ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment analysis. Then, a protein-protein interaction (PPI) network was constructed using the STRING database. ResultsA total of 18 DEGs were identified, of which 13 were upregulated and 5 were downregulated in secondary breast angiosarcoma. The GO enrichment analysis showed that the DEGs were most enriched in metabolism, energy pathways, and protein metabolism in biological processes. The enriched signaling pathways of DEGs were the transforming growth factor-β (TGF-β), Wnt, Hippo and PI3K-Akt signaling pathways. Then, the PPI network was conducted and hub genes were identified and they were involved in thyroid hormone, Hippo and other signaling pathways. ConclusionThis study lay the foundation for the discovery of effective and reliable molecular biomarkers and essential therapeutic targets for these malignancies.

EP045: Papillary renal cell carcinoma, glioma and colon polyps in a patient with novel POT1 variant

POT1 tumor predisposition (POT1-TPD) is a rare autosomal dominant cancer susceptibility syndrome with fewer than 100 families reported in the literature. It is caused by heterozygous pathogenic variants in the POT1 (protector of telomeres 1) gene. Core cancers associated with POT1-TPD include cutaneous melanoma, chronic lymphocytic leukemia (CLL), glioma and cardiac angiosarcoma. Limited evidence suggests that other associated cancers may include colorectal, thyroid and breast angiosarcomas. Less than 100 families have been identified with POT1-TPD to date.

Abstract P3-19-11: Long-term outcomes of primary and secondary angiosarcomas of the breast: A 20-year single-center experience

Abstract Introduction: Sarcomas of the breast comprise less than 1% of breast malignancies. Angiosarcomas are aggressive tumors that arise in the breast more than other sites, radiation-associated angiosarcomas (RAA) account for 0.05 - 0.2% of breast malignancies. Primary angiosarcomas are mostly diagnosed in women between 30-50 years of age while RAA occur after a prolonged latency following radiation therapy. This study aims to evaluate the management and subsequent outcomes of breast angiosarcomas (BAS) at the McGill University Health Centre (MUHC), a large tertiary care center, from 2000-2020. Methods: Using our institutional cancer registry database, we identified patients with histologically confirmed BAS diagnosed between 2000- 2020. Data was collected retrospectively including demographics, operative and oncologic interventions, and outcomes. Descriptive statistics were used to summarize the characteristics of the study population. Kaplan Meier curves were used to describe progression-free survival probability, overall survival, and corresponding 95% confidence intervals. Results: A total of 14 patients were identified including 3 (21.4%) with primary BAS and 11 (78.6%) with radiation-associated angiosarcoma (RAA). The mean age of RAA patients at presentation was 71.4 years (range 59-82), and 44 years for the primary BAS (range: 28-53). The mean latency period in developing RAA was 8.0 years (range 4.4-13.3). Treatment of primary angiosarcomas included surgery in all 3 patients, radiotherapy (RT) in 2 and chemotherapy in one who received neoadjuvant chemotherapy and radiotherapy followed by total mastectomy. Treatment of RAA (n=11) included surgery (100%), radiotherapy (18%), chemotherapy (9%). Total mastectomy was performed in ten (91%) patients, one of whom received neoadjuvant chemotherapy and two adjuvant RT. Partial mastectomy (PM) was performed in one patient. After a median follow-up time of 32.5 months (range 0-172), following initial treatment, five patients (35.7%) experienced local recurrence, two of whom had had positive margins after initial surgery. Salvage treatments included wide excision (100%), adjuvant RT in two patients (66.6%) and chemotherapy at time of development of distant metastasis in one. In the primary angiosarcoma population, after accounting for one patient that was lost to follow-up, none experienced recurrence or death. Overall survival probability in the whole study population and in the secondary RAA group was 100% at 36 months. The PFS at 24 months was 51.3% (95% CI: 29.6-88.8%) for the whole population and 41.6% (95% CI: 19.9-86.8%) in the RAA group. In the RAA group, out of the five patients that recurred locally, one patient developed a third malignancy (endometrial) and three patients are currently alive. Conclusion: Overall, our 20-year single center experience confirms the rare nature of BAS. Despite a favorable overall survival rate, local recurrence remains a significant challenge. Our results underscore the importance of optimal salvage therapies such as wide surgical excisions, RT, and chemotherapy that in our series lead to better-than-expected overall survival. Given the rarity of BAS, patients should be managed in large tertiary care centers where multidisciplinary management and clinical expertise can guide salvage approaches. Citation Format: Marwan Tolba, Vanessa Di Lalla, Farzin Khosrow-Khavar, Ayesha Baig, Carolyn Freeman, Valerie Panet-Raymond. Long-term outcomes of primary and secondary angiosarcomas of the breast: A 20-year single-center experience [abstract]. In: Proceedings of the 2021 San Antonio Breast Cancer Symposium; 2021 Dec 7-10; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2022;82(4 Suppl):Abstract nr P3-19-11.

Does Angiosarcoma of the Breast Need Nodal Staging?

Breast angiosarcoma is a rare malignancy classically associated with hematogenous metastases. We sought to determine the prevalence of pathologic nodal involvement in patients with nonmetastatic, resected breast angiosarcoma and its association with overall survival. The National Cancer Database was used to identify patients with nonmetastatic angiosarcoma of the breast who underwent surgical resection from 2004 to 2017. The prevalence of regional lymph node operation and nodal positivity was calculated. The Kaplan-Meier method was used to evaluate overall survival among node-positive and node-negative patients. Cox proportional hazard modeling was used to evaluate the adjusted association of nodal positivity with overall survival. We included 991 patients with angiosarcoma. The median age was 69 years (interquartile range 57 to 78), and the cohort was 99% female. A total of 298 patients (30%) had pathologic regional nodal evaluation. Of those, 15 (5.0%) had positive regional lymph nodes. Node-positive patients had significantly worse survival than patients with negative regional lymph nodes. After adjusting for patient, tumor, and treatment factors, a positive regional lymph node was associated with worse overall survival compared with patients with no nodal evaluation (hazard ratio 3.20; 95% CI 1.75 to 5.86; p < 0.001). Patients with nonmetastatic angiosarcoma of the breast have a 5% regional lymph node positivity rate, which is at a common threshold to consider evaluation, and identifies patients with poor survival. A prospective study to determine performance characteristics of sentinel lymph node biopsy is warranted.

Radiotherapy induced breast angiosarcoma as a rare entity : A case report

Background: Angiosarcoma(AS) is a rare vascular malignancy that originates from the vascular endothelial cells.It can arise anywhere in the body,most commonly in skin of the scalp and neck followed by breast,liver,spleen,and heart.Based on etiology,breast Angiosarcoma(BAS) can be classified into:primary(de novo) and secondary after radiotherapy.Due to its rarity,treatment of BAS is based on the experience gained from other soft tissue sarcomas management.In this paper we report a case of secondary BAS.

Hyperfractionated-Accelerated Reirradiation with Proton Therapy for Radiation-Associated Breast Angiosarcoma.

PurposeRadiation-associated angiosarcoma (RAAS) is a rare complication among patients treated with radiation therapy for breast cancer. Hyperfractionated-accelerated reirradiation (HART) improves local control after surgery. Proton therapy may further improve the therapeutic ratio by mitigating potential toxicity.Materials and MethodsSix patients enrolled in a prospective registry with localized RAAS received HART with proton therapy between 2015 and 2021. HART was delivered twice or thrice daily in fraction sizes of 1.5 or 1.0 Gy, respectively. All patients received 45 Gy to a large elective volume followed by boosts to a median dose of 65 (range, 60-75) Gy. Toxicity was recorded prospectively by using the Common Terminology Criteria for Adverse Events, version 4.0.ResultsThe median follow-up duration was 1.5 (range, 0.25-2.9) years. The median age at RAAS diagnosis was 73 (range, 60-83) years with a median latency of 8.9 (range, 5-14) years between radiation therapy completion and RAAS diagnosis. The median mean heart dose was 2.2 (range, 0.1-4.96) Gy. HART was delivered postoperatively (n = 1), preoperatively (n = 3), preoperatively for local recurrence after initial management with mastectomy (n = 1), and as definitive treatment (n = 1). All patients had local control of disease throughout follow-up. Three of 4 patients treated preoperatively had a pathologic complete response. The patient treated definitively had a complete metabolic response on her posttreatment PET/CT (positron emission tomography–computed tomography) scan. Two patients developed distant metastatic disease despite local control and died of their disease. Acute grade 3 toxicity occurred in 3 patients: 2 patients undergoing preoperative HART experienced wound dehiscence and 1 postoperatively developed grade 3 wound infection, which resolved.ConclusionHART with proton therapy appears effective for local control of RAAS with a high rate of pathologic complete response and no local recurrences to date. However, vigilant surveillance for distant metastasis should occur. Toxicity is comparable to that in photon/electron series. Proton therapy for RAAS may maximize normal tissue sparing in this large-volume reirradiation setting.

Use of extended field-of-view ultrasound imaging in giant primary breast angiosarcoma: a case description.

Use of extended field-of-view ultrasound imaging in giant primary breast angiosarcoma: a case description.

Radiation Therapy Dose and Fractionation Schedules for Breast Angiosarcomas: An Analysis of the National Cancer Database

Breast angiosarcoma (AS) is an uncommon malignancy that is either secondary to prior radiation therapy (RT) or can occur in the primary setting in the absence of prior RT. Since these malignancies are rare, there are scant prospective data to guide the optimal use and timing of surgery, chemotherapy and RT. Here, we set to focus on details of RT use for primary AS and secondary AS with a focus on the dose, fractionation and timing of RT relative to surgery using the National Cancer Database (NCDB). We hypothesized that RT use would be more frequent in primary AS compared to secondary AS. In addition, we hypothesized that when RT is used, hyperfractionated schedules and preoperative RT would be more common in secondary AS compared to primary AS.We identified patients with a histologic diagnosis of AS from the NCDB from 2004-2016. Patients that were coded as having a prior malignancy were used as a surrogate to denote secondary AS whereas the rest were classified as primary AS. RT use was defined as conventional fractionation (1.8-2.0 Gy/fraction given daily); hypofractionated (> 2.0 Gy/fraction given daily); accelerated hyperfractionated (≤1.5 Gy/fraction given 2-3 times/day); palliative (total dose≤30 Gy); or unknown (insufficient RT details provided). Comparisons between the primary AS and secondary AS groups were performed using the chi-square test with P < 0.05 considered significant.We identified 1,174 patients with non-metastatic AS: 864 secondary AS, 310 primary AS. Radiation was used in 211 patients (18.0%) and was significantly more common in primary AS compared to secondary AS (36.1% [N = 113] vs. 11.4% [N = 98], P < 0.0001). The majority of patients received conventional fractionation (63.5%, N = 134) followed by unknown schedule (16.1%, N = 34), hyperfractionated RT (10.4%, N = 22), palliative RT (6.6%, N = 14), and hypofractionated RT (3.3%, N = 7). Fewer than 10% of patients (N = 18) received preoperative RT but its use was more common in secondary AS vs. primary AS (13.3% vs. 4.4%, P = 0.02). Accelerated hyperfractionated RT use was also more common in secondary AS vs. primary AS (19.4% vs. 2.7%, P < 0.0001). Fractionation schedules for the 22 patients treated with accelerated hyperfractionation included: 1.5 Gy BID to 45-60 Gy in 11 patients; 1.2 Gy BID to 50.4-60 Gy in 8 patients; 1 Gy TID to 45-60 Gy in 3 patients. An additional boost dose of 7.2-15 Gy was given to 11 of these 22 patients.Radiation use for the treatment of breast AS is uncommon, even for patients with primary AS. When RT is used, conventional fractionation schedules predominate. However, accelerated hyperfractionation is used more frequently in secondary AS with 1.5 Gy BID to doses≥45 Gy as the most common regimen. Preoperative RT is rarely used for AS based on this analysis. The under-utilization of RT and wide variations in RT dose/fractionation schedules emphasize the need for more standardized consensus guidelines for this rare but aggressive malignancy.

Clinical Outcomes for Primary and Radiation-Associated Angiosarcoma of the Breast with Multimodal Treatment: Long-Term Survival Is Achievable.

Simple SummaryPrimary angiosarcoma of the breast (PAS) and radiation-associated angiosarcoma of the breast (RAAS) are rare sarcomas that affect the inner lining of blood vessels in the breast with incidences of 0.05% and 0.02%, respectively. This study demonstrates that multimodal treatment with neoadjuvant chemotherapy, radiation therapy (in some patients), surgery, and adjuvant chemotherapy may result in optimal clinical outcomes, including prolonged survival. While this study reflects a small sample size, it demonstrates that neoadjuvant chemotherapy, especially when associated with a pathologic complete response, may contribute to these substantial results. Breast angiosarcoma, however, remains a rare disease and prospective, multi-institutional studies need to be performed to overcome the inherent limitations associated with low incidence.Background: The optimal management of primary angiosarcoma (PAS) and radiation-associated angiosarcoma (RAAS) of the breast remains undefined. Available data show persistently poor survival outcomes following treatment with surgery or chemotherapy alone. The objective of this study was to evaluate long-term outcomes in patients treated with multimodality therapy. Methods: Patients diagnosed with stage I–III PAS or RAAS of the breast were identified from our local tumor registry (2010–2020). Patient demographics, tumor characteristics, and treatment were collected. Primary outcomes were local recurrence (LR), distant recurrence (DR), and median overall survival (OS). A secondary outcome was pathologic complete response (pCR) following neoadjuvant chemotherapy (NAC). Mann–Whitney U, chi-squared, or Fisher exact tests were used to analyze data. Kaplan–Meier curves compared OS for PAS and RAAS. Results: Twenty-two patients met inclusion criteria, including 11 (50%) with RAAS and 11 (50%) with PAS. Compared to PAS patients, RAAS patients were older and had more comorbidities. For RAAS patients, median time from radiation to diagnosis was 6 years (IQR: 5–11). RAAS patients were more likely to have a pCR to NAC (40% vs. 20%, p = 0.72). RAAS patients had a higher LR rate (43% vs. 38%, p = 0.83), and PAS patients were more likely to develop a DR (38% vs. 0%, p = 0.07). Median OS was 81 months in PAS patients and 90 months in RAAS patients (p = 1.00). Discussion: Long-term survival can be achieved in patients with PAS and RAAS who undergo multimodality treatment. NAC can result in pCR. The long-term clinical implications of pCR warrant further investigation.

Clinicopathologic and immunohistochemical study of breast angiosarcoma

Breast angiosarcoma (AS) is a rare malignancy which can be classified into primary or secondary as a result of breast cancer therapy. On histology, breast AS has a wide spectrum of morphologic presentations, and its diagnosis can be challenging based on morphologic evaluation alone. Here, we studied 10 cases of breast AS diagnosed at our institution during a 20-year period, in which 7 cases were radiation-associated AS (RA-AS) and 3 cases were primary AS (P-AS). The average latency between radiotherapy and RA-AS was 8.1 years. RA-AS mostly occurred in breast skin, while all P-AS involved breast parenchyma. All 10 AS cases were high grade, including 4 RA-AS cases demonstrating epithelioid morphology. Histologic morphologies of AS varied from confluent growth of atypical spindle or epithelioid cells to scattered marked pleomorphic cells. Some cases appeared deceptively bland or low grade, but the presence of areas of haemorrhage (‘blood lake’) or necrosis upgraded them to high grade lesions. Additionally, some epithelioid RA-AS cases with lymphatic differentiation (D2–40 positive) showed pseudopapillary morphology characterized by discohesive cells sloughing off at periphery of vascular cores, resembling papillary breast carcinoma. P-AS did not show prominent vesicular nuclei and/or conspicuous nucleoli, which were features observed in RA-AS. C-MYC immunostain results showed P-AS was completely negative or focal weakly positive in hypercellular areas. In comparison, RA-AS were consistently positive for c-MYC. Epithelioid RA-AS with lymphatic differentiation tended to show stronger and/or more diffuse c-MYC positivity than other AS cases. CD31 and ERG immunostains showed positivity in all cases, while CD34 were negative in some cases with lymphatic differentiation. This study offers a detailed morphologic and immunohistochemical assessment of a rare tumor of the breast that is important to recognize. Common differential diagnosis for breast AS, including post-radiation atypical vascular proliferation (AVP), are also reviewed and discussed.

Early diagnosis of breast angiosarcoma

Early diagnosis of breast angiosarcoma

Primary Angiosarcoma of Breast – A Rare Case Presentation

Breast sarcomas are histologically heterogenous group of non- epithelial malignancies arising from mesenchymal component of the breast. Angiosarcoma of the breast constitutes 1 % of all soft tissue tumour of the breast and primary angiosarcoma is an extremely rare entity with an incidence of 0.05% of all the breast tumour. We report a case of primary angiosarcoma of the breast in a 57 year old woman, with no previous radiotherapy and is treated with breast conservative surgery.

Primary Angiosarcoma of Breast – A Rare Case Presentation

Breast sarcomas are histologically heterogenous group of non- epithelial malignancies arising from mesenchymal component of the breast. Angiosarcoma of the breast constitutes 1 % of all soft tissue tumour of the breast and primary angiosarcoma is an extremely rare entity with an incidence of 0.05% of all the breast tumour. We report a case of primary angiosarcoma of the breast in a 57 year old woman, with no previous radiotherapy and is treated with breast conservative surgery.

Primary breast angiosarcoma: a case report

BackgroundAngiosarcomas are rare and fast growing tumors that arise from vascular structures. This case report is significant because it is rare and may cause treatment delay if not considered as a differential diagnosis. Hence, we aim to inform efficient, appropriate, and evidence-based imaging practice to the benefit of patients regionally and worldwide.Case presentationThirty-five-year-old lady, presented with a large left breast swelling. She had recent history of trauma to the breast which initially caused confusion regarding the possible diagnosis. Ultrasound and MRI raised high suspicion of an angiosarcoma. Patient went through embolectomy prior to mastectomy and axillary clearance. Histopathology confirmed the diagnosis of an angiosarcoma. She had no distant metastases at the time of diagnosis and the patient was treated with chemotherapy and radiotherapy. However, she returned with recurrence within 1-year duration.ConclusionPrimary breast angiosarcomas are rare aggressive vascular tumors with generally poor prognosis and no pathognomonic signs on radiological imaging, especially mammogram. Ultrasound and MRI do help in characterizing these tumors and raising the suspicion of a vascular tumor. Ultrasound-guided biopsy is recommended to avoid unnecessary complications. In young patients with rapidly growing, highly vascular breast tumors, a differential diagnosis of primary angiosarcomas should be considered until proven otherwise.

Primary and Secondary Angiosarcoma of the Breast: Report of Two Cases and Review of the Literature

Introduction: The breast's angiosarcoma is a rare entity, representing 1% of the breast's malignant neoplasms and is classified as primary and secondary; the latter is associated with radiotherapy and chronic lymphedema. Clinically both present as a voluminous and rapidly growing lesion. Surgery is the cornerstone of a treatment since it provides the most outstanding local control, whereas radiotherapy and chemotherapy have little impact on the disease. Similarly, the oncological prognosis is poor because this type of tumor has a high recurrence rate and overall, 5-year survival of only 20%. Objective: Two cases of angiosarcoma of the breast (primary and secondary) are reported, both treated in the Breast Tumor Service and a bibliographic review of the disease is presented. Materials and Methods: All breast cancer cases were reviewed in the Breast Tumor Service for two years. Results: In the first clinical case, a 61-year-old patient was presented with a 5-month-old primary angiosarcoma in the right breast, treated with modified radical mastectomy. While in the second case, the clinical history of a 76-year-old woman with secondary breast angiosarcoma is related; the patient had a history of right breast cancer, treated ten years previously with conservative surgery, sentinel node and adjuvant radiotherapy. However, the woman presented local recurrence six years later, and she was operated on with a total mastectomy. Three years later, she developed an angiosarcoma in the surgical bed that warranted a wide resection of the chest wall. Conclusion: The breast's angiosarcoma is an aggressive and rare tumor of unknown aetiology, since it has a high recurrence rate and poor survival. Its main prognostic factors are tumor volume and resection margins. Therefore, early diagnosis is essential because it allows complete resection of the lesion.