Primary Angiosarcoma Of Breast Research Articles

Clinicopathologic and molecular correlates to neoadjuvant chemotherapy-induced pathologic response in breast angiosarcoma.

Both primary and secondary breast angiosarcoma (AS) are characterized by multifocal presentation and aggressive behavior. Despite multimodality therapy, local and distant relapse rates remain high. Therefore, neoadjuvant chemotherapy (NACT) is employed to improve the R0 resection rates and survival, but its benefits remain controversial. Herein, we investigate pathologic and molecular correlates to NACT-induced histologic response in a group of 29 breast AS, 4 primary and 25 radiation-associated (RA). The two NACT regimens applied were anthracycline- and non-anthracycline-based. The pathologic response grade was defined as: I: ≤ 50%, II: 51%-90%, III: 91%-99%, and IV: 100%. An additional 45 primary AS and 102 RA-AS treated by surgery alone were included for survival comparison. The genomic landscape was analyzed in a subset of cases and compared to a cohort of AS without NACT on a paired tumor-normal targeted DNA NGS platform. All patients were females, with a median age of 31 years in primary AS and 68 years in RA-AS. All surgical margins were negative in NACT group. The NACT response was evenly divided between poor (Grades I-II; n = 15) and good responders (Grades III-IV; n = 14). Mitotic count >10/mm2 was the only factor inversely associated with pathologic response. By targeted NGS, all 10 post-NACT RA-AS demonstrated MYC amplification, while both primary AS harbored KDR mutations. TMB or other genomic alterations did not correlate with pathologic response. All four patients with Grade IV response remained free of disease. The good responders had a significantly better disease-specific survival (p = 0.04). There was no survival difference with NACT status or the NACT regimens applied. However, NACT patients with MYC-amplified tumors showed better disease-free survival (p = 0.04) compared to MYC-amplified patients without NACT. The overall survival of NACT group correlated with size >10 cm (p = 0.02), pathologic response (p = 0.04), and multifocality (p = 0.01) by univariate, while only size >10 cm (p = 0.03) remained significant by multivariate analysis.

Primary Breast Angiosarcoma: Serial Cases

Background: Breast angiosarcoma is a rare malignancy arising from endothelial cell lining, approximately 0.04–0.05% of all breast malignancies and less than 1% of all sarcomas. Consisting of primary breast angiosarcoma (PAS) and secondary breast angiosarcoma (SAS). Diagnosis is often delayed because the case is rare and asymptomatic, the lump is painless, grows quickly, progressive disease can develop distant metastasize, and the prognosis is reportedly poor. Case presentation: This case series presents two cases of primary breast angiosarcoma; the first patient is a patient who needs to obtain a diagnosis through physical examination, imaging, surgery, histopathology, and immunohistochemistry. The second patient is how to reconstruct the surgical defect using the Latissimus dorsi flap, and interestingly, with thirteen years of survival without recurrence, Surgery is the main treatment with high local recurrence. Wide excision in the form of a simple mastectomy is recommended if a tumor-free margin of 2-3 cm cannot be achieved. Tumor excision, or cosmesis, is not achieved, which is related to the proportion of breast and tumor size. The use of radiation therapy and neoadjuvant or adjuvant chemotherapy is still controversial. Conclusion: Establishing a diagnosis with a thorough examination starts with anamnesis, physical examination, radiological examination, and histopathology, including immunohistochemistry, which is an important examination in confirming the diagnosis.

Secondary Breast Angiosarcoma After a Primary Diagnosis of Breast Cancer: A Retrospective Analysis of the Surveillance, Epidemiology, and End Results (SEER) Database.

Angiosarcoma is a rare complication of breast-conserving therapy. This study evaluated the change in incidence between 1992 and 2016 of secondary breast angiosarcoma (SBA) in patients with a history of breast cancer and the impact of management strategies for the original breast carcinoma on angiosarcoma treatment. Breast cancer and angiosarcoma cases were abstracted from the Surveillance, Epidemiology, and End Result (SEER) database. SBAs were defined as angiosarcomas located in the breast occurring after a prior breast cancer diagnosis. Primary breast angiosarcomas (PBAs) were defined as an angiosarcoma diagnosis listed as "one primary only." Incidence rates were estimated using a proportion of the US total population. Survival was analyzed by the Kaplan-Meier method, and Cox proportional hazard models were used to assess the association of clinicopathologic characteristics on overall survival. Between 1992 and 2016, 193 cases of SBA were reported in the SEER dataset in patients with a prior history of breast cancer. The incidence of breast angiosarcoma in patients with a prior diagnosis of breast cancer increased 3-fold from about 10 cases per 100,000 person-years to about 30 cases per 100,000 person-years over this same period ( P =0.0037). For treatment of SBA (n=193), almost all (95%) had surgery. Nine percent received radiation (compared with 35% of patients with PBA, P <0.001) and 23% received chemotherapy (vs. 45% for PBA, P =0.11). We demonstrate an increasing incidence of SBA over the study period. These data can help inform shared decision-making for optimal management of locoregional breast cancer and raise awareness of secondary angiosarcoma.

Angiosarcoma in the breast: a population-based cohort from Sweden.

Breast angiosarcoma is a rare disease mostly observed in breast cancer (BC) patients who have previously received radiotherapy (RT). Little is known about angiosarcoma aetiology, management, and outcome. The study aim was to estimate risk and to characterize breast angiosarcoma in a Swedish population-based cohort. The Swedish Cancer Registry was searched for breast angiosarcoma between 1992 and 2018 in three Swedish healthcare regions (population 5.5 million). Information on previous BC, RT, management, and outcome were retrieved from medical records. Overall, 49 angiosarcomas located in the breast, chest wall, or axilla were identified, 8 primary and 41 secondary to BC treatment. Median age was 51 and 73 years, respectively. The minimum latency period of secondary angiosarcoma after a BC diagnosis was 4 years (range 4-21 years). The cumulative incidence of angiosarcoma after breast RT increased continuously, reaching 1.4‰ after 20 years. Among 44 women with angiosarcoma treated by surgery, 29 developed subsequent local recurrence. Median recurrence-free survival was 3.4 and 1.8 years for primary and secondary angiosarcoma, respectively. The 5-year overall survival probability for the whole cohort was 50 per cent (95 per cent c.i., 21 per cent-100 per cent) for primary breast angiosarcoma and 35 per cent (95 per cent c.i., 23 per cent-54 per cent) for secondary angiosarcoma. Breast angiosarcoma is a rare disease strongly associated with a history of previous BC RT. Overall survival is poor with high rates of local recurrences and distant metastasis.

Clinicopathological analysis and prognostic treatment study of angiosarcoma of the breast: a SEER population-based analysis

IntroductionBreast angiosarcoma is a rare malignancy of endovascular origin, accounting for less than 1% of all mammary cancers. Our aim was to explore clinicopathological features and the factors associated with prognosis.MethodsWe extracted information from the Surveillance, Epidemiology, and End Results Program (SEER) for all patients with breast angiosarcoma between 2004 and 2015. Chi-square analysis was used to compare the clinicopathological features in all patients. Overall survival (OS) was assessed using the Kaplan and Meier method. Univariate and multivariate analyses were performed to evaluate the factors associated with prognosis.ResultsA total of 247 patients were included in the analyses. The median OS of patients with primary breast angiosarcoma (PBSA) and secondary breast angiosarcoma (SBAB) was 38 months and 42 months, respectively. The 1-, 3- and 5-year OS with PBSA was 80%, 39%, and 25%, respectively, and the 1-, 3- and 5-year OS with SBAB was 80%, 42%, and 34%, respectively. Multivariate analysis revealed that tumor size (p = 0.001), tumor grade (p < 0.001), tumor extension (p = 0.015), and tumor spread (p < 0.001) were statistically significant factors for OS. Partial mastectomy with radiation (HR = 0.160, 95% CI, 0.036–0.719, p = 0.016), partial mastectomy with chemotherapy (HR = 0.105, 95% CI, 0.011–1.015, p = 0.052), and partial mastectomy (HR = 0.125, 95% CI, 0.028–0.583, p = 0.007) were related to significantly better OS outcomes in primary angiosarcoma.ConclusionPrimary breast angiosarcoma has a better clinical phenotype than secondary breast angiosarcoma. Although overall survival was not statistically significant, primary breast angiosarcoma was better than secondary breast angiosarcoma with systemic therapy. Depending on the outcome of survival, partial mastectomy is effective in treating primary breast angiosarcoma.

Clinicopathological characteristics and survival outcomes in patients with angiosarcoma of breast.

Angiosarcoma of the breast is a rare malignancy. There are little data evaluating the survival and estimating the prognostic factors. The best surgical management and the role of systemic adjuvant therapy remain ill-defined. This study aimed to investigate the clinicopathological features, survival, and prognostic factors of breast angiosarcoma. The data on patients diagnosed with breast angiosarcoma were extracted from the Surveillance, Epidemiology, and End Results database (1975-2016). Univariate and multivariate Cox regression analyses were used to estimate the influential prognostic factors. The overall survival (OS) and disease-specific survival (DSS) of patients with breast angiosarcoma were evaluated. This study included 656 patients diagnosed with breast angiosarcoma between 1975 and 2016. The 5-year OS rate of all patients was 44.9% (95% CI 40.8-49.0). In both OS and DSS, Kaplan-Meier survival analyses revealed significant differences for both OS and DSS according to age, year at diagnosis, laterality, grade, and stage (all log-rank p < 0.05). Multivariate analysis suggested that lesions of the right breast, poor differentiation, and advanced stage were independent risk factors for OS or DSS (all p < 0.05). Older age was a risk factor in OS, but was protective in DSS. In primary breast angiosarcoma, age, laterality, grade, and stage were independent prognostic factors in OS and DSS (all p < 0.05). Mastectomy was also a risk factor in DSS (p = 0.034). The proportion of patients with grade III and regional disease was larger in the mastectomy group. Angiosarcoma of the breast had a poor prognosis. In our study, age, laterality, histologic grade, and stage were identified as significant prognostic factors. Why patients with angiosarcoma of the right breast had a worse prognosis remains equivocal. Mastectomy was adopted more often by surgeons in this cohort study for patients with advanced primary breast angiosarcoma.

Angiosarcoma of the Breast: Overview of Current Data and Multimodal Imaging Findings

Angiosarcoma of the breast is a rare breast cancer, which can arise de novo (primary breast angiosarcoma, PBA) or as a secondary malignancy (secondary breast angiosarcoma, SBA) as a result of a biological insult. In the latter case, it is usually diagnosed in patients with a previous history of radiation therapy following a conserving treatment for breast cancer. Over the years, the advances in early diagnosis and treatment of breast cancer, with increasing use of breast-conserving surgery and radiation therapy (instead of radical mastectomy), brought about an increased incidence of the secondary type. PBA and SBA have different clinical presentations and often represent a diagnostic challenge due to the nonspecific imaging findings. The purpose of this paper is to review and describe the radiological features of breast angiosarcoma, both in conventional and advanced imaging to guide radiologists in the diagnosis and management of this rare tumor.

Primary Breast Angiosarcoma: A case report and literature review

Primary Breast Angiosarcoma: A case report and literature review

Primary angiosarcoma of the breast: a case report

Background: Angiosarcoma of the breast is a rare form of breast malignancy. Primary breast angiosarcoma occurs sporadically primarily in young women while secondary breast angiosarcoma most frequently develops in older patients with prior history of radiation therapy or chronic lymphedema. This aggressive malignant tumor of the vascular endothelium has a high risk of local recurrence and distant metastasis and the diagnosis carries a poor prognosis.

A Review on Angiosarcoma of the Breast: Case Studies

An uncommon kind of cancer called angiosarcoma originates in the lining of lymph and blood arteries. The immune system includes the lymphatic vessels. The lymph vessels remove waste materials, viruses, and germs from the body. Any region of the body can develop cancer of this kind. Angiosarcoma is an aggressive tumour, It is a form of soft tissue sarcoma. The treatment of angiosarcoma is very difficult. Breast cancer arises in the breast's lymphatic or blood vessel networks eventually migrating to the breast and the skin of the arms. Most angiosarcomas have unknown origins. Researchers have found a number of variables that could raise the disease's risk. Angiosarcoma develops when the DNA of cells lining a blood artery or lymph channel changes. The instructions that inform a cell what to do are encoded in its DNA. The adjustment which scientists refer to as mutations, instruct the cells to divide quickly. When healthy cells would perish, the alterations prevent the cells from dying. As a result, cancer cells can accumulate and spread outside of the blood vessel or lymph channel. Cancerous cells are able to infiltrate and obliterate healthy body tissue. Cancer cells could eventually separate and travel to different parts of the body. Angiosarcomas have a rapid development rate and body-wide dissemination. Breast angiosarcomas come in two varieties, primary and secondary, and they are both treated surgically by removing the tumour. Angiosarcoma can be spread in other part of body from breast. Primary breast angiosarcoma is an uncommon kind of breast cancer that only affects women, typically developing in the third to fourth decade. For diagnosis, biopsy, mammogram, breast MRI, PET Scan, ultrasound were performed and for the treatment of breast cancer surgery, chemotherapy, radiotherapy were performed. Only 0.04% of malignant breast tumours are primary breast angiosarcomas, making it a rare form of breast cancer. Secondary malignant tumour growth is one of the dangers of therapeutic radiation. In this paper, various cases are reported of angiosarcoma of the breast.

MYC amplification in angiosarcoma depends on etiological/clinical subgroups – Diagnostic and prognostic value

Angiosarcomas (AS) are rare and heterogeneous malignant vascular tumors with a dismal prognosis. We undertook a retrospective study of AS cases with the intent of elucidating the prevalence of MYC amplification amongst different etiological/clinical subgroups and identifying MYC's potential as a prognostic biomarker.Retrospective collection of data and tumor samples from 110 patients diagnosed with AS in the Netherlands was conducted. Histopathological review and investigation of MYC gene amplification and MYC protein overexpression (using fluorescent in situ hybridization and immunohistochemistry, respectively) was performed.MYC amplification was identified in 50.9 % of cases and predominantly present in secondary AS (sAS) (especially radiotherapy-associated and Stewart-Treves AS, both 92.9 %). Within the primary AS (pAS) subgroup, skin non-UV-associated AS (69.2 %) and primary breast AS (29.4 %) demonstrated MYC amplification. MYC protein overexpression was observed in 47.7 % of all tumors with an overall sensitivity and specificity of 75 % and 81 % respectively. There was no significant difference in median overall survival (OS) between patients with MYC and non-MYC amplified AS. Patients with AS displaying MYC protein overexpression had significantly shorter OS than those without (p = 0.028).Although MYC amplification is characteristic for secondary radiotherapy-associated and Stewart-Treves AS, it is not exclusive to these groups and can also be found in a subset of pAS. Overall concordance of FISH and IHC is rather poor. Only primary breast AS showed 100 % concordance. Therefore MYC expression as surrogate marker should be used with caution. The role of MYC/MYC as a prognostic indicator is undefined, however resulting tailored treatment options could be considered.

The Complex Management of the Breast Angiosarcoma: A Retrospective Study

Background/Aim: Breast angiosarcoma is a rare and aggressive disease with a poor prognosis. Two subtypes have been identified: primary angiosarcoma (PBA) and secondary breast angiosarcoma (SBA). In this retrospective analysis, we describe and compare our institute experience with the data existing in the literature. Materials and Methods: We included in our analysis 29 patients who received a diagnosis of PBA or SBA between 2006 and 2019. Results: All patients received surgery as frontline treatment, but only 6 patients underwent to adjuvant treatment. Neoadjuvant chemotherapy was administered 2 patients. The preferred chemotherapeutic regimen was taxanes with or without gemcitabine and associated with anthracyclines. A lower median RFS and OS were reported in patients with PBA compared to those with SBA, but the difference observed was not statistically significant. Patients with PBA had a lower median age at the diagnosis (38 vs. 75). Conclusion: In our analysis, we have shown a lower median RFS and OS in patients with PBA compared with those with SBA, and a significantly younger age at diagnosis in patients affected by PBA.

Radiotherapy for primary angiosarcoma of the breast: a case report

Background: Primary breast angiosarcomas are very rare type malignant breast tumor. It may have an insidious clinical onset, presenting as a painless, often discrete palpable mass that grows rapidly. The ranged age of disease is 20-50 years. Case description: We report a case of a young woman with palpable and painless mass of her left breast. Based on radiological findings with mammography and MRI examination reported as malignant mass of the left breast suspected angiosarcoma. Pathological confirmation post-surgery reported a well-differentiated angiosarcoma that arose primarily in the left breast. Patient treated with simple mastectomy followed by whole breast radiotherapy. External beam radiotherapy delivered using 3DCRT with FIF technique, prescribed dose 60Gy in 30 fractions within 6 weeks. Conclusion: There is no established standard treatment for primary angiosarcoma of the breast. Mastectomy remains the mainstay of treatment. Adjuvant radiotherapy appears to improve local control.

The Role of Imaging in the Diagnosis of Primary and Secondary Breast Angiosarcoma: Twenty-Five-Year Experience of a Provincial Cancer Institution

BackgroundBreast angiosarcoma may arise spontaneously (primary breast angiosarcoma (PBA)) or may arise secondary to a biological insult, such as radiation therapy (secondary breast angiosarcoma (SBA)). We evaluated the imaging findings of patients diagnosed with PBA and SBA within the province of British Columbia, Canada. Materials and MethodsThis was a multi-center, retrospective study of patients diagnosed with PBA and SBA over a 25-year period. Patients were identified via a provincial database which registers all cases of sarcoma. Patients diagnosed with histologically proven PBA and SBA were eligible for inclusion. Multimodal breast imaging reviewed included mammography, ultrasound, magnetic resonance imaging, and computed tomography. ResultsThirteen patients were diagnosed with PBA and 22 patients were diagnosed with SBA. The median (interquartile range (IQR)) age of patients diagnosed with PBA (45.5 years (19.7 years)) was less than that of patients diagnosed with SBA (75.8 years (13.8 years), P < .001). Patients diagnosed with PBA (90.9%) were more likely to present with a parenchymal mass clinically and radiographically than those with SBA (28.6%, P < .002). Patients diagnosed with SBA (71.4%) were more likely to present with cutaneous findings than patients diagnosed with PBA (0.0%, P < .05). Without specific clinical context, the imaging findings of PBA and SBA were observed to be non-specific. ConclusionThis is the only study which evaluated the imaging findings of patients diagnosed with PBA and SBA within a large, defined geographical area. Given non-specific imaging findings, awareness of the disease and clear and timely communication between radiologists and clinicians is required to ensure appropriate diagnosis and management.

Histopathologic Grading Is of Prognostic Significance in Primary Angiosarcoma of Breast: Proposal of a Simplified 2-tier Grading System.

Despite a wide spectrum of clinical presentations, including primary or secondary, most angiosarcomas are considered high grade. One exception is primary breast angiosarcoma, where historically, histologic grading has shown to predict outcome using the Rosen 3-tier system. However, more recent studies have challenged this concept suggesting that even in this specific clinical context angiosarcomas should be considered high grade. This study aimed to critically reevaluate the impact of histologic grade in a clinically uniform cohort managed at a single institution using a newly proposed grading system. Our study included 49 primary breast angiosarcomas diagnosed during 1994 to 2022 (median follow-up: 33mo), classified as low grade (29%), intermediate grade (20%), and high grade (51%), based on mitotic count, extent of solid components, and necrosis. At last follow-up, 22% patients developed locoregional recurrences, 63% distant metastases, and 47% patients died of disease. As patients with low and intermediate-grade angiosarcomas had relatively similar outcomes, our cohort was further analyzed using a 2-tier system (low grade and high grade). Targeted-DNA next-generation sequencing (505 cancer gene panel) performed in 11 cases found KDR mutations in 78% and PIK3CA mutations in 44% of high-grade lesions. Histologic grade, by either 3-tier or 2-tier grading systems, had a strong impact on survival, with the 2-tier system being an independent predictor of disease-specific survival and overall survival. Based on 2-tier system, the 5-year overall survival was 38% for high-grade angiosarcoma and 74% for low-grade angiosarcoma. PIK3CA mutations alone or concurrent with KDR alterations were identified in angiosarcomas with worse prognosis.

A clinicopathologic and immunohistochemical study of primary and secondary breast angiosarcoma.

We aimed to study the clinicopathologic and immunohistochemical (IHC) (CD117, c-Myc, and p53) characteristics, and overall survival of primary and secondary breast angiosarcoma (BAS). This was a retrospective study of BAS cases diagnosed between 1997 and 2020 at our institution. Hematoxylin and eosin-stained slides were reviewed for tumor morphology, margin status, and lymph node metastasis. CD117, p53, D2-40, CD31, and c-Myc IHC stains were performed on 11 viable tissue blocks. Additional clinical information was obtained from the electronic medical records. Seventeen patients with BAS were identified. Of these, five (29%) were primary and 12 (71%) were secondary BAS, respectively. The median age at diagnosis for primary BAS was 36 years. The median age at diagnosis for secondary BAS was 67 years. The median time to secondary BAS development following radiotherapy was 6.5 years (range, 2 to 12 years). There was no significant difference between primary and secondary BAS in several histopathologic parameters examined, including histologic grade, necrosis, mitotic count, lymph node metastasis, and positive tumor margins. There was also no difference in CD117, p53, D2-40, CD31, and c-Myc expression by IHC between primary and secondary BAS. During a median followup of 21 months, primary BAS had two (40%) reported deaths and secondary BAS had three (25%) reported deaths. However, this difference in survival between both groups was not statistically significant (hazard ratio, 0.51; 95% confidence interval, 0.09 to 3.28; p = .450). BAS is a rare and aggressive disease. No histologic, IHC (CD117, c-Myc, and p53), or survival differences were identified between primary and secondary BAS in this study.

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.

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.

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.