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Purpose: Radiation-associated angiosarcoma of the breast (RAASB) is a rare side effect after breast radiation and has been associated with poor outcomes. At this time, there is no consensus regarding management of RAASB, and the role of reirradiation remains controversial. We present our modern institutional outcomes in managing RAASB with incorporation of neoadjuvant hyperfractionated reirradiation., Methods and Materials: Patients identified were treated between 2016 and 2020 with inclusion of any histologically proven RAASB without metastatic disease at diagnosis, while excluding those with a history of radiation therapy outside of the breast/chest wall or other sarcoma histologies. Major wound complications were defined as requiring wound care and/or wound vacuum or return to the operating room for wound repair at any time after surgery., Results: Eight patients were identified, and the median follow-up was 34 months. Median time to RAASB development was 8 years from initial radiation therapy. With respect to RAASB management, all underwent surgery and neoadjuvant reirradiation therapy, and all but 1 patient received taxol-based chemotherapy. At last follow-up, 7 patients remained free of disease, and 1 patient died with distant disease. With respect to acute toxicity after reirradiation, all patients developed at least acute grade 2 toxicities. Five of the 8 patients developed a major wound complication., Conclusions: Our institutional analysis suggests excellent local control and survival outcomes for RAASB treated with neoadjuvant hyperfractionated reirradiation, surgery, and taxol-based chemotherapy. However, major wound complications represent a major challenge with this approach. Future studies should consider how best to improve the therapeutic ratio while maintaining high rates of local control and survival., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: We report on a series of consecutive patients with localized radiation-associated angiosarcoma (RAAS) of the breast region (BR) treated at two Italian sarcoma reference centers., Materials and Methods: We retrospectively reviewed all cases of primary, localized, resectable RAAS of the BR, treated at one of the two participating institutions from 2000 to 2019. Relapse-free survival (RFS) and overall survival (OS) were calculated. The prognostic role of several variables was investigated. A propensity score matched (PSM) analysis was carried out., Results: Eighty-four patients were retrospectively identified. Nineteen out of 84 patients (22.6%) were pretreated with an anthracycline-based regimen for previous cancer. All patients but one underwent surgery, with 37/84 (44.1%) receiving surgery alone and 46/84 (54.8%) a multimodal approach: 18/84 (21.4%) received radiation therapy (RT) and 46/84 (54.9%) received chemotherapy. An anthracycline-based regimen was used in 10/84 patients (11.9%), while a gemcitabine-based regimen was used in 33/84 (39.3%). With a median follow-up of 51 months (interquartile range: 30-126 months), 36/84 patients (42.9%) relapsed and 35/84 patients (41.7%) died (8/84, 9.5% in the lack of metastatic disease). Five-year OS and 5-year RFS were 57% [95% confidence interval (CI) 43% to 68%] and 52% (95% CI 39% to 63%), respectively. Both (neo)adjuvant RT and chemotherapy were associated with better RFS [hazard ratio (HR) 0.25, 95% CI 0.08-0.83; HR 0.45, 95% CI 0.23-0.89] with a trend towards a better OS (HR 0.51, 95% CI 0.18-1.46; HR 0.60, 95% CI 0.29-1.24). Gemcitabine-based regimens seemed to perform better (HR 4.28, 95% CI 1.29-14.14). PSM analysis retained the above results., Conclusions: This retrospective study supports the use of (neo)adjuvant RT and chemotherapy, in primary, localized resectable RAAS of the BR. An effort to prospectively validate the role of (neo)adjuvant RT and chemotherapy is warranted., Competing Interests: Disclosure EP—outside the submitted work: institutional financial interests: Advenchen Laboratories, Amgen Dompe', Bayer, Blueprint Medicines, Daiichi Sankyo, Deciphera, Eisai, Eli Lilly, Epizyme Inc, Glaxo Smith Kline, Karyopharm Pharmaceuticals, Novartis, Pfizer, PharmaMar. GGB—outside the submitted work: consulting fees from Eli Lilly, PharmaMar, AboutEvents; honoraria from PharmaMar, Eli Lilly, Glaxo Smith Kline, Merck Sharp & Dome, Eisai, Istituto Gentili; support for attending meetings and/or travels from Novartis, PharmaMar, Eli Lilly; participation on advisory board from PharmaMar, Eli Lilly, Glaxo Smith Kline, Merck Sharp & Dome, Eisai. AMF—outside the submitted work: institutional financial interests: Advenchen Laboratories, Amgen Dompe’, Bayer, Blueprint Medicines, Daiichi Sankyo, Deciphera, Eisai, Eli Lilly, Epizyme Inc, Glaxo Smith Kline, Karyopharm Pharmaceuticals, Novartis, Pfizer, PharmaMar. IP—outside the submitted work: institutional financial interests: Advenchen Laboratories, Amgen Dompe', Bayer, Blueprint Medicines, Daiichi Sankyo, Deciphera, Eisai, Eli Lilly, Epizyme Inc, Glaxo Smith Kline, Karyopharm Pharmaceuticals, Novartis, Pfizer, PharmaMar. PGC—outside the submitted work: institutional financial interests: Advenchen Laboratories, Amgen Dompe', Bayer, Blueprint Medicines, Daiichi Sankyo, Deciphera, Eisai, Eli Lilly, Epizyme Inc, Glaxo Smith Kline, Karyopharm Pharmaceuticals, Novartis, Pfizer, PharmaMar. TDP—outside the submitted work: participation on advisory board from Glaxo Smith Kline, Boehringer Ingelheim. Trial support from: Pfizer, BluPrint Medicine, Gilead, Amgen, Merck. SS—outside the submitted work: personal financial interests (honoraria, consultancy or advisory role): Aadi, Astex Pharmaceuticals, Bavarian Nordic, Bayer, Boehringer Ingelheim, Daiichi Sankyo, Deciphera, Epizyme, Gentili, GSK, Agenus, Ikena, MaxiVAX, Novartis, PharmaMar, Pharma Essentia, Rain Therapeutics, Servier; support for attending meetings and/or travel PharmaMar; institutional financial interests: Advenchen, Bayer, Blueprint, Daiichi Sankyo, Deciphera, Epizyme, Eli Lilly, GSK, Hutchinson, Inhibrx, Karyopharm, Novartis, PharmaMar, Rain Therapeutics, SpringWorks; unpaid Member of the Scientific Advisory Board of the Chordoma Foundation, Member of the Scientific Advisory Board of the Desmoid Foundation, Member of the Scientific Advisory Board of the Epithelioid Hemangioendothelioma Group, Member of the Scientific Advisory Board of the Leiomyosarcoma Foundation. All other authors have declared no conflicts of interest., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Angiosarcomas are rare and highly malignant soft tissue sarcomas originating from endothelial cells lining the lymphatic or vascular system. While they predominantly emerge from (sub)cutaneous regions, occurrences have been reported throughout the body. The etiology of angiosarcoma remains elusive in most clinical cases. Nevertheless, several prognosis risk factors play a pivotal role, including chronic lymphedema, therapeutic irradiation, environmental carcinogens, familial syndromes, and the presence of foreign materials like metallic objects and biomedical implants. Despite evidence implicating retained foreign material in angiosarcoma development, understanding its prognosis and pathogenesis remains limited. The pathogenesis of angiosarcoma appears to involve a complex interplay of chronic inflammation, tissue remodeling, and genetic factors that create a conducive microenvironment for malignant transformation. Management of these sarcomas remains challenging due to their infiltrative nature owing to the high chance of metastasis and local recurrence. The primary treatment modalities currently include surgery, radiotherapy, and chemotherapy, but recent advances in targeted immunotherapy and gene therapy hold promise for more effective approaches. This comprehensive review delves into the potential etiological and pathogenic roles of foreign materials, such as metallic objects, biomedical implants, and biomaterials, in the development of angiosarcoma. Further research into the underlying molecular mechanisms could provide valuable insights for tailored management and developing novel targeted therapeutic strategies., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Competing Interests: Declaration of interests We declare no competing interests.

Background: Radiation-induced angiosarcoma (RIA) is an uncommon but morbid complication after radiotherapy for breast cancer., Methods: Retrospective analysis of breast RIA patients at Cambridge University Hospital (CUH), a regional treatment centre in the East of England., Results: 22 patients were identified between 2010 and 2022. Median age of diagnosis was 65 years (range 41-78). Median time from breast radiotherapy to RIA diagnosis was 6.5 years (range 2.4-16.0)-this interval has decreased over the last 24 years (r 2  = 0.6601). 9% had metastasis at presentation. All patients underwent surgery (55% at CUH, 45% at local hospitals). 27% received peri-operative pegylated liposomal doxorubicin in the first-line setting. 62% relapsed following their primary curative-intent treatments after a median of 28 months. Metastases occurred in 36%, the commonest sites being lung (100%) and lymph node (50%). 2-year and 5-year overall survival (OS) rates for all patients were 73% and 60%, respectively. No correlation between progression-free survival (PFS) and OS was found with tumour size, margin, peri-operative chemotherapy, and whether surgery was performed at CUH. Patients with multifocal disease on their breasts had shorter PFS following surgery compared to single-lesion disease (median 10 vs 65 months; HR = 4.359 [95% CI 1.342-14.16]; P = 0.0143). Patients aged > 72 years had a median OS of 45 months vs 102 months for those ≤ 72 years (HR = 7.129 [95% CI 1.646-30.88]; P = 0.0086)., Conclusion: RIA has high rates of recurrence and mortality and appears to be occurring sooner after breast radiotherapy. Further studies on its pathogenesis and effective treatment are warranted., (© 2023. The Author(s), under exclusive licence to The Japanese Breast Cancer Society.)

Cutaneous angiosarcoma (CAS) is a rare soft-tissue sarcoma of vascular endothelial origin. Paclitaxel (PTX) and docetaxel (DTX) are used as systemic chemotherapy; however, chemoresistance often occurs in CAS. Switching one taxane to the other (i.e., PTX to DTX, or vice versa) is an option when the first taxane is no longer effective in malignant cancers such as ovarian or breast cancer. However, the efficacy of the same strategy in CAS has not been reported. Herein, we report the clinical response of switching one taxane-based chemotherapy to the other in CAS patients with resistance to the first taxane. Twelve CAS patients were included for analyses. In all patients, the median overall survival from the start of the first taxane treatment was 29.0 months (range, 6.47-58.5). During the first taxane, the median PFS for all patients was 5.96 months (1.81-47.1). Similarly, the median (range) PFS for all patients during the second taxane was 5.87 months (1.60-18.2). Furthermore, the median OS was 22.7 months (PTX to DTX) and 39.5 months (DTX to PTX) (p = 0.307). The median PFS during the first taxane was 5.14 (PTX to DTX) and 12.5 months (DTX to PTX), respectively (p = 0.380). The median PFS during the second taxane was 3.5 (PTX to DTX) and 7.1 months (DTX to PTX), respectively (p = 0.906). The objective response rate, defined as the sum of complete response (CR) and partial response (PR) rates, was 16.7%. The disease control rate, defined as the sum of CR, PR, and stable disease rates, was 50%. The frequency of adverse events during the second taxane was the same between the two groups (p > 0.999). Our report suggests that CAS patients could benefit from the second taxane treatment if the tumor is resistant to the first taxane., (© 2023 Japanese Dermatological Association.)

Background: Radiation-induced angiosarcoma (RIAS) of the breast is a very rare and poor prognostic disease. According to previous studies, the efficacy of chemotherapy for RIAS is still controversial. However, no study has assessed the prognosis of RIAS and the prognostic impact of preoperative or postoperative chemotherapy in Japanese patients. Our study aimed to assess them in Japanese people using publication data with our three patients., Methods: Thirty-nine patients diagnosed with RIAS, including 36 patients from 34 published case series, and three patients from our hospital were used for analysis. Disease-free survival (DFS), distant disease-free survival (DDFS), and overall survival (OS) were assessed., Results: Among the 39 patients, 36 patients (92.3%) underwent surgery. The median DFS and OS periods were 14 months (range 1-75 months) and 23 months (range 4-84 months), respectively. Chemotherapy with taxane-based regimen was administered in 13 cases (33.2%) pre- or post-operatively. DFS was significantly improved with chemotherapy in addition to surgery (p = 0.037). However, addition of chemotherapy to surgery did not improve DDFS (p = 0.09) and OS (p = 0.878). In multivariate analysis, age ≥ 70 years was an independent but poor prognostic factor of DFS. Additionally, a lack of chemotherapy showed a trend to be associated with worse DFS. There was no independent variable contributing to DDFS and OS., Conclusions: Chemotherapy may have reduced the recurrence rate of RIAS in Japanese patients but did not improve OS. Further data are needed to confirm the efficacy and proper regimen of chemotherapy., (© 2023. The Author(s), under exclusive licence to The Japanese Breast Cancer Society.)

Purpose: To evaluate the impact of trimodality treatment versus monotherapy or dual therapy for radiation-associated angiosarcoma of the breast (RAASB) after prior breast cancer treatment., Experimental Design: With Institutional Review Board approval, we identified patients diagnosed with RAASB and abstracted data on disease presentation, treatment, and oncologic outcomes. Trimodality therapy included (i) taxane induction, (ii) concurrent taxane/radiation, and then (iii) surgical resection with wide margins., Results: A total of 38 patients (median age 69 years) met inclusion criteria. Sixteen received trimodality therapy and 22 monotherapy/dual therapy. Skin involvement and disease extent were similar in both groups. All trimodality patients required reconstructive procedures for wound closure/coverage, compared with 48% of monotherapy/dual therapy patients (P < 0.001). Twelve of 16 (75%) patients receiving trimodality therapy had a pathologic complete response (pCR). With median follow-up of 5.6 years, none had local recurrence, 1 patient (6%) had distant recurrence, and no patients died. Among 22 patients in the monotherapy/dual therapy group, 10 (45%) had local recurrence, 8 (36%) had distant recurrence, and 7 (32%) died of disease. Trimodality therapy demonstrated significantly better 5-year recurrence-free survival [RFS; 93.8% vs. 42.9%; P = 0.004; HR, 7.6 (95% confidence interval, CI: 1.3-44.2)]. Combining all patients with RAASB regardless of treatment, local recurrence was associated with subsequent distant recurrence (HR, 9.0; P = 0.002); distant recurrence developed in 3 of 28 (11%) patients without local recurrence compared with 6 of 10 (60%) with local recurrence. The trimodality group had more surgical complications that required reoperation or prolonged healing., Conclusions: Trimodality therapy for RAASB was more toxic but is promising, with a high rate of pCR, durable local control, and improved RFS., (©2023 American Association for Cancer Research.)

Purpose of Review: Radiation-associated angiosarcoma is a cutaneous aggressive tumor that is very rare and it represents a specific entity poorly studied in literature. It requires new therapeutic opportunity., Recent Findings: The complete surgical resection with negative margins is the mainstay treatment of localized treatment, even though it is difficult to reach in case of diffuse cutaneous infiltration. Adjuvant re-irradiation may improve local control with no benefit demonstrated on survival. Many systemic treatments can be efficient not only in metastatic setting but also in neoadjuvant setting in case of diffuse presentation. These treatments have never been compared to each other; the most efficient regimen remains to be determined, and a high heterogeneity of treatment is observed, even between sarcoma reference centers., Summary: Immune therapy represents the most promising treatment under development. At the time of building clinical trial to assess the efficacy of immune therapy, the lack of randomized studies prevents the identification of a strong and consensual reference arm treatment. Given the rarity of the disease, only international collaborative clinical trials may have a chance to include enough patients to draw any conclusion and so will have to counteract the heterogeneity of management., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Epithelioid angiosarcoma is a rare soft tissue sarcoma with a poor prognosis. We report two cases of patients who presented with a history of lower back pain, inflammatory signs and weight loss 5 and 6 years after endovascular aortic repair (EVAR) of an elective infrarenal abdominal aortic aneurysm (AAA). Imaging suggested graft infection but tissue samples revealed an epithelioid angiosarcoma. The objective is to report the clinical presentation, investigative modalities and immunohistochemical findings of an angiosarcoma after EVAR., Patients and Methods: Two cases are described of an angiosarcoma of the aorta after EVAR. A literature search using PubMed, Embase and Web of Science was performed in English about angiosarcoma after EVAR published between 2007 and 2021. Relevant reports were selected and analysed., Results: Fifteen case reports were identified, including the current two cases. Time to tumour detection after EVAR ranged from 6 to 120 months with a mean interval of 68 months. Most patients underwent endovascular repair of an AAA (13/15). Males (13 male/2 female patients) were predominant with a median age of 72 years (IQR 68-78 years). Over half of the patients had metastases at the time of diagnosis (9/15), most frequently in bones and liver., Conclusion: Diagnosis of angiosarcoma after EVAR remains challenging due to indistinctive clinical and radiological findings mimicking graft infection or endoleak. Angiosarcoma should be included in the differential diagnosis in patients previously treated with EVAR presenting with unintended weight loss, abdominal back pain and contrast enhancement of the aortic wall.AbbreviationsAAAabdominal aortic aneurysmCTAcomputed tomography angiographyCRPc-reactive proteinEVARendovascular aortic repairESRerythrocyte sedimentation rateFDGfluoro-deoxyglucoseMRImagnetic resonance imagingMeSHmedical subject headings.

Background: Angiosarcoma (AS) of the breast is very rare, accounting for 1% of all soft tissue breast tumors. AS may present as primary tumors of the breast or as secondary lesions usually associated with previous radiotherapy. Commonly, secondary AS affects older women (median age 67-71 years) with a clinical history of breast cancer. The preferred site of onset of RIAS is the edge of radiation fields, where radiation doses and tumor necrosis may be heterogeneous, resulting in a DNA damage and instability. Radical surgery is the treatment of choice, but no clear consensus exists on surgical management of breast AS., Case Report: We describe an atypical case of relapsed RIAS after radical mastectomy, treated with new surgery and, considering the higher risk of recurrence, subsequent adjuvant chemotherapy with weekly paclitaxel., Conclusions: The frequency of radiation-induced angiosarcomas (RIAS) after breast-conserving surgery and radiotherapy has been increased to 0.14-0.5% among long survivors. Nevertheless, even if RIAS continues to be prognostically an extremely unfavorable cancer due to a high rate of recurrence, distant spread, and median overall survival (OS) of about 60 months, the benefits of loco-regional breast radiotherapy are clearly higher than the risk in developing angiosarcoma.

Case: Two cases of revision total hip arthroplasty (THA) for pseudotumor and infection with persistent postoperative bleeding because of angiosarcoma are presented. After surgery, both patients' health deteriorated because of hypovolemic shock despite transfusion, pressors, embolization, and prothrombotics. Diagnosis was obscure and delayed despite extensive imaging. Standard and computed tomography angiogram were nondiagnostic and did not localize the tumors or bleeding. Repeat surgeries and biopsies requiring special staining ultimately revealed epithelioid angiosarcoma., Conclusion: A diagnosis of angiosarcoma was etiologic for persistent postoperative bleeding after revision THA and should be considered in such cases., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/C75)., (Copyright © 2023 by The Journal of Bone and Joint Surgery, Incorporated.)

Gemcitabine has shown clinical activity against angiosarcoma in small series, alone, or combined with taxanes. We aimed to evaluate its activity as a single-agent in a larger series of patients with advanced angiosarcoma. We retrospectively reviewed the electronic medical records of consecutive adult patients with advanced angiosarcoma treated with single-agent gemcitabine at our institutions from January 2010 to January 2021. Response was evaluated according to RECIST 1.1, and toxicity was graded according to NCI-CTC v5.0. 42 patients were identified. 38 patients (90%) had received prior anthracyclines and weekly paclitaxel, and 9 (21%) had received pazopanib. The best tumor response was partial response (PR) in 16 patients (38%), or stable disease (10 patients, 24%). All 8 patients with cardiac angiosarcoma experienced a PR. Median PFS was 5.4 months (95%CI: 3.1-6.5), and median OS was 9.9 months (95%CI: 6.6-13.4). Single-agent gemcitabine has clinically meaningful activity in advanced, heavily pre-treated angiosarcoma., (© 2022 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Aim: Post-radiation angiosarcoma is an iatrogenic event seen in the setting of breast cancer treatment. Histopathologically, there are morphologic variants of angiosarcoma that mimic benign entities, including the capillary lobule variant of post-radiation angiosarcoma. We present the largest case series to date of this histopathologic variant of post-radiation angiosarcoma., Methods and Results: Cases of the capillary lobule variant of post-radiation angiosarcoma from institutional/consultation archives from 2008 to June 2022 were reviewed. For inclusion, tumors had to occur in irradiated skin and exhibit a multi-lobular proliferation of tightly packed capillary-like vessels, as previously described in this variant. Prior ancillary studies were also reviewed. Eight cases met the criteria. All occurred in women treated with radiation for breast cancer (median age 75 years). All cases had similar findings, including a multi-lobular proliferation of tightly packed vessels, infiltrative cords, and atypical single endothelial cells. A conventional angiosarcoma pattern was also seen in five cases. All cases tested were positive for vascular markers (CD31, CD34, and/or ERG) and MYC. MYC amplification was shown by FISH in all cases tested. Smooth muscle actin (SMA) was positive in pericytes in the capillary lobules in all five cases tested and areas of conventional angiosarcoma in two of three cases., Conclusions: The capillary lobule variant of angiosarcoma is a rare and therefore potentially under-recognized variant of post-radiation angiosarcoma. The lobular architecture and SMA positivity may mimic benign vascular proliferations. Careful attention to histopathologic features and ancillary tests may facilitate accurate diagnosis., (© 2022 The Authors. Journal of Cutaneous Pathology published by John Wiley & Sons Ltd.)

Background: Soft tissue sarcoma is a rare but serious side-effect of radiotherapy to treat breast cancer, and rates are increasing in the USA. We evaluated potential co-factors in two complimentary cohorts of US breast cancer survivors., Methods: In this retrospective cohort study, we sourced data from the Kaiser Permanente (KP) cohort and the Surveillance, Epidemiology, and End Results (SEER) 13 registries cohort, both in the USA. The KP cohort included 15 940 women diagnosed with breast cancer from Jan 1, 1990, to Dec 31, 2016, in KP Colorado, KP Northwest (which serves Oregon and Southwest Washington state), or KP Washington, with detailed treatment data and comorbidities (including hypertension and diabetes at or before breast cancer diagnosis) from electronic medical records. The SEER cohort included 457 300 women diagnosed with breast cancer from Jan 1, 1992, to Dec 31, 2016, within the 13 SEER registries across the USA, with initial treatment data (yes vs no or unknown). Eligibility criteria in both cohorts were female breast cancer survivors (stage I-III) aged 20-84 years at diagnosis who had breast cancer surgery, and had survived at least 1 year after breast cancer diagnosis. The outcome of interest was any second thoracic soft tissue sarcoma (angiosarcomas and other subtypes) that developed at least 1 year after breast cancer diagnosis. Risk factors for thoracic soft tissue sarcoma were assessed using multivariable Poisson regression models., Findings: In the KP cohort, median follow-up was 9·3 years (IQR 5·7-13·9) and 19 (0·1%) of 15 940 eligible, evaluable women developed a thoracic soft tissue sarcoma (11 angiosarcomas, eight other subtypes). Most (94·7%; 18 of 19) thoracic soft tissue sarcomas occurred in women treated with radiotherapy; thus, radiotherapy was associated with a significantly increased risk of developing a thoracic soft tissue sarcoma (relative risk [RR] 8·1 [95% CI 1·1-60·4]; p=0·0052), but there was no association with prescribed dose, fractionation, or boost. The RR of angiosarcoma after anthracyclines was 3·6 (95% CI 1·0-13·3; p=0·058). Alkylating agents were associated with an increased risk of developing other sarcomas (RR 7·7 [95% CI 1·2-150·8]; p=0·026). History of hypertension (RR 4·8 [95% CI 1·3-17·6]; p=0·017) and diabetes (5·3 [1·4-20·8]; p=0·036) were each associated with around a five-times increased risk of angiosarcoma. In the SEER cohort, 430 (0·1%) of 457 300 patients had subsequent thoracic soft tissue sarcomas (268 angiosarcomas and 162 other subtypes) after a median follow-up of 8·3 years (IQR 4·3-13·9). Most (77·9%; 335 of 430) cases occurred after radiotherapy; thus, radiotherapy was associated with a significantly increased risk of developing a thoracic soft tissue sarcoma (RR 3·0 [95% CI 2·4-3·8]; p&lt;0·0001) and, for angiosarcomas, the RR for breast-conserving surgery plus radiotherapy versus mastectomy plus radiotherapy was 1·9 (1·1-3·3; p=0·012). By 10 years after radiotherapy, the cumulative incidence of thoracic soft tissue sarcoma was 0·21% (95% CI 0·12-0·34) in the KP cohort and 0·15% (95% CI 0·13-0·17) in SEER., Interpretation: Radiotherapy was the strongest risk factor for thoracic soft tissue sarcoma in both cohorts. This finding, along with the novel findings for diabetes and hypertension as potential risk factors for angiosarcomas, warrant further investigation as potential targets for prevention strategies and increased surveillance., Funding: US National Cancer Institute and National Institutes of Health., Competing Interests: Declaration of interests We declare no competing interests., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Epithelioid angiosarcoma is a rare variant of angiosarcoma. Radiation-associated epithelioid angiosarcoma of the urinary bladder and prostate is an exceedingly rare tumor and there are only 8 cases of epithelioid angiosarcoma of the urinary bladder and prostate associated with previous radiotherapy in the literature. To the best of our knowledge, MYC gene amplification has not been previously reported in epithelioid angiosarcoma of the urinary bladder and prostate following radiotherapy, although it is observed in radiation-associated angiosarcoma of other anatomic sites. Here we report the first case of epithelioid angiosarcoma of the urinary bladder and prostate with MYC gene amplification detected by fluorescence in situ hybridization (FISH) analysis in a 70-year-old male patient 10 years after receiving radiation and hormonal therapy for prostate cancer.

Background: Expansion after popliteal artery aneurysm exclusion with bypass is a common phenomenon. Popliteal angiosarcoma is seldom reported in literature and in most cases after popliteal artery aneurysm surgery. This paper aims to present the case of a popliteal angiosarcoma, initially diagnosed as late aneurysmal growth after exclusion surgery, to conduct a systematic review of popliteal angiosarcoma and assess any association between angiosarcoma and previous popliteal aneurysm surgery., Methods: We performed a secondary popliteal aneurysmorraphy through posterior approach for symptomatic aneurysm expansion in a 79-year-old woman, 9 years after medial femoropopliteal venous bypass and aneurysm exclusion. The postoperative course was complicated by recurrent hematomas and wound spillages requiring multiple revisions. Pathological analysis identified an angiosarcoma. Staging revealed bone invasion and pulmonary metastasis. Despite transfemoral amputation and adjuvant chemotherapy the patient died 8 months later. We performed a systematic review through MEDLINE on 'primary' and 'secondary' (with previous vascular surgery) angiosarcoma in popliteal artery aneurysm. Research was done using the terms '(hem) angiosarcoma', 'aneurysm', 'popliteal aneurysm or artery', 'femoral aneurysm or artery'. Other soft tissue sarcoma or nonpopliteal locations were excluded., Results: Including this case, only 13 angiosarcomas in popliteal aneurysms are currently described. Two were reported without previous surgery considered as primary angiosarcoma and 11 after popliteal artery aneurysm surgery (secondary angiosarcoma). Patient age ranges from 8 months to 83 years with a male predominance (10/3). Nine of the 11 patients with secondary angiosarcoma were initially diagnosed as popliteal aneurysm expansion after previous bypass surgery, the 2 other secondary cases presented respectively with pain and inflammatory syndrome without expansion. All prior surgical exclusion was carried out by a medial approach. Interval with the index operation ranges from 3 months to 15 years. Death was reported in 8 of the 13 cases within the first year of diagnosis., Conclusions: Although seldom reported, popliteal angiosarcomas are mainly described after popliteal artery aneurysm exclusion surgery, raising suspicion on a potential association, yet causality cannot be demonstrated. Angiosarcoma should be included in the differential diagnosis of popliteal aneurysm growth or unexpected outcome after exclusion bypass surgery. Systematic imaging and pathological studies should be undertaken to allow early diagnosis and treatment. Routine use of a posterior approach, with aneurysm resection, when feasible as initial popliteal artery aneurysm treatment, might reduce the risk of late sarcomatous transformation., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: A potentially fatal disease of breast angiosarcoma (AS) can occur after breast cancer treatments, and previous studies have revealed that lymphedema is a risk factor of AS development. However, little is known, and there is no systematic review focusing on the relationship between lymphedema and breast AS. This systematic review aimed to evaluate all publications on breast AS after primary breast cancer surgery to identify prognostic factors and evaluate treatment modalities., Methods: Databases were searched for articles with published individual patient data on prognostic factors, treatment, and follow-up of patients with breast AS after primary breast cancer surgery. Statistical analysis was performed to test the prognostic values of age, gender, location, tumor-node-metastasis classification of primary breast cancer (tumor and node), stage of primary breast cancer, treatment for primary breast cancer, breast cancer recurrence, post-operative lymphedema, latent period from primary breast cancer to AS, treatment for AS, AS recurrence, and time until death or last follow-up., Results: Twenty articles were included, representing data on 41 patients. In these articles, 15 patients were referred to post-operative lymphedema. Eleven patients of them had post-operative lymphedema. As for this research, post-operative lymphedema was not a significant prognostic factor. AS recurrence was significant as a prognostic factor (P-value < 0.001) in univariate analysis for follow-up status; patients who had AS recurrence had a shorter time until death., Conclusion: In this systematic review, AS recurrence was of prognostic value for breast AS patients after primary breast cancer surgery. There are only a few articles that refer post-operative breast lymphedema., Competing Interests: Conflicts of Interest None., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Background: DNA damage and oncogenic viruses increase the risk of cancer post-kidney transplantation, including skin cancer, Kaposi's sarcoma, oral cancer, and non-Hodgkin lymphoma. Here we report an uncommon case of liver angiosarcoma that occurred 8 years after kidney transplantation. This study strictly complied with the Helsinki Congress and the Istanbul Declaration regarding donor source., Case Report: A 57-year-old female patient received a cadaver kidney transplantation 8 years ago. She followed a long-term regimen of tacrolimus, mycophenolate sodium, and everolimus, with good renal function. She received annual regular abdominal ultrasound examinations after kidney transplantation, which showed no findings. The patient suffered from several symptoms for approximately 2 weeks before a scheduled abdominal ultrasound: diarrhea, epigastric pain, abdominal fullness, tea-colored urine, and little stool passage. The abdominal computerized tomography showed multiple hepatic tumors in both the hepatic lobes with engorged vasculatures and mild hemoperitoneum. A liver biopsy revealed the histopathology of angiosarcoma. The patient suffered multiple organ failure within one month of treatment., Conclusions: Various post-transplant malignancies are not uncommon after transplantation, warranting periodic screenings for any symptoms in these patients., (Copyright © 2022 Elsevier Inc. All rights reserved.)

A 67-year-old man was admitted to our hospital for massive pleural effusion. He had a history of mandibular gingival carcinoma treated with radiation therapy (RT). Based on the cytology findings of pleural effusion and a thoracoscopic pleural biopsy, we finally diagnosed him with radiation-associated angiosarcoma. Retrospective cell-block immunocytochemistry with pleural effusion also showed potential utility for the diagnosis. This case highlights the importance of considering the possibility of radiation-associated secondary cancer in patients with pleural effusion who have a history of RT.

A 40-year-old woman with silicone implants inserted 21 years before presented with sudden onset of painful right breast swelling. Clinical examination revealed a firm swollen breast with appearance of old bruising. Ultrasound showed fluid around the implant. Cytology of the fluid showed cells with large pleomorphic nuclei with prominent nucleoli including elongated forms and very occasional vacuoles. The cell block also contained small fragments with atypical spindle cells around slit-like spaces that were positive for CD31 and CD34. MRI showed a 25 mm serpiginous area of enhancement on the inner aspect of the fibrous capsule with haematoma between the capsule and the implant. The capsule and adjacent area were excised. Histology showed angiosarcoma extending from the inner aspect of the capsule into the cavity around the implant. The location of the tumour on the inner aspect of the capsule is the same site that breast implant associated anaplastic large cell lymphomas arise and suggests a possible causal link between the implant and the angiosarcoma. This case emphasises the value of cytological assessment of fluid around breast implants and the role of cell blocks and immunohistochemistry., (© 2022 Wiley Periodicals LLC.)

The authors present the case of a 94-year-old woman suffering from a right arm angiosarcoma developed after primary breast cancer and treated with success by oral metronomic chemotherapy based on daily low doses of cyclophosphamide and prednisone. The case description is followed by a short review of actual knowledge on the subject., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Opinion Statement: Cutaneous angiosarcoma is a rare and invasive malignant tumor. For localized cAS patients, wide-margin excision was recommended. Due to the latent local invasion characteristic of cAS, we suggest preoperative and postoperative radiotherapy to nearly all patients. Recently, there is growing interest in using neoadjuvant chemotherapy and/or radiotherapy as part of a combination therapy regimen, which may allow some patients to undergo potentially less disabling surgery. For metastatic cAS patients with unresectable tumors and who refuse surgery, radical radiotherapy or chemoradiotherapy may be an option. Paclitaxel was recognized as the first-line treatment. For tumors resistant to taxanes, emerging medications such as targeted agents and immunotherapy are also under investigation., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Primary angiosarcoma of the breast is very rare. Surgery is the principal mode of treatment for primary angiosarcoma of the breast and generally consists of a total mastectomy. To discuss its etiology and treatment principles, we reported a case of primary mammary angiosarcoma in an adolescent girl. The patient had been treated in other hospitals several times due to erythema of the breast as inflammatory treatment was considered, until pathological nature was determined after we performed pathological needle puncture. The case discussed here is unique in its own way, having clinical imaging and pathological features of primary angiosarcoma in an adolescent girl and it is known that primary angiosarcoma is unusual for this age group.

Background: Radiation-associated angiosarcoma of the breast (RAASB) is a serious late consequence caused by breast cancer treatment. Initial symptoms are often inconspicuous, thus contributing to diagnostic delay. Most previous studies of the diagnostic aspects of RAASB are case reports., Purpose: To perform a complete review of the imaging findings and biopsy methods in a nationwide RAASB cohort., Material and Methods: RAASB patients were identified from a national cancer registry and additional patients were included from our hospital. All available information from imaging (mammogram [MGR], ultrasound [US], magnetic resonance imaging [MRI], and computed tomography [CT]) and biopsies was reviewed. The sensitivity of imaging and biopsy methods for detection of RAASB was calculated., Results: Fifty-eight patients with RAASB were found. Fourteen MGR, 30 US, 24 MRI, and 25 CT studies were available for evaluation. The sensitivity of MGR, US, MRI, and CT for detection of RAASB was 43%, 50%, 92%, and 84%, respectively. Superior sensitivity was demonstrated for punch biopsy (84%) and incisional biopsy (93%) compared to fine-needle aspiration cytology (0%) and core needle biopsy (18%)., Conclusion: MRI and CT have comparable sensitivity for detection of RAASB, while MGR and US are unreliable. However, negative findings in MRI or CT must be interpreted with caution. Punch biopsy and incisional biopsy are the preferred biopsy methods.

Background: Radiation-associated angiosarcoma (RAAS) of the breast is an aggressive malignancy affecting 1 in 1000 breast cancer patients. This study aimed to determine differences in treatments and outcomes for RAAS initially managed through a sarcoma multi-disciplinary team (SMDT) compared with an outside center (OC) and to describe outcomes after recurrence., Methods: Patients with a diagnosis of breast RAAS between 2004 and 2019 were identified from our sarcoma database. Clinicopathologic characteristics, recurrence patterns, and factors predictive of survival were assessed. Differences in local recurrence-free survival (LRFS) and disease-specific survival (DSS) were estimated using Kaplan-Meier and compared using the log-rank test., Results: Surgery was performed for 49 women with RAAS, who had a median age of 74 years (range 41-89 years). Primary management was performed by SMDT for 26 patients and by OC for 23 patients. Radical mastectomy and reconstruction were performed for 96% of the SMDT group versus 17% of the OC group (p = 0.00001). The proportion patients who received chemotherapy, radiation, or both was 42.3% in the SMDT group and 0% in the OC group. During a median follow-up period of 26 months, recurrence was experienced by 38% (10/26) of the SMDT cohort and 83% (19/23) of the OC cohort (p = 0.002). The 3-year LRFS was better in the SMDT cohort (59.3% vs 31.8%; p = 0.019). Of the 29 recurrences 16 received chemotherapy and 6 received radiation, surgery, or both. At the last follow-up visit, 20 patients were in first remission, 1 patient was in second remission, 8 patients were alive with disease, and 20 patients had died of disease., Conclusion: Initial treatment by SMDT was associated with more extensive surgery, multimodal treatments, and a better 3-year LRFS. Patients with breast RAAS likely benefit from early referral and treatment by an SMDT., (© 2021. Society of Surgical Oncology.)

Introduction: Angiosarcoma secondary to post-irradiation and lymphedema is rare, but it is aggressive with a poor prognosis. It is essential to understand these patients' clinical features and distinguish them from benign diseases or other malignant tumors., Patient Concerns: Three patients who had radiotherapy for cancer treatment and chronic lymphedema admitted to the hospital with specific skin lesions at upper or lower extremities., Diagnosis: Excisional biopsies revealed prominent, highly atypical cells with a vasoformative area, composed of atypical, large epithelioid cells with vesicular nuclei, prominent nucleoli, and mitoses. Immunohistochemistry revealed diffuse expression of endothelial cell markers suggestive of angiosarcoma., Interventions: One patient had shoulder disarticulation with wide excision with adjuvant radiotherapy and chemotherapy and other 2 discontinued the treatment., Outcomes: After the treatment, one patient was transferred to rehabilitation department for shoulder disarticulation prosthesis fitting without recurrence sign for 1 year. Two patient refused further treatment and was lost to follow-up., Conclusion: In cases of patients with irratiation and chronic lymphedema, clinical findings suggestive of angiosarcoma, biopsy and imaging studies should be performed as soon as possible., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objectives: We report the case of a 68-year-old male who was hospitalized for suspicion of endoleak and thrombosis of an aortic endoprosthesis, arising after multiple endovascular interventions during the last few months. During the intervention, amass was discovered, and biopsies were made. The anatomopathology results were in favor of an epithelioid angiosarcoma with atypical expression of CD31 and ERG- and no amplification of c-MYC. The main objective of this review is to highlight the difficulty of differential diagnosis, but also to evaluate overall survival according to treatments. Methods: We performed a large review of the literature using PubMed for reports concerning angiosarcoma arising from Dacron grafts from 1981 to 2019. Articles presenting potentially relevant studies were read and analyzed. Results: In our review, most of the patients were male (10 cases over 11 described), with amedian age of 63 years old (50-84 years old, 11 cases described). The overall interval time for the diagnosis after the endoprosthesis placement was 7.8 years (from 3.5 years to 17 years, 10 cases with the description) and the overall survival was 5 months (from 0 to 10 months, the only patient alive is not included, and only 8 cases had the description). Conclusion: In most of the reviewed cases, there was no information concerning immunohistochemistry. Biopsies remain the standard for the diagnosis with immunochemistry and molecular test to avoid amisdiagnosis. Epithelioid angiosarcomas derived from Dacron grafts are a rare entity, which are difficult to diagnose because of the paucity of cases. Prognosis is poor, even if surgical option is possible.

Introduction : Spleen angiosarcoma (SA) is a rare malignant neoplasm that arises from the splenic vascular endothelium, with only around 300 cases reported to date. Due to a limited number of reported cases, there is a paucity of data and a lack of understanding of its presentation, diagnosis, and management. In this study, we aim to provide a comprehensive review of SA. Areas covered : On 27 February 2021, a literature search was done in PubMed and Embase database. The search yielded 122 articles involving 205 patients. The focus was on patient demographics, risk factors, clinical presentations, investigation results, preliminary diagnoses, therapies provided, and patient outcomes. These factors were analyzed to identify possible risk factors, diagnostic modalities, and therapeutic principles that were not mentioned before. Expert opinion : The clinical presentation or investigation results of patients with SA are often nonspecific. Hence, they may not be sufficient to clinch the diagnosis of SA if used alone. The authors recommend a triple assessment of clinical examination, imaging findings, and pathology to diagnose SA with high accuracy. Splenectomy should be the mainstay of management, with chemotherapy and radiotherapy considered as adjuncts, especially in the presence of metastases.

Purpose: Angiosarcoma (AS) is a rare vasoformative sarcoma, with poor overall survival and a high need for novel treatment options. Clinically, AS consists of different subtypes, including AS related to previous UV exposure (UV AS) which could indicate susceptibility to DNA damage repair inhibition. We, therefore, investigated the presence of biomarkers PARP1 (poly(ADP-ribose)polymerase-1) and Schlafen-11 (SLFN11) in UV AS. Based on experiences in other sarcomas, we examined (combination) treatment of PARP inhibitor (PARPi) olaparib and temozolomide (TMZ) in UV AS cell lines., Methods: Previously collected UV AS (n = 47) and non-UV AS (n = 96) patient samples and two UV AS cell lines (MO-LAS and AS-M) were immunohistochemically assessed for PARP1 and SLFN11 expression. Both cell lines were treated with single agents PARPi olaparib and TMZ, and the combination treatment. Next, cell viability and treatment synergy were analyzed. In addition, effects on apoptosis and DNA damage were examined., Results: In 46/47 UV AS samples (98%), PARP1 expression was present. SLFN11 was expressed in 80% (37/46) of cases. Olaparib and TMZ combination treatment was synergistic in both cell lines, with significantly increased apoptosis compared to single agent treatment. Furthermore, a significant increase in DNA damage marker γH2AX was present in both cell lines after combination therapy., Conclusion: We showed combination treatment of olaparib with TMZ was synergistic in UV AS cell lines. Expression of PARP1 and SLFN11 was present in the majority of UV AS tumor samples. Together, these results suggest combination treatment of olaparib and TMZ is a potential novel AS subtype-specific treatment option for UV AS patients., (© 2021. The Author(s).)

Background: Angiosarcoma of the breast is rare and aggressive. It can occur as a de novo tumor or secondary to breast cancer treatment. The purpose of this study is to analyze differences between patients with primary and secondary angiosarcoma of the breast and investigate potential risk factors for its development., Patients and Methods: The Surveillance, Epidemiology, and End Results program of the National Cancer Institute database was queried to identify patients with angiosarcoma of the breast, trunk, shoulder, and upper arm. The population-based incidence was analyzed. Primary and secondary angiosarcoma cases were identified and compared. Breast cancer characteristics of secondary angiosarcoma patients were compared with all breast cancer patients in the database who did not develop angiosarcoma., Results: Overall, 904 patients were included, and 65.4% were secondary angiosarcomas. These patients had worse survival, were older, more likely to be White, more likely to have regionally advanced disease, and had angiosarcoma tumors of higher pathologic grade. Independent factors associated with development of secondary angiosarcoma among breast cancer patients included White race, older age, invasive tumor, lymph node removal, lumpectomy, radiation treatment, and left-sided tumor. Although the mean time to develop angiosarcoma after breast cancer diagnosis was 8.2 years, the risk continues to increase up to 30 years after breast cancer treatment., Conclusion: Angiosarcoma is rare but increasing in incidence. Secondary angiosarcomas are more common and exhibit more aggressive behavior. Several factors for angiosarcoma after breast cancer treatment could be identified, which may help us counsel and identify patients at risk., (© 2021. Society of Surgical Oncology.)

Purpose: The breast sarcoma induced by radiation therapy is rare but increasing, given the increased long-term survival of patients receiving radiation therapy. Fibrosarcoma, histiocytofibroma and angiosarcoma are the most common breast sarcoma. Angiosarcoma is the most common after breast cancer treated by radiation therapy, often diagnosed too late, with a severe prognosis and a high rate of recurrence. However, because of the low incidence of angiosarcoma associated with radiation therapy (AAR), the benefit of radiation therapy in breast cancer treatment outweighs the risk to develop angiosarcoma. The aim of this study is to evaluate these rare cases of AAR diagnosed in eastern Belgium in comparison to the data from the literature., Patients and Methods: Nine cases of AAR after radiation for breast ductal carcinoma were included in this retrospective study. AAR was diagnosed according to Cahan criteria between January 2007 and December 2016. Latency, incidence, management and prognosis are comparable to the literature., Results, Conclusion: The median latency was 10 (4-24) years, the incidence of AAR in the East Belgian area was 0.09% of the patients irradiated on the same period. Patients were treated by surgery with wide local excision with or without reconstructive surgery, without radiotherapy and chemotherapy treatment. Kaplan-Meier analysis showed median overall survival of 61.8 months, patient survival of 55.6% at one year and 29.6% at five years. With the constant progress of medicine and its technologies, it would be possible to limit the occurrence of AAR or to diagnose it at an earlier stage., (Copyright © 2020 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.)

Postradiation cutaneous angiosarcoma of the breast is a rare, delayed complication of adjuvant radiation treatment for breast carcinoma and is associated with a worse prognosis than the original primary cancer. Recent studies have characterized the diagnostic utility of MYC and NOTCH1 receptor expression as markers for secondary radiation-associated angiosarcomas. Herein, we report an exophytic secondary breast angiosarcoma with MYC and NOTCH1 immunoreactivity. This case illustrates the utility of these markers for the identification of radiation-associated angiosarcoma with MYC and NOTCH1 expression, potential for targeted therapy and need to identify patients for further studies of the clinicopathologic and prognostic significance., (© 2021 Wiley Periodicals LLC.)

Secondary angiosarcoma of the breast following catheter-based brachytherapy after lumpectomy is rare. We describe a case of a patient with breast cancer treated with partial mastectomy and sentinel node biopsy followed by accelerated partial breast irradiation (APBI), who developed skin changes 6 years after completion of therapy. Punch biopsy confirmed the diagnosis of secondary angiosarcoma. This case is even more unique in that the location of the skin changes was remote to the lumpectomy site. There is a critical need to recognize secondary angiosarcoma presentation after APBI and determine the rate of occurrence compared with traditional external beam irradiation., (© 2020 Wiley Periodicals LLC.)

Background: Radiotherapy-associated angiosarcoma (RAA) of the breast is a rare complication of radiotherapy, which is often difficult to identify and has poor prognosis. It usually presents as violaceous skin, erythema or rapidly growing palpable firm mass that can be confused with other benign skin lesions., Patients and Methods: After reviewing the literature, we found only four cases with RAA after mastectomy and autologous breast reconstruction. The presented case is the first that was treated by electrochemotherapy. The patient presented with secondary angiosarcoma of the breast five years after mastectomy, immediate breast reconstruction with deep inferior epigastric artery perforator free flap and adjuvant radiotherapy., Results: Electrochemotherapy was feasible, safe and effective in treatment of radiation induced sarcoma. Most of the treated lesions in several consecutive electrochemotherapy sessions responded with complete response, but multiple recurrences occurred in non-treated areas., Conclusions: Patients with breast cancer after skin-sparing mastectomy and immediate breast reconstruction, who receive radiotherapy, need regular long-term follow up and low threshold for biopsy of any suspicious lesions is mandatory. Electrochemotherapy proved as one of feasible modalities of treatment for RAA., (© 2021 Romi Cencelj-Arnez, Jerica Novak, Andreja Klevisar Ivancic, Masa Bosnjak, Maja Cemazar, Marko Snoj, published by Sciendo.)

Primary angiosarcoma (AS) of the breast is an extremely unusual variant of breast malignancies, and its incidence is about 0.05% of all primary breast tumors. In this article, we present a rare case of a primary AS that developed in a young woman with breast implants. This case report emphasizes importance of early investigation for accurate diagnosis and proper management of the breast AS, along with a correlation of histopathologic, radiologic, and clinical findings.

Background: Angiosarcoma is a rare malignant tumor, originating from vascular endothelial cells, accounting for approximatively 1-2% of soft tissue sarcomas. It is characterized by a rapid proliferation and high metastatic potential. Some cases of angiosarcoma are described in association with vascular prosthesis, orthopedic devices and foreign bodies. Hereby, we report a case of a patient treated with the endovascular placement of a PTFE aorto bis-iliac prosthesis for aortic aneurysm, who developed a graft-related angiosarcoma with bone and peritoneal localizations. The peritoneal "sarcomatosis" led to an acute presentation with hemoperitoneum and anemia. We perform a thorough review of the literature summarizing the description of similar cases, their epidemiology and the possibilities for treatment., Case Presentation: An 84-year-old male with a history of abdominal aortic aneurysm endovascular repair presented to our emergency department complaining with low back pain radiating to the left limb. He underwent a type II endoleak embolization of the aneurysmal sac nine days before. During hospitalization he underwent a spine MRI which documented a vertebral alteration of non-univocal interpretation. Vertebral biopsy was performed revealing groups of cells of uncertain nature. He lately underwent percutaneous L2-L4 arthrodesis. Forty-two days after admission, he developed acute anemia. Emergency laparotomy revealed a massive hemoperitoneum and actively bleeding peritoneal nodules. Abdominal packing was performed, and several nodules were sent for definitive histological examination. After surgery, he developed progressive and severe hypovolemic shock and expired on postoperative day 5., Conclusions: Angiosarcoma associated with foreign bodies, especially vascular prosthesis, is a very rare entity. In patients who have a history of prosthetic vascular graft placement that present with lumbar pain, osteolytic changes at radiologic imaging or the development of ascites, angiosarcoma should be considered in the differential diagnosis. Despite the poor prognosis, a prompt diagnosis might give access to an adequate treatment planning, with the aim for disease control and increased survival.