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Background Primary and secondary breast angiosarcoma is a rare and aggressive malignancy with limited published literature. Optimal management is mostly based on expert opinion. Our study aims to describe a single institution experience with breast angiosarcoma and evaluate other publications on this topic to further clarify prognostic outcomes and treatment modalities in this disease. Methods Twenty two cases of breast angiosarcoma from Roswell Park Comprehensive Cancer Center were retrospectively analyzed. Additionally, a systemic review and meta-analysis was conducted to study the association between survival outcomes, overall survival (OS), and recurrence-free survival (RFS) in both primary (PAS) and secondary breast angiosarcoma (SAS). Results 9 PAS patients (41%) and 13 SAS patients (59%) were retrospectively analyzed. No significant differences were noted in tumor characteristics and survival outcomes between PAS and SAS. Treatment modality had no significant effects on survival outcomes although adjuvant chemotherapy demonstrated a trend towards improved RFS in high grade tumors. 380 PAS and 595 SAS patients were included in the outcome meta-analysis. Survival outcomes were significantly worse with high grade tumors and tumor size of > 5 cm. Adjuvant radiation therapy demonstrated significantly better RFS, while adjuvant chemotherapy had no effect on survival outcomes. Conclusion Tumor size and grade seem to be reliable predictors of survival in both PAS and SAS. Mastectomy does not seem to be adding any additional benefit to BCS. Adjuvant radiation therapy showed statistically significant RFS benefit, while adjuvant chemotherapy can be beneficial in high grade tumors.

Background Breast angiosarcomas (AS) are rare and aggressive malignancies, categorised as primary angiosarcoma (PAS) and radiation-associated angiosarcoma (RAAS). Due to its rarity, large series of PAS and RAAS are limited. We aimed to analyse the outcome of a large cohort of angiosarcomas with emphasis on the prognostic factors, latency, margin width and significance of locoregional recurrences. Methods A retrospective study was conducted on angiosarcomas managed at a large UK Regional Sarcoma Centre between 2013 and 2024. Clinical, pathological, disease-free survival (DFS) and overall survival (OS) data were collected. The interval between radiotherapy delivery and the development of angiosarcoma was calculated. Cox regression models and binary logistic regression were utilised for optimal threshold values for resection margins and patient outcomes. Results PAS presented at a younger age (median 31 vs 71 years for RAAS). A significant shortening of latency between radiotherapy and onset of RAAS was found (r = − 0.719, p  < 0.001). DFS and OS were 59.6%, and 54.2% respectively. Smaller microscopic resection margins were significantly associated with recurrence ( p  = 0.001). Recurrence was a strong predictor of mortality, HR = 2.856, p = 0.005. Mean survival with and without angiosarcoma recurrences was 32.9 and 80.5 months respectively ( p  = 0.005). Resection margins ≥ 10 mm were significantly associated with lower rates of recurrences ( p  = 0.044, OR = 0.323). However, resection margin distance did not directly impact survival (HR = 1.002, p  = 0. 798). Cox regression analysis showed angiosarcoma size was not a predictor of survival ( p  = 0.278, HR = 1.002). Neither patient age nor the angiosarcoma histological grade correlated with recurrences or patient survival. C-Myc immunohistochemistry was positive in four of nine (44.44%) PASs and in 95.52% of RAAS. Its expression did not correlate with patient survival. Conclusion Over the last decade, the number of diagnosed RAAS cases has increased with a shortening in the interval between radiotherapy and onset of RAAS. Margin status and recurrences, but not angiosarcoma grade or size, impact survival. Achieving a clear surgical margin is therefore critical for improved patients’ outcome.

Angiosarcoma is a rare, aggressive malignant tumor of vascular origin, often characterized by rapid growth and infiltrative behavior. While it commonly occurs in the skin, particularly on the scalp of elderly patients, it can also affect other regions. We present a case of a 76-year-old male with a rapidly growing, well-demarcated nodule on the dorsum of the hand, which was excised for pathological examination. Histopatho-logical analysis revealed spindle-shaped tumoral cells with a storiform pattern, poor vascularization, and immunohistochemistry positivity for CD31 and CD34. The case underscores the importance of distinguishing angiosarcoma from other spindle-cell tumors, such as leiomyosarcoma and atypical fibroxanthoma, and highlights the essential role of immunohistochemistry in achieving an accurate diagnosis. Early detection and appropriate surgical management are crucial for optimal outcomes in patients with this aggressive malignancy.

Epithelioid angiosarcoma of the vulva is an exceptionally rare and highly aggressive malignancy originating from endothelial cells. Its etiology remains unclear, though risk factors such as prior radiotherapy and chronic lymphedema have been identified. We report a case of a 44-year-old woman with no significant medical history who presented with a rapidly enlarging, foul-smelling mass on the right labia majora. The tumor measured 30 cm and exhibited central necrosis, ulceration, and bleeding. Radical tumor resection and right inguinal lymphadenectomy were performed, followed by reconstruction using a keystone design perforator island flap and a V-Y advancement flap. Histopathology confirmed a high-grade epithelioid angiosarcoma, positive for ERG, CD31, CD34, podoplanin, EMA, and a Ki-67 proliferation index of 60%. Further treatment consisted of adjuvant radiotherapy (61 Gy) and doxorubicin-based chemotherapy. At one-year follow-up, the patient remained free of recurrence or metastasis. Primary epithelioid angiosarcoma of the vulva is extremely rare, particularly in patients without known risk factors. Management involves radical surgical resection with adjuvant radiotherapy and chemotherapy as indicated. Reporting such cases is essential to enhance understanding and guide evidence-based management of these challenging neoplasms.

Introduction: Considering the increasing number of conservative surgeries (quadrantectomies) for primary breast carcinoma, especially in the early stages, often followed by adjuvant radiotherapy, the incidence of radiation-associated angiosarcoma (RAS) is expected to rise in the coming decades, and it will represent a clinical and therapeutic challenge, as limited data are available due to the rarity and heterogeneity of the disease. Though the prognosis of these patients is poor, a number of clinical and pathological factors can be evaluated to better understand the course of RAS. The aim of this systematic review is to explore the available clinical-pathological, therapeutic, and prognostic data regarding RAS to evaluate its occurrence, diagnosis, treatment, and outcomes. Method: RAS clinical data were identified by a systematic review conducted in five different databases (Medline, Web of Knowledge, Google Scholar, Scopus, and Cochrane). Only RAS works published in English, with access to the full manuscript text, and with clear results, were considered as eligible. Results: We considered 52 papers comprising 319 RAS cases. The patient age at diagnosis ranged from 37 to 93 years, with most cases occurring from 5 to 10 years after breast irradiation. The most common clinical presentation was an aggressive development of macules, papules, or erythematous-violaceous skin discoloration at the site of previous radiation treatment for primary breast carcinoma. Complete surgical excision appeared to be the first-line treatment of the disease, in some cases followed by adjuvant local and/or systemic therapies. Despite different treatments, local recurrence rates ranged from 40% to 90%, leading to negative outcomes and poor prognosis for patients. Conclusion: Although the literature is limited and the data are heterogeneous and contentious, our review aims to highlight the importance of early diagnosis, multimodal treatment, and long-term follow-up of RAS in order to limit and prevent the aggressiveness of this neoplasm.

Background and purpose Angiosarcoma (AS), a rare and highly malignant tumour, can manifest spontaneously (primary AS, pAS) or secondary to previous radiation, exposure to chemical agents or Stewart-Treves syndrome (secondary AS, sAS). The aim of this study was to characterise the clinical presentation, management, treatment and outcome—including local recurrence, metastasis and overall survival—among patients diagnosed with AS and treated at Sahlgrenska University Hospital, Gothenburg, Sweden. Study methods and design This is a retrospective single-centre study analysing patients treated for AS at Sahlgrenska University Hospital. Patients were identified over a 21-year period (1 January 2000 to 31 December 2020) using Systematised Nomenclature of Medicine (SNOMED) code M91203. Medical records for all patients were reviewed by the authors. Results The study identified 64 patients with AS. The mean age among all patients was 67 (SD 16.6 range 18–96) years. The cohort comprised 37 patients diagnosed with pAS and 27 patients with sAS. Of the patients diagnosed with pAS, 18 (48.6%) were women while 25 (92.6%) women were diagnosed with sAS. Fifty-four (84%) patients were treated with surgery and 34 (63%) with adjuvant chemotherapy, radiation, or both. Local recurrence after primary surgery was 65.4% in pAS and 63% for sAS patients. Upon study completion, 15 (23%) patients were alive. Thirty-nine (60.9%) patients died of AS, while 10 (15.6%) died of other diseases or undetermined causes. Five-year overall survival rates were 10.2% and 43.5% in the pAS and sAS groups, respectively. Conclusion pASs are observed in both men and women, presenting at different locations in the body. In contrast, the majority of sASs arise in the thoracic region in women previously treated for breast cancer. The study shows a 60% local recurrence rate across both pAS and sAS groups, yielding 5-year overall survival rates of 10.2% and 43.5% in the pAS and sAS groups, respectively.

In the article titled “Primary Angiosarcoma of the Spleen: An Oncological Enigma” [1], the first and last names of all the authors were reversed. The revised authors’ list is shown above.

Hepatic angiosarcoma is an extremely rare, uncontrollable tumor with strong invasion, high mortality rate, and extremely poor prognosis. There is first report on hepatic angiosarcoma accompanied by eosinophilia. A woman with a chief complaint of poor appetite for about 3 months presented to a local hospital with a liver mass. Laboratory examination revealed a significant increase in eosinophils count was 9.58% in the serum. Abdominal MRI revealed multiple focal high-density lesions in the liver and spleen, and cranial MRI revealed multiple high-density shadows in the skull. Laparoscopic biopsy revealed a hepatic angiosarcoma and targeted treatment with Anlotinib was performed. Two months later, the patient died of splenic rupture and bleeding. Hepatic angiosarcoma often has no specific clinical manifestations, and we found that this case can lead to an increase in eosinophils, which is worth the attention of clinical physicians.

We present the case of a young woman without a medical history who presented with a giant right atrial, transtricuspid, and right ventricular mass and in a severe clinical state. Multimodal imaging raised the suspicion of primary cardiac angiosarcoma. Due to rapid hemodynamic and respiratory deterioration, we were forced to perform surgical removal of the mass with a concomitant reconstruction of the involved right heart structures, only 48 h after presentation. The postoperative course was uneventful, and the patient was discharged from the intensive care unit 2 days later. Radical surgical resection with reconstruction of the resected heart structures was the only possible salvage option for giant angiosarcoma, which led to hemodynamic instability. Followed by chemotherapy, this radical approach may prolong survival.