Primary Angiosarcoma Research Articles

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

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

Clinicopathologic and immunohistochemical study of breast angiosarcoma

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

Primary angiosarcoma of aorta: A systematic review

To elucidate the epidemiology, anatomical, presentation, classification, pathology, investigative modalities, management and prognosis of primary angiosarcoma of the aorta. A systematic review of literature from the database inception to January 2021 in PubMed and Embase, CINAHL and Cochrane Library in accordance to PRISMA was conducted. Retrieval and extraction was performed by two independent reviewers. The hierarchy of the evidence was assessed through the National Institute for Health and Care Excellence Checklist. Data were subjected to pooled prevalence analysis, Kaplan-Meier survival and test of probability using log-rank analysis. This review is registered with International Prospective Register of Systematic Reviews: RD42021231314. 82 studies with n = 123 cases met the inclusion criterion. Abdominal (45%) aorta was the commonest anatomical site with female predominance in ascending aorta (4:1) and aortic arch (2:1). The longest survival was in the ascending aorta and the shortest in the abdominal aorta [540 (interquartile range [IQR], 7-1560days vs. 180 (IQR, 1-5730days)], respectively. The overall median survival was 210 days (IQR, 1-5730days) or 7months. Lack of metastasis (47%) was a marker of longer survival (p < 0.03) irrespective of other attributes. The pathophysiology appears to be a trend of increasing fatigue, fever and weight loss associated with segmental dysfunction of the aorta projecting occlusive or destructive phenotypes. Computed tomography angiography features of volume-occupying, bulky, polypoid (intraluminal), protrusive vegetation, hyper vascular without atherosclerotic lesions are extremely suggestive of PA of the aorta at 5th and 6th decades of life.

Treatment of primary cardiac angiosarcoma in a 35 weeks pregnant woman

Cardiac angiosarcoma is a rare but highly malignant cardiac tumor. It is characterized by poor prognosis, and current treatment approaches are not effective. A 37-year-old female with 35 weeks pregnancy experienced chest tightness and shortness of breath for 1 month. She was diagnosed with primary cardiac angiosarcoma. Delivery of fetus was performed early to treat the mother. The patient underwent resection of the tumor then she was treated with chemotherapy. However, the tumor recurred 11 months after surgery. Angiosarcoma is a highly malignant tumor explaining recurrence of the tumor recurred after surgery. Cardiac angiosarcoma should be treated through a comprehensive treatment plan, comprising surgery, radiotherapy, and chemotherapy approaches.

Epithelioid angiosarcoma of the small intestine presenting with intractable gastrointestinal bleeding: An uncommon tumor with diagnostic challenge and poor prognosis

Introduction: Angiosarcoma of the gastrointestinal tract is a rare, aggressive malignant neoplasm of vascular origin. Commonly, this tumor occurs due to metastases from other sites like the head and neck region and the soft tissue. When occurring as a primary tumor of the intestines, this entity poses a diagnostic dilemma due to its non-specific clinical presentation, coupled to its infiltrative features that may occur without an obvious lesion amenable to routine gastrointestinal interventions. The findings from an autopsy and surgical case with a brief review of its diagnostic features and relevant literature are discussed.Methods: We present a 73-year-old male with a history of a recent onset of gastrointestinal bleeding who was referred to our facility for persisting bleeding. He underwent endoscopy without an identifiable source of bleeding and subsequently received a colectomy at our facility, with repeated blood transfusions for persisting gastrointestinal bleeding. He was eventually transitioned to hospice and died shortly after presentation. A complete autopsy was performed.Results: There was anasarca with hemoperitoneum and intestinal intraluminal blood. Present in the small intestinal mucosa were three hemorrhagic nodules. Histologically, the nodules demonstrated medium to large epithelioid vasoformative neoplastic cells infiltrating through the wall of the small intestine and extending to the omentum. The findings from the gross and histology examination, as well as the immunohistochemical stains were consistent with an epithelioid angiosarcoma.Conclusion: Primary epithelioid angiosarcoma of the intestine is an uncommon aggressive tumor that poses a diagnostic challenge. Although rare, this diagnosis needs to be considered in patients with intractable gastrointestinal bleeding. Due to its morphologic overlap with other benign and malignant neoplasms, the use of ancillary testing during histologic examination is key in arriving at an accurate diagnosis.

Multimodality imaging for diagnosis in primary cardiac angiosarcoma

Multimodality imaging for diagnosis in primary cardiac angiosarcoma

Survival outcomes in primary angiosarcoma of the head and neck: A systematic review and meta-analysis.

e18039 Background: Angiosarcoma of the head and neck (ASHN) is a rare entity and confers substantial morbidity and mortality. Yet, the optimal management of ASHN remains unclear. This study aimed to describe the epidemiology of ASHN and to identify the most favorable treatment approach. Methods: We performed a systematic review based on the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines, using the PubMed (Medline), EMBASE, and Cochrane Library databases, queried from 1990 until present. Articles in the English language reporting on survival outcomes of adult primary ASHN treated with curative-intent, were included. All estimates were weighted based on sample size. Analysis of variance (ANOVA) and two-sample t-tests were used as appropriate. This study was registered with PROSPERO, CRD42021220970. Results: A total of 3652 studies were identified, with 14 articles reporting on 2265 ASHN patients, meeting inclusion criteria. Mean ± SD age was 70.6 ± 7.7 years with 1621 (66.6%) men and 812 (33.4%) women. ASHN involved the scalp (n = 176, 57.9%) and the face (n = 128, 42.1%). 249 patients had early stage I-II disease (39.6%) whereas 379 had late stage III-IV disease (60.4%). Most (n = 529, 45.6%) received surgery and radiotherapy (RT), 305 (26.3%) received surgery alone, 210 (18.1%) received definitive RT/chemoradiotherapy (CRT), 75 (6.5%) received surgery and CRT, and 33 (2.8%) received surgery and chemotherapy. Negative margins were achieved in 471 (55.9%) whereas 371 (44.1%) had positive margins. Mean ± SD follow-up was 41.7 ± 15.4 months. Weighted mean, 1-, 5-, and 10-year overall survival (OS) were 26.9 months, 67.3%, 30.6%, and 20.8% respectively. Mean and 5-year disease-specific survival (DSS) were 72.9 months and 50.3% respectively. Mean ± SD local recurrence rate (LRR) was 32.1 ± 11.7%. Median RT dose delivered was 60 Gy (interquartile range: 60-70). Patients who received surgery had a significantly higher mean OS (34.9 vs. 18.7 months, P = 0.04) and 5-year OS (30.1 vs. 14.2%, P = 0.01) compared with those who did not receive surgery. There was no significant difference in mean OS for receiving adjuvant chemotherapy (P = 0.99) or RT (P = 0.51). Conclusions: In the largest ASHN study to date, definitive surgical resection was associated with an improvement in OS. Multimodality treatment did not confer an OS benefit. Randomized trials are needed to establish the optimal treatment approach for ASHN.

Thyroid Angiosarcoma Complicated by Neck Hematoma

Abstract Background: Primary angiosarcoma of the thyroid is a rare, aggressive thyroid malignancy most typically found in the Alpine regions of Europe. We present a case of non-Alpine thyroid angiosarcoma complicated by recurrent surgical bed hematoma. Case: An 81-year-old Albanian man presented with two months of dyspnea and hemoptysis and was found to have a large thyroid mass, multiple subcentimeter pulmonary nodules, and a left pleural effusion seen on CTA of the chest. He had a suppressed TSH &amp;lt; 0.008 uIU/mL (n 0.400-4.200 uIU/mL) on presentation with a normal free T4 (1.36 ng/dL; n 0.80-1.50 ng/dL) and total T3 (107 ng/dL; n 87-178 ng/dL). Labs from 3 years prior were consistent with prior subclinical hyperthyroidism: TSH 0.28 uIU/mL, free T4 1.35 ng/dL, and free T3 3.98 pg/mL (n 2-4.4 pg/mL). A thyroid ultrasound was performed and showed multiple bilateral thyroid nodules, the largest of which was 6.0 x 7.8 x 5.4 cm in the right lower pole. He had no compressive symptoms and was clinically euthyroid. FNA of the nodule was non-diagnostic and cytology of the pleural fluid was negative for malignancy. He underwent a bronchoscopy which showed blood clots without endobronchial lesions, VATS with pleural biopsy, and right hemithyroidectomy. Pathology showed high grade epithelioid malignancy arranged in solid, variably discohesive nests and sheets, associated with vague vasoformative features and hemorrhage. Immunostains were diffusely and strongly positive for ERG as well as CD31 and AE1/3, but negative for PAX-8, TTF-1, and thyroglobulin, supporting a diagnosis of angiosarcoma. The remainder of the thyroid tissue was non-neoplastic with multinodular hyperplasia. After initial discharge, he re-presented on post-op day 10 with bleeding from the surgical site. CT of the neck showed extensive hematoma (10.3 x 5.0 x 8.7 cm) and he was taken back to the OR for evacuation. Residual tumor tissue found along the R carotid artery was determined to be the culprit. Over the next month the patient remained admitted for supportive transfusions, and palliative radiation to the pulmonary nodules and the residual tumor was initiated. His hospitalization was complicated by a proximal lower extremity DVT, which was treated with an IVC filter. Repeat chest imaging at six weeks showed new and progressive pulmonary nodules. Upon completion of 3 weeks of RT the patient was discharged debilitated, fatigued, and using supplemental oxygen. His family elected to pursue hospice care. Conclusion: This case represents a rare, aggressive malignancy complicated by extrathyroidal extension and recurrent hematoma resulting in severe morbidity and precluding further systemic interventions. These highly vascular tumors likely present an elevated risk for postoperative hematoma which clinicians should recognize.

Primary Angiosarcoma of Breast – A Rare Case Presentation

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

Primary Angiosarcoma of Breast – A Rare Case Presentation

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

Primary angiosarcoma of the spleen, a rare indication for splenectomy: a case report.

Introduction and importancePrimary angiosarcoma of the spleen is a rare condition with a nonspecific clinical presentation and is associated with a poor prognosis. We describe two patients with primary splenic angiosarcoma successfully treated with splenectomy and adjuvant chemotherapy.Case presentationsCase 1: A 50-year-old female presented with fatigue and left-sided rib, shoulder, and abdominal pain. A CT scan demonstrated a large splenic mass, and biopsy was diagnostic of angiosarcoma. An open en bloc resection of the spleen was performed, and pathologic examination confirmed high-grade angiosarcoma; the surgical margins were negative. The patient received pegylated liposomal doxorubicin (PLD) and ifosfamide; she demonstrated no evidence of recurrence with four years of follow-up. Case 2: A 70-year-old male presented with acute back pain. A CT scan demonstrated a splenic mass; biopsy was diagnostic of angiosarcoma. The patient underwent open splenectomy, and pathology revealed high-grade angiosarcoma; the surgical margins were positive. The patient received PLD and ifosfamide but presented three years later with metastatic tumor to the spine. The patient had a favorable tumor response to pembrolizumab. The patient's tumor burden remains stable at 5 years following splenectomy.Clinical discussionAngiosarcoma of the spleen is a rare clinical entity and is often challenging to diagnose early. Moratality is high, especially in the case of metastasis or spontaneous rupture.ConclusionDue to the rare nature of this tumor, optimal treatment is not known. Here, we show excellent response in two patients to surgery combined with adjuvant therapy.

Primary breast angiosarcoma: a case report

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

Clinical and imaging characteristics of patients with primary cardiac angiosarcoma

Objective: To investigate the clinical, cardiac imaging characteristics and prognosis of patients with primary cardiac angiosarcoma. Methods: The clinical data of 14 patients hospitalized with primary cardiac angiosarcoma from January 2001 to December 2017 in Peking Union Medical College Hospital were collected and analyzed. Metastatic cardiac angiosarcoma was not included in this study. Patients were followed up post discharge per telephone call or clinical visit. Results: Of the 14 patients, 8 were males and 6 were females, average age was 48 years. The main clinical symptoms were shortness of breath (8/14), hemoptysis (6/14), fever (5/14), chest pain (4/14) and cough (3/14). Imaging examinations showed that the tumors of 8 patients were located in the right heart and 6 in the pericardial cavity. Tumors in the right heart often infiltrate the atrial wall and cause pericardial effusion (7/8). Tumors in the pericardium were characterized by recurrent bloody pericardial effusion (6/6), prone to progressive constrictive pericarditis (3/6), pericardial fluid cytology was often negative (6/6). MRI showed heterogeneous high signal intensity (cauliflower aspect) on T2-weighted image and heterogeneous enhancement with a"sunray" aspect at the perfusion study. At the time of diagnosis, 8 patients developed lung or adrenal metastasis (8/14). The median survival was only 305 days. Conclusions: Primary cardiac angiosarcoma is a rare disease with non-specific clinical manifestation and poor prognosis. Imaging examinations may help diagnosis. The high invasiveness and the easy-to-metastasis feature of the tumor contribute to the poor prognosis of cardiac angiosarcoma.

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

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

Clinical characteristics and surgical treatments of primary hepatic angiosarcoma

PurposePrimary hepatic angiosarcoma is a very rare and highly malignant tumor with poor prognosis. It is difficult to diagnose because of the lack of typical clinical features, and the treatment protocols for PHA are also not clear. Therefore, this study wants to find out the clinical characteristics and surgical treatments of primary hepatic angiosarcoma.MethodsAmong 8990 patients diagnosed with primary malignant tumor of the liver from January 2000 to December 2019 in our hospital, only four patients were diagnosed with primary hepatic angiosarcoma. The demographics, clinical manifestation, past history, serology test results, MRI features, pathology, treatment modality and prognosis of four patients were collected and analyzed.ResultsThree of four patients had no clinical symptoms, while one patient's symptom was abdominal pain. The levels of tumor markers of all four patients were within the normal reference range and serological tests were negative for hepatitis B and C virus. The MRI imaging findings of all four patients were mixed mass with highly disordered vascular characteristics. All four patients were misdiagnosed preoperatively. One patient who underwent hepatic lobectomy was still alive for about 18 months after surgery. One patient who underwent hepatic lobectomy has survived for only 6 months due to severe pneumonia. The other two patients who received transarterial chemoembolization survived 16 months and 11 months respectively.ConclusionThe clinical symptoms of primary hepatic angiosarcoma are not typical, and primary hepatic angiosarcoma is easily misdiagnosed. The typical imaging manifestations are structural disorder and heterogeneous tumor. Hepatic lobectomy and transarterial chemoembolization may be important surgical treatments to improve the prognosis of patients.

Solitary Subcutaneous Tissue Metastasis as Recurrence in a Case of Primary Angiosarcoma of Breast: Findings on 18F-Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography.

Primary angiosarcomas of the breast are rare tumors, with a fatal outcome. We present a rare case of an operated primary angiosarcoma of the right breast in a 20-year-old female who showed disease recurrence in the right posterior arm subcutaneous tissue on 18F-fluorodeoxyglucose positron emission tomography-computed tomography after 1 year of surgery without any other visceral metastasis.

Primary Epithelioid Angiosarcoma of the Thyroid: Long-Term Survival Case Report and Literature Review

Thyroid angiosarcoma is a rare neoplasm of debated clinical management. In addition, the epithelioid variant poses microscopic challenges due to its close resemblance to poorly differentiated carcinoma. Only 63 cases of thyroid angiosarcoma have been published in English literature. Due to its rarity, the limited clinical experience can make its management complex. We report a new case of primary thyroid angiosarcoma showing preponderant epithelioid features characterized by an exceptional long survival. We also provide a discussion on the role of radiotherapy in tumor treatment and a brief review of the pertinent literature.

Resection of primary cardiac angiosarcoma infiltrating the right atrioventricular junction and tricuspid valve: a case report

BackgroundPrimary cardiac tumours are extremely rare. Most of them are benign. Sarcomas account for 95% of the malignant tumours. Prognosis of primary cardiac angiosarcoma remains poor. Complete surgical resection is oftentimes hampered when there is extensive tumour involvement into important cardiac apparatus. We report a case of cardiac angiosarcoma of the right atrium and ventricle, infiltrating the right atrioventricular junction and tricuspid valve.Case presentationInitially, a 22-year-old man presented with dyspnoea. One year later, he had recurrent pericardial effusion. Afterwards, echocardiography revealed a large mass in the right atrium, expanding from the roof of the right atrium to the tricuspid valve. The mass was causing compression on the tricuspid valve, and another mass was seen in the right ventricle. Complete resection of the tumour was impossible. The mass was resected with the biggest possible margins. The right atrium was reconstructed using heterologous pericardium. The patient’s postoperative course was uneventful. Postoperative echocardiography showed a small mass remaining in the right side of the heart. Histopathology and immunohistochemistry confirmed the diagnosis of angiosarcoma. The patient underwent adjuvant chemotherapy and radiotherapy later on. He survived for 1 year and 5 days after the surgery. After a diagnosis of lung and brain metastases, he ended up on mechanical ventilation for 48 h and died.ConclusionsSurgical resection combined with postoperative chemotherapy and radiotherapy is feasible even in patients with an advanced stage of cardiac angiosarcoma when it is impossible to perform complete surgical resection.

Primary breast angiosarcoma with disseminated intravascular coagulation is successfully treated with self‑subcutaneous unfractionated heparin calcium injection: A case report

Angiosarcoma is a rare sarcoma with a poor prognosis and is prone to disseminated intravascular coagulation (DIC), where DIC often interferes with chemotherapy. Primary angiosarcoma of the breast (PASB) is a type of angiosarcoma that is located in mammary parenchyma and is not associated with radiation exposure. The current study reported a 47-year-old female with DIC associated with PASB. The DIC of the patient relapsed during mono-chemotherapy with paclitaxel (PTX) after first-line anticoagulant therapy using thrombomodulin-α. The second-line danaparoid sodium therapy, followed by self-subcutaneous injection of unfractionated heparin calcium (UFH), resulted in long-term stabilization of DIC. Under this second-line anticoagulant therapy, the patient continued chemotherapy and chemoradiotherapy for >13 months in the outpatient setting without impairment of quality of life. The present case suggested that self-subcutaneous injections of UFH may be a useful therapeutic option for long-term control of DIC associated with PASB. However, further prospective clinical trails are needed to verify the efficacy of self-subcutaneous injection of UFH in similar settings.

COVID-19 infection and cardiac angiosarcoma: a dangerous combination\u2014a case report

BackgroundThe COVID-19 pandemic has strained all medical systems, especially in countries like Ecuador, where health services were already limited. These conditions, combined with a deadly and unusual disease, like primary heart angiosarcoma, can lead to severe outcomes. Angiosarcomas represent the most common and aggressive primary malignant heart tumor; regretfully, its clinical manifestations are vague and can be easily missed. Most patients become symptomatic when there is local invasion, embolization, or metastases, leading to late diagnosis and poor survival. High clinical awareness, adequate diagnosis, and prompt treatment are vital in these rare diseases, in which time is of paramount importance.Case presentationWe report the case of a 28-year-old female who had cough, hemoptysis, and ground-glass opacities in the CT (computed tomography). Since Ecuador is in the middle of this pandemic, she was misdiagnosed and mistreated. Primary heart angiosarcoma was diagnosed, and regretfully, the patient suffered multiple complications due to diagnosis and died.ConclusionTo this day, most cardiac angiosarcomas are found in a late-stage with distal metastasis and advanced local invasion. Sadly, this tumor is frequently missed due to its incidence and broad-spectrum of clinical symptoms. Considering that its manifestations can be misleading, misdiagnosis can occur, especially in pandemic times. Therefore, knowledge of other pathologies prevents COVID-19 from overshadowing other diagnoses, hence preventing delayed diagnosis or even misdiagnosis and consequent adverse outcomes for patients.