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

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.

Primary angiosarcoma of aorta: A systematic review

Purpose: To elucidate the epidemiology, anatomical, presentation, classification, pathology, investigative modalities, management and prognosis of primary angiosarcoma of the aorta. Material and Methods: 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. Results: 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], 7–1560 days vs. 180 (IQR, 1–5730 days)], respectively. The overall median survival was 210 days (IQR, 1–5730 days) or 7 months. Lack of metastasis (47%) was a marker of longer survival ( p < 0.03) irrespective of other attributes. Conclusion: 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.

Primary Pericardial Angiosarcoma: A Case Report and Literature Review

Primary angiosarcoma is extremely rare malignant tumor that has no typical symptoms and progress rapidly with poor prognosis. It is mesenchymal in origin and observed most frequently in the right atrium, cases in the pericardium is much more rare. Only few can detected in the early-stage allowing complete radical resection with a mean survival of 3 months to 1 year. There is few pericardial angiosarcoma reported among these years. The present study reports a case of a 44-year-old woman with primary pericardial angiosarcoma, who underwent a wide range of imaging methods, including transthoracic echocardiography, contrast-enhanced computed tomography (CT) and positron emission tomography-magnetic resonance imaging (PET-MRI). The patient recovered well after operation in two years and died due to the recrudescence and pulmonary metastases in April, 2020. We report the case for its rarity and revealing the early detection of primary pericardial angiosarcoma with imaging examinations is critical for prognosis. Finally a literature review is done.

Primary Angiosarcoma of the Adrenal Gland: Report of 2 Cases and Review of the Literature

Primary adrenal angiosarcoma is a rare, malignant, vascular neoplasm. These neoplasms typically arise in middle age (median age of 60 years) and are more common in males (65%) than in females. Although rare, these neoplasms are aggressive with a propensity for local recurrence and metastasis and a median survival of 18 months. We present 2 cases of primary adrenal angiosarcoma with synchronous, ipsilateral adrenocortical adenomas. We review the cases of adrenal angiosarcoma reported since 1988 and discuss their clinical and histopathologic characteristics.

Thyroid Angiosarcoma Complicated by Neck Hematoma

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 &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.