Primary and secondary cutaneous angiosarcoma: Distinctive clinical,pathological and molecular features |
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Affiliation: | 1. Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, 8700 Beverly Boulevard, Room 8612, Los Angeles, CA 90048, USA;2. Department of Pathology, Cleveland Clinic Lerner College of Medicine, Cleveland Clinic, 9500 Euclid Avenue, L25, Cleveland, OH 44195, USA;1. Department of Pathology, Langone Medical Center, New York University, 160 East 34th Street, New York, NY 10016, USA;2. Robert J. Tomsich Pathology & Laboratory Medicine Institute, Cleveland Clinic and Lerner Research Institute, Department of Pathology, 9500 Euclid Avenue L25, Cleveland, OH 44195, USA;1. Department of Radiation Oncology, Atlanta, GA, USA;2. Department of Biostatistics and Bioinformatics, Atlanta, GA, USA;3. Department of Medical Oncology, Atlanta, GA, USA;4. Department of Orthopedic Oncology, Atlanta, GA, USA;5. Department of Radiation Oncologyand Surgery, Atlanta, GA, USA;6. Department of Radiation Oncology of the Winship Cancer Institute of Emory University, Atlanta, GA, USA;7. Department of Therapeutic Radiology of the Yale School of Medicine, New Haven, CT, USA;2. Department of Radiation Oncology, Washington University School of Medicine, Saint Louis, Missouri, USA;3. Division of Medical Oncology, Washington University School of Medicine, Saint Louis, Missouri, USA;4. Department of Pathology, Washington University School of Medicine, Saint Louis, Missouri, USA;1. Plastic and reconstructive surgery unit, Moulay Ismaïl Military Hospital, Nehrou St, Meknes 50000, Morocco;2. Department of Oral and Maxillofacial Surgery, Dalhousie University, Halifax, Nova Scotia, Canada |
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Abstract: | Angiosarcomas are ubiquitous neoplasms involving both cutaneous and soft tissue and visceral locations. Accumulating biomolecular evidences suggest that cutaneous angiosarcomas are distinctive entities with molecular, clinical and pathological peculiarities. Despite several ongoing clinical trials with promising therapeutic agents, the prognosis of cutaneous angiosarcomas is dismal and survival still rely on early diagnosis and surgery. An accurate diagnosis and the knowledge of the underlying molecular landscape are therefore essential to improve the prognosis. We detail the molecular, clinical, dermoscopic, morphological and prognostic features of cutaneous angiosarcoma. Although the molecular landscape of cutaneous angiosarcoma is not completely understood, accumulating evidences suggest that there are characteristic molecular alterations including dysregulation of angiogenesis and several complex molecular pathways. Secondary cutaneous angiosarcomas, arising in correlation with chronic lymphedema and ionizing radiation, have different molecular hallmarks, which are also leading to the first diagnostic applications. The diagnosis of cutaneous angiosarcoma may be challenging, as well-differentiated forms can be hard to distinguish from benign and low-grade vascular neoplasms, while poorly differentiated forms can be easily confounded with other non-vascular high-grade neoplasms. An accurate and early diagnosis, which is mandatory to ensure the best survival for the patients, is mainly based on morphological hallmarks. |
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