Primary Angiosarcoma of Bone (original) (raw)
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Angiosarcoma of bone: a retrospective study of the European Musculoskeletal Oncology Society (EMSOS)
Scientific Reports
Angiosarcoma of bone (B-AS) is a rare malignant tumor of vascular origin. the aim of this retrospective study is to report on treatments and prognosis. Data were collected from the EMSOS website. 80 patients in 9 centers included: 51 male/29 female; median age 54 years (range 17 to 92); 56% with localized disease, 44% metastatic. Primary tumor surgery: 76% (30% amputation, 26% intralesional margins); radiotherapy (RT): 41%; chemotherapy (CT): 47% (56% in metastatic, 41% in localized cases). With a median follow-up of 31 months (range 40 to 309), 5-year overall survival (OS) was 27% (95%CI 16-30): 41% (95%CI 25-56) for localized patients, and 8% (95%CI 0-20) for metastatic (p = 0.002). In metastatic patients, 1 year OS was significantly influenced by chemotherapy response: 67% (95CI% 29-100) for those who responded or had stable disease (n = 7), and 18% (95CI% 0-41) for patients with progressive disease (n = 11), p 0.002. The surgical complete remission (SCR) status was pivotal in localized patients (5-year OS 45% for SCR, 17% no SCR, p = 0.03); also 5-year OS was significantly influenced by age and site of the tumor. After multivariate analysis, the addition of radiotherapy to surgery significantly influenced the disease-free survival (DFS) rate, whereas the use of chemotherapy lost the significance showed at the univariate analysis. Overall, patients with metastatic B-AS have a dismal prognosis, with a prolonged survival in case with a response to chemotherapy. experimental trials with more active systemic treatment regimens are needed. in patients with localized disease, the patient's age and site of the tumor are prognostic factors and any effort must be made to achieve an SCR status. No definitive conclusions can be drawn from our data on the use of adjuvant chemotherapy, while the use of adjuvant radiotherapy might improve DSf in patients surgically free of disease. Angiosarcoma of bone (B-AS) is exceedingly rare, accounting for less than 1% of all primary bone sarcomas, with the highest incidence between 50 and 70 years of age 1,4. The diagnosis of B-AS is challenging and represents the malignant end of the spectrum of CD31/ERG positive vascular tumors, including hemangiomas, hemangioendotheliomas, well-differentiated and poorly differentiated angiosarcomas 1-3 (Fig. 1). The disease might present as unifocal or with multifocal bone lesions, and it is usually associated with a poor prognosis 1-6. Given the rarity of B-AS, only scant information can be found in the literature, mostly reported in form of small series and case reports. No specific treatment guidelines or position papers are available, so the treatment of choice is based on data from other types of bone and soft tissue sarcomas 1,3-7. The role of chemotherapy and prognostic factors for
Distinct histological features characterize primary angiosarcoma of bone
Histopathology, 2011
Aims: To define the histological criteria of primary angiosarcoma of bone. Methods and results: Forty-two angiosarcomas of bone in 23 males and 15 females were studied. Histological criteria were related to patients' outcome. Eleven patients had multifocal lesions. Lesions were located in the long and short tubular bones followed by the pelvis, spine and trunk. Tumour cells were positive for CD31 in 38 of 40, von Willebrand Factor in 21 of 35, CD34 in 15 of 38, smooth muscle actin in 22 of 36, D2-40 in 11 of 35 and keratinAE1AE3 in 27 of 39. Thirty-nine tumours showed an epithelioid phenotype.
International Seminars in Surgical Oncology, 2008
Angiosarcoma of bone is an exceedingly rare primary bone malignancy that can present as an aggressive osteolytic lesion. Histological diagnosis can be extremely challenging, as the pathological features often resemble that of aneurysmal bone cysts. We report an interesting and peculiar case of an intraosseous angiosarcoma that presented as a diagnostic dilemma and discuss the relevant radiological and pathologic findings.
Skeletal Radiology, 2012
The classification of vascular bone tumors remains challenging, with considerable morphological overlap spanning across benign to malignant categories. The vast majority of both benign and malignant vascular tumors are readily diagnosed based on their characteristic histological features, such as the formation of vascular spaces and the expression of endothelial markers. However, some vascular tumors have atypical histological features, such as a solid growth pattern, epithelioid change, or spindle cell morphology, which complicates their diagnosis. Pathologically, these tumors are remarkably similar, which makes differentiating them from each other very difficult. For this rare subset of vascular bone tumors, there remains considerable controversy with regard to the terminology and the classification that should be used. Moreover, one of the most confusing issues related to vascular bone tumors is the myriad of names that are used to describe them. Because the clinical behavior and, consequently, treatment and prognosis of vascular bone tumors can vary significantly, it is important to effectively and accurately distinguish them from each other. Upon review of the nomenclature and the characteristic clinicopathological, radiographic and genetic features of vascular bone tumors, we propose a classification scheme that includes hemangioma, hemangioendothelioma, angiosarcoma, and their epithelioid variants.
Rare Tumors, 2013
Multicentric primary angiosarcoma of bone has been described as a distinct entity from bone metastases from angiosarcoma. Bone angiosarcoma accounts for less than 1% of sarcomas. It has dismal prognosis overall, but the multicentric expression does not confer worse prognosis. We describe the case of an old male with bone angiosarcoma of the extremities with multicentric presentation. He soon after had soft tissue angiosarcoma of the head and neck. Histology and immunohistochemistry were consistent with the diagnosis of highgrade angiosarcoma. Comparative genomic hybridization on paraffin-embedded samples of the bone and head and neck samples suggested additional abnormalities in the bone fragment, thus suggesting than bone lesions were indeed metastatic from his head and neck angiosarcoma; although these preliminary analyses warrant confirmation in other similar rare cases. The patient died after 3 years of relapsed acute leukemia with progressive angiosarcoma.
2022
Angiosarcoma is a rare malignant neoplasm arising from lymphatic or vascular endothelial cells with an annual incidence of approximately 2 in 1,000,000. Angiosarcomas are commonly found in the skin, soft tissue, bone and viscera. Angiosarcomas are usually metastatic at presentation with an average survival of 6-16 months. The risk factors associated with developing angiosarcoma are radiation exposure, longstanding lymphoedema, environmental carcinogens and genetic syndromes. Angiosarcoma present with non-specific findings making it difficult to diagnose. Imaging studies may aid in diagnosis of angiosarcoma however histology is required for definitive diagnosis. Histologically, angiosarcoma appears as spindled, polygonal, epithelioid and primitive round cells staining positively for vascular and endothelial antigens such as CD31, CD34 and VEGF. Angiosarcoma can be treated with surgery, radiotherapy, chemotherapy, and targeted immunotherapy. We present a case of 62-year-old man diagno...
Primary angiosarcoma of the femur in a patient with Takayasu arteritis
Revista brasileira de ortopedia
Primary osseous angiosarcoma is a rare entity with variable biological behavior and poor prognosis. Little is known about the oncologic treatment and its etiology is still unknown. This study presents a case of lytic lesion in the right femur with dissemination to other bones, such as the vertebral column and skull, and to the lungs and central nervous system. Orthopedic surgery was performed in order to improve quality of life. Surgical specimen confirmed the diagnosis of high-grade malignant osseous angiosarcoma. Despite oncologic and orthopedic treatment, the patient had rapid and aggressive progression with a poor outcome.
Human Pathology, 1971
Primary tumors of bone of vascular origin are rare. Consequently, knowledge of their clinical and pathologic features is still scant)'. A morass of ntisleading terminolog-y, particularly in connection with the malignant vascular tumors, has added to the difficulties. Tiffs paper is a review of our current knowledge of these lesions based on a series of 42 personally studied cases. It presents a classification of the vascular ttmtors of bone and its coverings and attempts to sort out in an orderly way tim confusing array of terms applied to the ntalignant bone tumors of blood vessel origin.