Fine Needle Aspiration Cytology Diagnosis of Angiosarcoma of the Rib bone. Report of a Case and a Brief Review of the Literature (original) (raw)
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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.
American Journal of Clinical Oncology, 2014
BACKGROUND-Angiosarcoma of bone is a rare high-grade malignant vascular tumor. The literature regarding treatment and outcome of patients with this tumor is limited. We performed a two-institutional retrospective study to analyze treatment and survival of patients with angiosarcoma of bone. PATIENTS AND METHODS-We reviewed patients with the histological diagnosis of primary angiosarcoma of bone treated from 1980 to 2009. Demographic details, histology, treatment and survival were reviewed. RESULTS-38 men, 22 women (median age 54 years). Most lesions occurred in the femur and the pelvis. Metastatic disease at presentation was diagnosed in 24 patients (40%). Forty-three patients underwent surgery, with 30 of them achieving surgical complete remission (SCR). Radiotherapy (RT) was applied to 17 patients, and chemotherapy (CT) to 13/35 and 15/22 patients with localized and metastatic disease, respectively. The 5-year overall survival (OS) was 20%: 33% for patients with localized disease and 0% for metastatic patients. Higher 5-year OS was reported for patients who achieved SCR (46%) than for
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
Multicentric epithelioid angiosarcoma of the spine: a case report of a rare bone tumor
Spine Journal, 2007
BACKGROUND CONTEXT: Epithelioid angiosarcoma (EA) is a high-grade sarcoma of vascular origin. EA is a rare variant of angiosarcoma. It is characterized by large cells with an epethelioid morphology. EA has been reported in sites like skin thyroid, adrenal gland, soft tissue, and rarely in bone. PURPOSE: The current article describes a case of multicentric EA of the spine that presented simultaneously at T4 spine and the posterior one third of the left fourth rib. STUDY DESIGN/SETTINGS: This study included a patient with a rare spinal tumor, which presented insidiously. Radiologically, the lesions were osteolytic with erosion of cortices. The tumor was composed of nests and cords of malignant cells with epithelioid morphology with areas of vascular differentiation, necrosis, and hemorrhage. Anastomosing vascular spaces lined by epithelioid endothelial cells suggested focal endotheliod differentiation. METHODS: Histopathological evaluation of the tumor obtained from en bloc resection of the fourth rib and specimen from decompression of the fourth thoracic vertebra was done. Immunohistochemistry showed positivity for endothelioid cell markers like CD31 and factor VIII related antigen. The authors point out the need for immunohistochemical evaluation after careful histological analysis for vascular differentiation for an accurate diagnosis of vascular bone tumors with epithelioid features so that an erroneous diagnosis of metastatic carcinoma can be avoided. EA occurs rarely in bone. EA is marked by the presence of large polygonal epithelioid malignant cells with marked cellular atypia and pleomorphism. The arrangement of the cells may mimic epithelial neoplasm. These types of lesions are a definite diagnostic challenge in bone biopsy. The tumor was treated with en bloc resection of the rib and decompression of the body of T4 spine, followed by megavoltage radiotherapy. Follow-up X-rays showed regression of the tumor at 2 years. CD31 is the most sensitive marker for EA. Even in the absence of obvious vascular differentiation, abundant intratumoral hemorrhage and intratumoral neutrophils are definite morphologic changes that should suggest a vascular origin. RESULTS: The patient was followed up for 2 years. A conservative surgical approach with a radical course of megavoltage radiotherapy could yield a good result in EA of bone. At the time of writing this report, there is no evidence of tumor recurrence. CONCLUSION: It is important to differentiate EA from other tumors because the clinical course and treatment are specific for these conditions. Careful histologic and immunohistochemical analysis will clinch the diagnosis. Even though rare, we stress the importance to be aware of the existence of this tumor, which is essential for correct diagnosis. Ó
Cytologic features of angiosarcoma: A review of 26 cases diagnosed on FNA
Cancer Cytopathology, 2016
BACKGROUND: This study describes the cytologic features of 26 angiosarcomas diagnosed on fine-needle aspiration. METHODS: Twenty-six angiosarcomas from 20 patients were confirmed by cytomorphology and immunocytochemical (immunohistochemistry) positivity for at least 2 of 3 vascular markers. Specimens were examined for spindled/epithelioid/plasmacytoid single cells, 3-dimensional clusters, multiple prominent/bar-shaped nucleoli (5 times longer than their width), chromatin strands, abnormal mitoses, necrosis, and vasoformative features. RESULTS: Eight males and 12 females with a mean age of 52 years (range, 2-94 years) underwent aspiration of tumors in the following: soft tissue or skin/subcutis (n 5 10), bone (n 5 4), nodes (n 5 5), lung (n 5 2), liver (n 5 2), heart (n 5 1), parotid gland (n 5 1), and pleural fluid (n 5 1). An angiosarcoma diagnosis was rendered for 24 of the 26 cases (92%); 1 was diagnosed as "atypical cells, cannot exclude angiosarcoma," and another was diagnosed as a malignant vascular neoplasm. Abnormal mitoses were most frequent (85%), and they were followed by single malignant cells (81%: epithelioid [69%], spindled [62%], and plasmacytoid [19%]), 3-dimensional clusters (54%), multiple prominent (62%) or bar-shaped nucleoli (54%), and chromatin strands (31%). Vasoformative features, including hemophagocytosis (54%), cytoplasmic lumina/vacuoles (69%) containing red blood cells (54%)/neutrophils (31%), and endothelial wrapping (69%), were seen in 88%; 23% had all vasoformative features, 88% had at least 1, and 12% had none. CONCLUSIONS: Angiosarcomas show a range of cytomorphologic features that make them potentially recognizable on cytology. Although vasoformative features are highly suggestive, they are not specific for angiosarcoma and may be seen in some nonvascular neoplasms. Immunohistochemistry and a high index of suspicion are required for an accurate diagnosis.
Angiosarcoma—Report of an unusual case
British Journal of Plastic Surgery, 1974
ANGIOSARCOMAS are relatively rare malignant tumours of endothelial origin which may be divided histogenetically into haemangiosarcomas and lymphangiosarcomas; lymphangiosarcomas are angiosarcomas developing in post-mastectomy lymphoedematous arms (Stewart and Treves, 1948; Treswell et al., 1962). Angiosarcomas arising primarily on the extremities of young adults have been described (McCarthy and Pack, 1950) and several authors have reported angiosarcomas in the head and neck (Reed et al., 1966; Bardwill et al., 1968). Most agree that the prognosis is poor. The patient presented here survived for 5 years and his angiosarcoma behaved as if it were multicentric in origin rather than a primary with metastases.
Medicine, 2016
Rationale: Angiosarcomas are rare, malignant vascular tumors. Patient concerns: They represents about 2% of all soft tissue sarcoma, which can often metastasize through the hematogenous route. The radiological features have been analyzed in 4 patients with metastatic angiosarcoma in the chest. Diagnoses: The main radiologic findings included nodules, cysts, nodules with halo sign, and vascular tree-in-bud. Morphologic features, as observed in the histologic specimen, have been correlated with radiologic appearance. Lessons: Metastatic angiosarcomas to the lung are characterized by a wide variety of radiologic appearances that can be very characteristic. Computed tomographic findings observed include bilateral solid nodules, cystic, and bullous lesions sometimes associated with spontaneous hemopneumothoraces. Abbreviations: AE1/AE3, CD31, CD34, CT = computed tomography, CVD = collagen vascular disease, H&E = hematoxylin and eosin, PE = pulmonary embolism, PET-CT = positron emission tomographycomputed tomography.