Angiosarcoma Of Bone Research Articles

Metal Associated Angiosarcoma of the Distal Femur

Malignant vascular tumors (angiosarcoma) of bone are rare and represent a less than 1% of malignant bone tumors. The authors report a case of a patient with angiosarcoma misdiagnosed as infection;which developed adjacent to a stainless steel intra-medullary nail used for fixation of a femur fracture.

Epithelioid angiosarcoma of bone and soft tissue: A report of seven cases with emphasis on morphologic diversity, immunohistochemical features and clinical outcome

Epithelioid angiosarcoma is a rare histopathologic variant of angiosarcoma characterized by an epithelioid morphology. This subset can histologically mimic non-vascular neoplasms and impose serious challenges in reaching a correct diagnosis, especially in the context of limited tissue sampling (e.g., needle core biopsy). To improve recognition of epithelioid angiosarcoma - and the spectrum of morphologic diversity associated with this rare variant - and to avoid a misdiagnosis, we describe the clinical, histopathologic, and immunohistochemical findings of cases of epithelioid angiosarcoma diagnosed at our institution. Seven cases of epithelioid angiosarcoma with appropriate pathologic material were identified from our archives. Immunohistochemistry was used to detect the expression of CD31, CD34, Factor VIII, cytokeratin, epithelial membrane antigen, vimentin, HMB45, CD1a, CD68, lysozyme, CD45, desmin, and smooth muscle actin in all cases. Follow-up information was obtained by reviewing medical records or by direct communication with family members. The lesions involved the bone (n = 4) and soft tissues (n = 3). Microscopically, all tumors had a predominantly diffuse growth pattern, with a focal nested architecture in 6 cases, which closely mimicked metastatic carcinoma. The initial biopsy was performed in 2 of 6 patients and revealed the presence of a malignant neoplasm suggestive of metastatic carcinoma. Immunohistochemically, the epithelioid endothelial cells usually showed strong reactivity for CD31 (7/7), variable or focal positive staining for CD34 (5/7), Factor VIII (4/7), cytokeratin (6/7), epithelial membrane antigen (2/7), vimentin (7/7), and CD68 (3/7). In contrast, they were negative for CD1a, HMB45, lysozyme, CD45, desmin, and smooth muscle actin. Three patients died of disease within one year of the diagnosis, 2 patients developed local recurrence or metastases, and another 2 were disease-free at this writing. With any unusual epithelioid neoplasm displaying some or all of the morphologic features described above, epithelioid angiosarcoma should be included in the differential diagnosis. In such an instance, endothelial markers should be incorporated in the immunohistochemical analysis to avoid misdiagnosis, particularly with limited sampling.

Distinct histological features characterize primary angiosarcoma of bone

To define the histological criteria of primary angiosarcoma of bone. 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 keratin AE1AE3 in 27 of 39. Thirty-nine tumours showed an epithelioid phenotype. One- and 5-year survival rates were 55% and 33%, respectively. Survival analysis showed that a macronucleolus, three or more mitoses per 10 high-power field (HPF) and fewer than five eosinophilic granulocytes per 10 HPF within a tumour was associated with an even worse survival compared to the overall group. Because keratin positivity is seen in the majority of cases, pathologists should avoid misinterpretation as metastatic carcinoma. A macronucleolus, three or more mitoses per 10 HPF and fewer than five eosinophilic granulocytes per 10 HPF can be used to further define angiosarcoma of bone.

Angiosarcoma epitelioide vertebral primario

Primary bone angiosarcoma is rare, and is only seen in 1% of all sarcomas. These tumours are more often found in the long bones. Primary spinal involvement has been reported in 10% of angiosarcomas. As a result of its rarity, its pathological, clinical and therapeutic characterists are largely unknown. We presnt the case of a patient with a primary vertebral epithelioid angiosarcoma in D8 which initially presented as dorsal pain and paraparesis, with the aim of contributing to a better knowlwedge of this rare process.

Primary bone angiosarcoma in a patient with Gaucher disease

Skeletal pain and the resulting skeletal complications are common in Gaucher disease. The patients therefore usually receive symptomatic treatment and only rarely undergo additional diagnostic procedures. The paper describes the case of a patient with Gaucher disease who had advancing pain in the right knee and femur, which was first attributed to the basic disease. After a pathological fracture of the painful part of the leg, it became evident that the patient suffered from primary bone angiosarcoma. From this case, we learnt that not every skeletal pain in Gaucher disease represents a skeletal manifestation of this disease. Further surgical treatment was made difficult by the thrombocyte dysfunction discovered in the patient.

Primary epithelioid angiosarcoma of bone: A case report with immunohistochemical study

Primary malignant vascular tumors of the bone are exceedingly rare and represent <1% of primary malignant bone tumors. Angiosarcoma is a malignant mesenchymal neoplasm in which the neoplastic cells demonstrate endothelial differentiation. Epithelioid angiosarcoma (EA) is a rare variant of angiosarcoma that is characterized by large cells with an epithelioid morphology. EA is an aggressive tumor with poor prognosis. Here, we present a case of a 62-year-old man who had primary EA of the left tibia. He was treated with amputation and chemotherapy. After 1 month of chemotherapy, he developed pleural effusion and died.

Primary spinal epitheliod angiosarcoma

Primary bone angiosarcoma is rare, and is only seen in 1% of all sarcomas. These tumours are more often found in the long bones. Primary spinal involvement has been reported in 10% of angiosarcomas. As a result of its rarity, its pathological, clinical and therapeutic characterists are largely unknown. We presnt the case of a patient with a primary vertebral epithelioid angiosarcoma in D8 which initially presented as dorsal pain and paraparesis, with the aim of contributing to a better knowlwedge of this rare process.

Late recurrent hemarthrosis following knee arthroplasty associated with epithelioid angiosarcoma of bone

We report a case of recurrent hemarthrosis 1 year following total knee arthroplasty in a patient with no bleeding diathesis, the hemarthrosis was found to be related to, and led to the diagnosis of high grade sarcoma of the proximal tibia. Twenty five years earlier, he sustained a lateral tibial plateau fracture and was treated with open reduction and plating. Sarcoma developing in association with a metallic orthopedic prosthesis or hardware is an uncommon, but well-recognized complication. Sarcomas that occur adjacent to orthopaedic prostheses or hardware are of varied types, but are usually osteosarcoma or malignant fibrous histiocytoma.

Cytogenetic analysis of a primary bone angiosarcoma

Primary bone angiosarcomas are rare and aggressive vascular malignancies with a high mortality rate. To our knowledge, there are no reported cytogenetic abnormalities in primary bone angiosarcomas, although several have been reported in soft tissue angiosarcomas. We report a case of primary bone angiosarcoma, arising in the tibia of a 79-year-old woman, with a unique clonal chromosomal rearrangement: t(1;14)(p21;q24), that has not been reported in either soft tissue or primary bone angiosarcoma. The biologic significance of this translocation is not clear; however, the 1p21 locus is in the region of colony stimulating factor (CSF-1), which may play a role in tumorigenesis, as has been described in pigmented villonodular synovitis and tenosynovial giant cell tumor.

Intraosseous angiosarcoma with secondary aneurysmal bone cysts presenting as an elusive diagnostic challenge

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.

Sacral epithelioid angiosarcoma associated with a bleeding diathesis and spinal epidural hematoma

Epithelioid angiosarcoma of bone is a rare, high-grade lesion that is highly vascular and can be associated with a bleeding diathesis. An association has been reported in angiosarcomas in other locations with coagulopathy from tumor-related disseminated intravascular coagulopathy and fibrinolysis. The authors report the case of a rare occurrence of a primary sacral epithelioid angiosarcoma associated with a large epidural hematoma and a severe bleeding diathesis. A 25-year-old woman presented with weakness, fatigue, neck and low-back pain, and progressive left S-1 radiculopathy. Imaging studies revealed a large ventral epidural hematoma extending from the sacral region rostrally to C-2 and a vascular tumor located in the sacrum. The patient underwent a sacral laminectomy, complicated by postoperative bleeding from the wound, and required massive transfusions. Ultimately, multimodal therapy was required to obtain hemostasis, including the use of endovascular embolization, radiation therapy, and an infusion of epsilon-aminocaproic acid with heparin. This case represents the first report of a primary epithelioid angiosarcoma in the sacrum and emphasizes that the coagulopathy seen in angiosarcoma is also a feature of this epithelioid variant.

Primary temporal bone angiosarcoma: a case report

We present a rare case of temporal bone angiosarcoma diagnosed in a 26-year-old female patient at 36 week of pregnancy. The patient was referred with a 2 months history of left otalgia and tinnitus with a tender swelling above the mastoid. Cranial imaging studies showed a 7 x 5 x 4 cm hypervascularized mass located in the left middle fossa with lysis of the temporal bone and extension to the subcutis. After the baby was delivered by caesarean section, the patient entered the oncology protocol. Selective embolization of the feeding vessels was followed by gross total surgical resection using a combined supra- and infra-tentorial approach. Pathological findings were those of a poorly differentiated, highly malignant sarcoma with a large epitheloid component and immunohistochemical evidence of endothelial differentiation (CD31, Factor VIII related antigen, CD34), consistent with an angiosarcoma with epitheloid features. No extra-cranial tumor was found after extensive staging. The patient received adjuvant radiotherapy followed by a course of chemotherapy consisting of 6 cycles of paclitaxel. At 15 months follow-up, she developed multiple distant metastasis to a left postauricular lymph node and to the lungs and ribs. The patient was given a second line chemotherapy using doxorubicine and ifosfamide. Despite an initial good response, she died with metastatic disease 26 months after diagnosis. We present a rare case of primary temporal bone angiosarcoma and report our experience with a multimode therapeutic approach combining surgery, radiotherapy and chemotherapy.

Epithelioid angiosarcoma of the bone: a series of 10 cases.

The clinical and pathologic features of 10 epithelioid angiosarcomas of bone were analyzed. There were eight males and two females who ranged in age from 26 to 83 years (mean 62 years). Four tumors were solitary and six were multifocal. In two consultation cases, the submitted diagnosis was metastatic carcinoma. Microscopically, the tumor cells were arranged in solid and infiltrative sheets, and in most cases vascular channels or cystically dilated spaces were present. The neoplastic cells had abundant eosinophilic cytoplasm and large nuclei with open chromatin and prominent eosinophilic nucleoli. Intratumoral hemorrhage, neutrophilic infiltrates, and intracytoplasmic lumina were frequently present. All 10 tumors stained positive for one or more endothelial markers, with CD31 being the most sensitive marker. Seven cases stained positive for cytokeratin. Ultrastructural examination in three tumors confirmed their endothelial differentiation. In the absence of obvious vascular differentiation, abundant intratumoral hemorrhage and intratumoral neutrophils are useful ancillary morphologic features that may suggest a vascular origin. Six patients are dead of disease, one is alive with metastasis, and two patients are currently disease free. Epithelioid angiosarcoma of bone should be included in the differential diagnosis of epithelioid neoplasms of bone, and endothelial markers should be a part of their immunohistochemical analysis to avoid the misdiagnosis of a metastatic carcinoma because of the significant differences in the treatment and clinical outcomes of these entities.

Primary angiosarcoma of bone in Paget's disease

We report here a case of a primitive angiosarcoma of the bone occurring in Paget's disease. Only one case has been previously described in the literature. We review other types of tumours occurring in Pagetic bone and discuss medical and surgical management.

Epithelioid angiosarcoma of bone

Angiosarcoma of bone is a rare, high-grade sarcoma of vascular origin. This article describes an epithelioid angiosarcoma in the humerus of a 48-year-old man. A multilocular osteolytic lesion with undefined margins and destroyed cortical and medullary bone, associated with a large soft tissue mass was demonstrated radiologically in the proximal metaphysis of the right humerus. The tumor, resected by amputation, was composed mostly of proliferating malignant cells with an epithelioid morphology. It had a predominantly sheet-like growth pattern, and an occasional pseudoglandular or alveolar arrangement, mimicking an adenocarcinoma. The dilated anastomotic vascular spaces lined by epithelioid endothelial cells and the intracytoplasmic lumina/vacuoles that sometimes contained erythrocytes suggested focal endothelial differentiation. On immunohistochemical investigation, many neoplastic cells expressed cytokeratin and endothelial markers: factor-VIII related antigen, CD31, and UEA-I. The ultrastructure of the tumor was consistent with that of an angiosarcoma. Our patient died of disease shortly after the diagnosis, implying an aggressive clinical course. Awareness of the existence of skeletal epithelioid angiosarcoma, combined with the identification of intracytoplasmic lumina, or at least small vasoformative foci, and immunohistochemical positivity for endothelial markers provide the best guide for distinguishing this tumor from metastatic carcinomas.

Multi‐Focal, Multi‐Centric Angiosarcoma of Bone

A multi-focal multi-centric, malignant tumour of vascular origin arising in bone in a 55-year-old man is described. The presenting symptoms were pain and weight loss. Radiologically, multiple lytic lesions were demonstrated in the long bones of both legs and throughout the pelvis. Histological examination demonstrated an angiosarcoma which was predominantly low grade in nature but with focal areas of intermediate grade. Turnout cells expressed the endothelial markers CD31, CD34 and von Willebrand's factor. There was rapid radiological progression of disease with no response to radiotherapy. Pain abated within a few days of institution of doxorubicin, 75 mg m-2, but the patient died of massive pulmonary thromboembolism 14 days later, 11 months after the first symptoms.

Oncologic Outcomes of Primary Bone Sarcomas of the Pelvis

Although many studies have been performed for evaluation of surgical techniques and surgical morbidity in cases of primary pelvic sarcoma, few have addressed the long term oncologic outcomes for these patients. Therefore, the outcome of 41 patients with primary pelvic bone sarcomas were followed for 2-13 years (average, 6 years; median, 5 years). There were 18 chondrosarcomas (15 low grade and 3 high grade tumors), 11 osteosarcomas (1 low grade and 10 high grade tumors), 5 high grade malignant fibrous histiocytomas of bone, 5 Ewing's sarcomas, 1 undifferentiated sarcoma, and 1 low grade angiosarcoma of bone. Fifteen of the 17 patients with low grade tumors survived (88%), whereas only 6 of the 24 patients with high grade tumors survived (25%). Local disease recurred in 1 of the 24 (4%) patients with high grade tumors and in none of the patients with low grade tumors. Reoperations for complications were required in 33% of the patients. A difference could not be demonstrated in the quality of surgical margins or in the surgical morbidity, patient survival, and rate of local recurrence between patients treated with hemipelvectomy (8) and those treated with pelvic resection (32). Patients with low grade pelvic sarcomas have a good prognosis after pelvic resection, but those with high grade sarcomas continue to have a poor prognosis.

Ｍａｒｌｅｘ ｍｅｓｈによる胸壁再建法の検討

We evaluated the results of repairing large chest wall defects using marlex mesh in 7 cases. There were 4 male and 3 female patients ranging in age from 10 to 64 years (median age, 29 years). Ewing's sarcoma accounted for 2 cases, followed by one chondrosarcoma, one angiosarcoma of bone, one metastatic sternal tumor, one fibrosarcoma and one desmoid tumor. In all cases resection of chest wall tumors was done including a partial resection of the sternum in 3 cases. There were no remarkable complications postoperatively, and all patients remain alive without any trouble in ventilatory and circulatory function. In this paper, we stressed the utility of marlex mesh for repairing large defects of the chest wall.

Primary angiosarcoma of the cuboidal bone: A case report

Primary angiosarcoma of bone is a rare neoplasm. The unique cytomorphologic and ultrastructural aspects of a primary angiosarcoma of the cuboid bone diagnosed by fine-needle aspiration are presented. Smears and cell block preparations revealed single and clustered large, pleomorphic, and spindle-shaped cells with a markedly hemorrhagic background. Electron microscopy revealed characteristic features of endothelial cells including rare Weibel-Palade bodies. The clinical, cytologic, histologic, and ultrastructural aspects of the case are discussed.

骨腫ようにおけるＭＲＩの有用性

The magnetic resonance imaging (MRI) characteristics of primary malignant bone tumors are evaluated in determination of the surgical margin. Fifteen patients were examined with Toshiba MR imager (0.22-T resistive magnet). Eight patients had osteosarcoma, three had chondrosarcoma, three had giant cell tumor of bone, and one had angiosarcoma of bone. All patients had CT scans available for comparison. The extra-and intra-osseous extension of the lesion were separately studied. MRI was considerably superior to CT in all cases with osteolytic lesion. MRI was, especially, superior to CT for demonstrating the extent of tumor in marrow. Moreover, MRI was extremely useful for the assessment in the lesion of thoracic wall. CT was only superior to MRI for depicting the extra-osseous extent in screlotic type of osteosarcoma. Above all, MRI was the imaging method of choice in determination of the surgical margin for malignant bone tumors.