Cases reported "Hemangiosarcoma"

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1/45. Metastatic cardiac angiosarcoma of the cervical spine. Case report.

    STUDY DESIGN: A case report of metastatic cardiac angiosarcoma of the cervical spine. OBJECTIVES: To show that this rare spine tumor behaves in the same manner as an arteriovenous malformation and embolization, which can allow for successful spine surgery, and to discuss the natural history and rarity of this tumor. SUMMARY OF BACKGROUND DATA: Primary angiosarcoma of the heart is a very rare tumor, with fewer than 200 reports in the English literature and nothing reported in the spine literature. RESULTS: The patient in this study initially sought treatment for neck pain, left arm pain, and weakness 17 months after cardiac surgery and subsequent chemotherapy. A cervical computed tomography scan demonstrated a C5 lytic vertebral body tumor with intracannilicular extension and cord compression. An anterior cervical approach was made, but the tumor was too vascular to resect, and surgery was aborted. The C5 vascular vertebral body metastasis subsequently was embolized successfully by an interventional neuroradiologist. reoperation via an anterior approach with corpectomy, cadaveric fibula, and anterior locking plate internal fixation was successful, producing marked improvement in the patients' symptoms. CONCLUSION: Spinal involvement by primary cardiac angiosarcoma is very rare, and this is only the second operative case ever reported. The vascular nature of this tumor makes it behave in a manner similar to that of a high-flow arteriovenous malformation. Surgery should not be undertaken before preoperative angiography and embolization. The dismal prognosis for this rare malignancy is discussed.
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2/45. Ruptured primary splenic angiosarcoma into the colon. Presentation as anal bleeding.

    A 71-year-old woman presented with a six month history of constipation and abdominal discomfort, with anal bleeding during the last days. ultrasonography and CT-scan of the abdomen showed a large heterogeneous mass that was located in the splenic region, but the nature and origin of the tumour could not be clearly established preoperatively. The clinical diagnosis was of abdominal tumour with colonic and splenic involvement, and a left hemicolectomy and splenectomy were performed. Pathologic examination revealed a primary angiosarcoma of the spleen with penetration and fistulization of the tumour into the large bowel. The patient received adjuvant radiation therapy, but she died of extensive metastastic disease from her primary angiosarcoma of the spleen nine months after surgery. In summary, splenic angiosarcoma is very difficult to diagnose preoperatively. This highly aggressive neoplasm has an overall poor prognosis, specially if it is associated with rupture and haemoperitoneum. As this case highlights, unusual forms of rupture may lead to atypical clinical presentations, increasing even more the difficulty in the diagnosis.
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3/45. Primary orbital angiosarcoma: a case report.

    PURPOSE: The pathogenesis, natural history, histopathology, and recommended treatment for orbital angiosarcoma are illustrated and reviewed. methods: Case report. RESULTS: A 71-year-old white male presented with bluish discoloration and swelling of the left medial canthal area. A fine needle aspiration and excisional biopsy with histopathologic examination was performed, which showed angiosarcoma. Pattern of growth was demonstrated radiographically and histopathologically, confirming primary orbital angiosarcoma. Subsequent wide surgical resection was carried out, with substantial reconstruction of the left orbital and periorbital area. The patient responded well to the surgery, and was free of tumor after six years of follow-up. CONCLUSION: Angiosarcoma is a rare and highly malignant tumor of epithelial origin. The aggressive nature of this tumor usually results in a high mortality rate despite treatment. However, early diagnosis and wide surgical excision has resulted in successful treatment of these tumors.
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4/45. Fine-needle aspiration biopsy of postradiation epithelioid angiosarcoma of breast.

    Angiosarcoma of breast skin and parenchyma is a rarely reported complication of irradiation for breast carcinoma. We report a case of a subareolar epithelioid angiosarcoma arising 8 years subsequent to lumpectomy and irradiation of the ipsilateral breast for infiltrating carcinoma. The epithelioid appearance of the neoplastic cells on fine-needle aspiration biopsy (FNA) biopsy suggested a recurrence of the primary carcinoma. Careful attention to certain cytomorphologic features and cell block immunohistochemistry were useful in the distinction from recurrent carcinoma. Cytologic features that identified this neoplasm as an angiosarcoma included marked cell discohesiveness, elongate cytoplasmic processes or "pseudopodia," heterogeneous cell size, large nucleoli or macronucleoli, and cytoplasmic lumina. Immunohistochemical markers, including factor viii antigen, CD31, and CD34, were positive, confirming the vascular nature of the neoplasm. Other markers ruled out morphologically similar neoplasms such as recurrent carcinoma and melanoma. Epithelioid angiosarcoma should be included in the differential diagnosis of a suspected recurrence of breast carcinoma several years postirradiation therapy. Diagn. Cytopathol. 2000;22:172-175.
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5/45. A case of malignant transformation in thoracic vertebral hemangioma following repetitive irradiation and extraction.

    We report a rare case of thoracic vertebral hemangioma which developed into angiosarcoma during the course of repetitive operations and irradiation. A 44 year old female was operated on for hemangioma of the first thoracic vertebra. The diagnosis of hemangioma was confirmed histopathologically with the specimen from the first operation. The tumor developed multiple lesions later in the clinical course after the first operation, these lesions were removed in four consecutive operations and each histological diagnosis was that of hemangioma. Throughout the period of these operations, the patient was treated with steroid, and with radiotherapy simultaneously. The patient underwent the fifth operation for the recurrence of the tumor on 26 March 1990, and the histopathological diagnosis was not hemangioma but hemangiosarcoma which was considered a malignant transformation. The tumor cells immunohistochemically revealed positive staining with UEA-I, Factor-VIII, as the tumor immunohistochemically showed a vascular endothelioid nature.
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6/45. Intravascular papillary endothelial hyperplasia in a vascular lesion of the paranasal sinus.

    Intravascular papillary endothelial hyperplasia (IPEH) is usually a cutaneous lesion thought to represent a peculiar manifestation of an organizing thrombus. The principal significance of IPEH is its microscopic resemblance to angiosarcoma and possible misinterpretation as such. The occurrence of IPEH in the paranasal sinus is exceedingly rare. In this article, we report the first case to our knowledge of IPEH in a vascular lesion that originated from the ethmoid sinus and extended into the sphenoid sinus and sella. Despite the benign nature of this lesion, it could have been mistaken for a malignant tumor because of its clinical course and radiologic findings.
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7/45. Establishment and characterization of a spontaneously immortalized myofibroblast cell line derived from a human liver angiosarcoma.

    BACKGROUND/AIM: fibrosis and/or cirrhosis are present in the precursor stages of most liver cancers. However, little is known about the reciprocal interactions of fibroblasts, mainly responsible for fibrosis, and the other liver cells. We report here the isolation of a new liver myofibroblast cell line from a human liver angiosarcoma and its characterization. methods: The cells were isolated by the explant technique and characterization was performed, on one hand, using immunohistochemical and ultrastructural analysis and, in the other hand, by determining their karyotype, ras and p53 status and their tumorigenic properties. RESULTS: To date, the cells have undergone approximately 170 population doublings and are still proliferating. Immunohistochemically, they were negative for desmin, smooth muscle myosin, cytokeratin 19 and von willebrand factor, positive for vimentin and alpha-smooth muscle actin, with an important deposition of fibronectin around the cells. Ultrastructure showed particularly cytoplasmic microfilament bundles. Their chromosome number ranged from 38 to 168 with a bimodal population, near diploid and hypotetraploid. No mutations were found in codons 12, 13 or 61 of Ha-, Ki- and N-ras genes but a homozygous missense mutation in codon 179 (CAT-->CTT) was detected in the p53 gene. They were unable to form foci in soft agar or tumors in nude mice. CONCLUSIONS: Taken together, these results show that these cells, called BM 2.2.1, exhibited typical myofibroblast-like features. Although they contained a karyotype suggestive of tumoral cells and a homozygous mutated p53 gene, they were not tumorigenic. The nature of these cells and the abnormalities of the p53 gene and the karyotype, suggest that: i) they were a component of the tumor stroma, and ii) they could have been involved in angiosarcoma development. Thus, this cell line may be valuable for the study of cellular interactions in liver carcinogenesis.
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8/45. Immunohistochemical characterization of thyroid gland angiomatoid tumors.

    The histogenesis of thyroid gland angiomatoid tumors, probably as a primary angiosarcoma, has been a controversy for many years. These tumors may be variants of undifferentiated (anaplastic) carcinomas. We report a thyroid gland angiomatoid carcinoma in a 61-year-old African American male. The tumor had a heterogeneous pattern with both sarcomatous and epithelioid areas. Tumor cells lined some vascular-like spaces and others had intracytoplasmic lumens containing red blood cells. The tumor cells were found to express multiple endothelial (factor viii-related antigen, CD31, CD34, and ulex europaeus I lectin) and epithelial (cytokeratin and epithelial membrane antigen) markers as well as thyroglobulin by immunohistochemistry. This rare presentation demonstrates the heterogeneous nature of thyroid gland angiomatoid carcinoma with both epithelial and endothelial differentiation.
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9/45. Primary retroperitoneal angiosarcoma: MR imaging features.

    We report a rare case of retroperitoneal angiosarcoma in a 72-year-old man who presented with abdominal pain. Diagnosis was obtained histologically after radical excision of the tumour. Fat-suppression MRI after intravenous administration of the contrast agent gadolinium-DTPA was able to define tissue planes between the lesion and the adjacent structures, suggesting the vascular nature of the lesion, and provided useful information for an accurate surgical approach. To the best of our knowledge, this is the first report that illustrates the MRI characteristics of a retroperitoneal angiosarcoma.
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10/45. hemorrhage in cerebral metastasis from angiosarcoma of the heart: case report.

    The purpose of this article is to describe the clinical and pathological features of metastatic angiosarcoma in the central nervous system. Only a few cases of cerebral metastasis from angiosarcoma of the heart have been recorded in the literature; particularly related to intracerebral hemorrhage. A case of secondary cerebral angiosarcoma of the heart in a 33 years old man is presented. The initial symptoms were headache, vomiting, lethargy and aphasia. There was a mass in the left temporal lobe with hemorrhage and edema on the computerized tomography (CT). After 24 hours the neurological status worsened and another CT scan showed rebleeding on the tumor area. He underwent an emergency craniotomy but died two days after. Considering the longer survival of sarcoma patients with new modalities of treatment, the incidence of brain metastasis may increase, demanding a better preventive and more aggressive approach. Besides, due to the hemorrhagic nature of such lesions, we suggest the immediate surgery to prevent a fast and lethal evolution because rebleeding.
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