1/13. Leiomyosarcomas of great vessels.Sarcomas of the great vessels are rare. Altogether 400 such cases have been described in the aorta, the pulmonary artery, and inferior vena cava. The clinical symptoms are generally related to embolic phenomena, aneurysm formation, and widespread metastases, especially to bones. With improved diagnostic modalities more cases are diagnosed and treated surgically. Resection of the tumor may prolong the patient s life. In this paper authors present two cases of such rare sarcomas. In our first case a tumor has developed in the thoracic aorta with symptoms of imminent aortic dissection. The tumorous nature of the lesion was revealed only histologically, since neither the operation, nor macroscopic picture gave any clue to its tumorous nature. The second case was a male patient with a huge retroperitoneal tumor arising from the inferior vena cava, which was clinically suspected to be a carcinomaarising in the adrenal gland.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/13. Combined resection and adjuvant therapy improves prognosis of sarcomas of the pulmonary trunk.BACKGROUND: diagnosis of pulmonary artery sarcoma is difficult and frequently delayed owing to the insidious, non-specific nature of the symptoms. We present our experience with surgery for this rare disease. methods: Between 1995 and 1998, four patients underwent surgery for pulmonary trunk sarcomas. They presented with similar symptoms and were initially treated for thromboembolic disease of the lung. Preoperative diagnosis was established using computer tomography and echocardiography. Postoperatively, two patients received radiotherapy and one combined radio-/chemotherapy. RESULTS: One patient underwent thrombendarteriectomy of the trunk with adjuvant radiotherapy and remains tumor-free after three years and one showed complete regression of metastases after chemotherapy and remains well after more than one year. The other two patients died within two months of surgery, one due to a tumor mass in the inferior caval vein and the other because of cerebral metastases. CONCLUSIONS: According to the literature, mean survival rate is 1.5 months without resection and 10 months with resection, with survival up to four years following combined resection and adjuvant therapy. Our findings are in agreement with this.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
3/13. Perivascular myoma: case report with immunohistochemical and ultrastructural studies.A subcutaneous myopericytoma-type perivascular myoma arising in the elbow of a 61-year-old woman is described. The tumor was well demarcated and consisted microscopically of small ovoid and spindle cells arranged in a concentric fashion, surrounding small to medium-sized vessels and imparting a superficial resemblance to hemangiopericytoma. In some areas, the cellular whorls were separated by myxoid stroma. cells located between cellular whorls appeared immature with scant cytoplasm but did not show distinct nuclear anaplasia, increased mitoses or foci of necrosis. immunohistochemistry showed that constituent cells were positive for alpha-smooth muscle actin and desmin. Electron microscopy disclosed that the immature-looking cells, as well as the ovoid and spindle-shaped cells, possessed focal densities along with thin filaments, subplasmalemmal densities, pinocytotic vesicles and an external lamina. These ultrastructural and immunohistochemical features indicate a myoid nature of pericytic cells and justify this type of neoplasm being categorized as perivascular myoid tumor.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
4/13. Fatal tumor thrombosis due to an inferior vena cava leiomyosarcoma in a patient with antiphospholipid antibody syndrome.We describe a patient with antiphospholipid antibody syndrome (APS) who died because of relentless inferior vena cava (IVC) tumor thrombosis due to an unsuspected leiomyosarcoma. Laboratory confirmation for APS was provided by functional identification of a lupus anticoagulant and anticardiolipin IgG and anti-beta2-glycoprotein I IgM antibodies. Although sensitive for detecting vascular obstruction, radiocontrast venography and magnetic resonance imaging and angiography detected the IVC thrombosis but failed to distinguish its malignant nature. Concomitant refractory thrombocytopenia prevented further invasive diagnostic and therapeutic maneuvers for progressive, severe IVC thrombosis unresponsive to aggressive treatment of APS. Deep venous thrombosis refractory to anticoagulant and immunomodulatory therapies in a patient with APS may be due to a concomitant underlying malignancy, such as a leiomyosarcoma, causing vascular obstruction.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
5/13. A case of leiomyosarcoma originating from pulmonary vein, occluding mitral inflow.Cardiac leiomyosarcoma is a very rare entity that is found in less than 0.2% of all cardiac tumors. Because of its rarity and classification problem, there are few prospective studies about therapy and outcomes. Generally, diagnosis is delayed because of the nonspecific nature of the symptoms and by the time of diagnosis, most cases present with large tumors. Primary treatment of these tumors is still complete surgical resection. In this study we present a myxoid leiomyosarcoma originating from a pulmonary vein and extending into the left atrium, occluding mitral inflow in a woman 49 years of age. The tumor was totally removed. Open heart surgery is the acceptable approach for the management of intracardiac tumors, which may cause obstruction of blood flow through the heart.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
6/13. angiolymphoid hyperplasia with eosinophilia: a rare artery lesion.BACKGROUND: angiolymphoid hyperplasia with eosinophilia (AHE) is a rare skin condition of unknown aetiology. The lesion seems neoplastic in nature, or at least an abnormal vasoproliferative reaction. CASE REPORT: A 40-year-old man presented with an 18-month history of erythematous papula over the right temporal area without a history of trauma. The patient reported a history of Hodgkin lymphoma at the age of 20, treated by radiochemotherapy. A subcutaneous nodule was found on the superior branch of the right temporal artery. An echocolordoppler revealed a normal temporal artery flow with pariental thickness. An excisional biopsy was performed and the patient remained asymptomatic at 24 months. The histological diagnosis was angiolymphoid hyperplasia with eosinophilia of the temporal artery. CONCLUSION: More appropriate studies are necessary to assess whether AHE is a manifestation of an unknown immunological disorder. If a correlation could be found between an altered immunological system and AHE, an intensive follow-up could be applied to patients. We report this case to encourage further studies to highlight potential challenges in the diagnosis and management of variants of vascular processes, such as AHE.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
7/13. Intravascular metastatic melanoma of the vena saphena magna.BACKGROUND: melanoma blood vessel metastases are very uncommon. We report the first recorded case of intravascular metastatic melanoma of the vena saphena magna. CASE REPORT: A 74-year-old woman presented with a swelling of the upper third of her left thigh and was suspected of superficial thrombophlebitis. Two intravascular black masses were excised in the vena saphena magna. Histopathologic examination showed an endovascular metastatic melanoma positively staining for HMB45 and S-100 proteins. Acral-lentiginous melanoma of her big toe was then found. Subcutaneous lymph node and chest metastases were discovered and treatment with dacarbazine was initiated, followed by treatment with fotemustine. The patient died 21 months after the diagnosis was made. CONCLUSIONS: Only a few cases of blood vessel metastatic melanoma have been reported. We here report the first recorded case, to our knowledge, of intravascular metastatic melanoma of the vena saphena magna. Surgical exploration allowed rapid diagnosis, and histological examination confirmed the intravascular nature of a hematogenous melanoma metastasis. This case clearly illustrates the need for histologic examination of any thrombotic material.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
8/13. Intravascular lymphoma - a rare cause of hemolytic anemia and neurologic disorders.Intravascular lymphoma is an uncommon and often overlooked form of non-Hodgkin's lymphoma characterized by extensive proliferation of lymphoid cells within the lumina of small and medium-sized vessels. Clinical symptoms of the disease are variable and often nonspecific, mostly neurologic in nature. With an aggressive course, intravascular lymphomatosis has a poor prognosis and is rarely diagnosed ante mortem. We describe here a 76-year-old woman with the clinical diagnoses of hemolytic anemia and progressive lethargy where intravascular lymphomatosis turned out as the underlying cause of the disease.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
9/13. Cutaneous intravascular NK-cell lymphoma: report of a rare variant associated with Epstein-Barr virus.Intravascular lymphoma (IVL) is a rare variant of non-Hodgkin lymphoma with a predilection for skin and brain. Except a few cases of T-cell lineage, most of the reported cases were large B-cell lymphomas. We encountered a case of cutaneous IVL in a 71-year-old woman presenting with multiple erythematous patches and nodules on her trunk and extremities. The intravascular large cells showed an immunophenotype of CD3epsilon( );, CD5(-), CD20(-), CD30(-), CD56( ), and TIA-1( ). The lymphoma cells were also positive for Epstein-Barr virus by Epstein-Barr virus-encoded rna in situ hybridization test and the T-cell receptor gene was germline. This IVL differs from nasal type NK/T-cell lymphoma only by its intravascular nature. Only 3 cases of intravascular NK-cell lymphoma have been reported before. Because this variant is extremely rare, our case is documented and compared with the 3 previously reported cases.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
10/13. Malignant peripheral nerve sheath tumour with vascular differentiation: a report of four cases.Four cases of malignant peripheral nerve sheath tumour showing vascular differentiation are described. One case was associated with neurofibromatosis 1 and contained angiosarcomatous, cartilaginous and rhabdomyoblastic elements. The other cases occurred in patients without neurofibromatosis and showed a spectrum of vascular lesions ranging from lobulated, haemangioma-like structures to angiosarcoma. These are the first recorded examples of this phenomenon not associated with neurofibromatosis. Immunohistochemical examination demonstrated the endothelial nature of the lesions in all cases and revealed cells positive for alpha-smooth muscle actin, probably pericytes, closely apposed to the endothelium.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
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