Cases reported "Vascular Neoplasms"

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1/451. Intravascular large cell lymphoma associated with hypoalbuminemia.

    We report here a 71-yr-old Japanese woman who presented with severe anasarca and hypoalbuminemia. She had a postmortem diagnosis of intravascular large B-cell lymphoma, which is a rare type of lymphoma characterized by an intravascular proliferation of lymphoma cells. Severe generalized edema was thought to be attributed to vascular and/or lymphatic obstruction due to proliferation of lymphoma cells within the lumina and/or an increase in catabolism induced by tumor proliferation. ( info)

2/451. Efficacy of induced hypotension in the surgical treatment of large cavernous sinus cavernomas.

    OBJECT: cavernous sinus cavernomas are rare lesions associated with high rates of intraoperative mortality and morbidity resulting from profuse bleeding. In this paper, the authors report their experience in treating five patients with histologically confirmed cavernous sinus cavernomas and describe the efficacy of induced hypotension in facilitating excision of the lesion. methods: All five patients were women ranging in age from 25 to 54 years, with an average age of 42 years. The mass was small in one and large (>3 cm in diameter) in four. In one patient with a large mass, cardiac arrest occurred after the craniotomy, and remarkable reduction in the size of the cavernoma was evident on postmortem examination. The other three large lesions were successfully removed piecemeal after induction of hypotension (60-80 mm Hg systolic pressure), which remarkably reduced the mass and the bleeding during surgery. In the remaining patient, who had a small lesion, the cavernoma was removed in one piece. CONCLUSIONS: cavernous sinus cavernoma can be thought of as a cluster of sinusoidal cavities, the size of which varies depending on the systemic blood pressure. During surgery, reduction of the mass and control of bleeding from the cavernoma can be achieved by inducing hypotension, which enables the safe excision of this lesion. This technique should be considered by surgeons resecting a cavernous sinus tumor, especially when cavernoma is suspected. ( info)

3/451. Epstein-Barr virus-associated intravascular lymphomatosis within Kaposi's sarcoma in an AIDS patient.

    Intravascular lymphomatosis (IL) is an unusual neoplasm characterized by multifocal proliferation of lymphoma cells exclusively within the blood vessels. We report here a patient with acquired immunodeficiency syndrome (AIDS) and disseminated Kaposi's sarcoma. A 233-bp amplification product of HHV-8 was detected in the dna extracted from specimens of Kaposi's sarcoma at different sites by polymerase chain reaction (PCR). At autopsy, the vessels within the Kaposi's sarcoma were dilated and filled with atypical large mononuclear cells. No such feature was seen in the vessels of non-Kaposi's sarcomatous regions. Immunohistochemically, the spindle cells of Kaposi's sarcoma were positive for CD31 (endothelial cell marker). The intravascular tumor cells were positive for CD45 (leukocyte common antigen) but negative for others, including chloroacetate esterase, CD45-RO (UCHL-1, Pan-T), CD3, CD43, CD20 (L26, Pan-B), CD30 (Ki-1), immunoglobulin heavy chains and light chains, CD56 (natural killer cell antigen), and CD31. Monoclonal rearrangement of immunoglobulin heavy chain gene was detected in the dna extracts from fresh tissue of Kaposi's sarcoma by PCR, which indicated that the lymphoma cells within the Kaposi's sarcoma were of monoclonal B cell origin. in situ hybridization revealed that EBER-1 transcripts were present in the lymphoma cells of IL but not in the spindle cells of Kaposi's sarcoma. To the authors' best knowledge, this is the first instance of IL in an AIDS patient with direct evidence of EBV association. ( info)

4/451. Pulmonary intravascular lymphomatosis: presentation with dyspnea and air trapping.

    Intravascular lymphomatosis (IVL) is a rare lymphoid neoplasm that is typically of B-cell lineage and characterized by proliferation of malignant cells within small arterioles, capillaries, and venules. We report a patient with pulmonary IVL who presented clinically with progressive dyspnea, fever, and a dry cough. Pulmonary function tests revealed a marked decrease in diffusion capacity with airflow obstruction and severe air trapping. High-resolution CT (HRCT) of the chest with inspiratory and expiratory images revealed mosaic attenuation consistent with air trapping. Transbronchial biopsies revealed the diagnosis of IVL with capillary expansion in the alveolar and peribronchiolar interstitial tissue. IVL should be considered in the differential diagnosis of a patient with an interstitial lung disease, air trapping on pulmonary function tests, and mosaic attenuation on HRCT. Transbronchial biopsies may be the initial diagnostic procedure of choice. ( info)

5/451. Hemorrhagic epithelioid and spindle cell hemangioma: a newly recognized, unique vascular tumor of bone.

    BACKGROUND: Epithelioid vascular tumors of bone are uncommon and include epithelioid hemangioma, epithelioid hemangioendothelioma, and epithelioid angiosarcoma. It is important to distinguish among them because they have significantly different biologic potential and require different forms of therapy. In the current study the authors describe six cases of a distinct benign epithelioid and spindle cell vascular tumor of bone that, because of their unusual morphology, were confused with aggressive vascular neoplasms. methods: Cases were retrieved from the surgical pathology files of the Department of pathology or from the consultation files of one of the authors. hematoxylin and eosin stained slides were examined. immunohistochemistry was performed on two cases and electron microscopy was performed on one case. RESULTS: The tumors arose in the small bones of the hands and feet and the tibia. Three patients had multifocal bone disease at the time of presentation. Histologically, all lesions were comprised of lobules of spindle cells that grew focally in a fascicular pattern and were associated with abundant hemorrhage. Plump epithelioid cells were intermixed and were present focally in the interlobular areas as well, in which they lined larger, more well developed vascular spaces, often protruding into the vascular lumen in a "tombstone" fashion. Immunohistochemically and ultrastructurally the neoplastic cells had features of endothelium. One case was treated by amputation, one by resection, three by curettage, and one by curettage plus radiation therapy. None of the lesions was locally aggressive nor did any metastasize. CONCLUSIONS: The authors believe that hemorrhagic epithelioid and spindle cell hemangioma of bone is a histologically benign bone tumor. It should be distinguished from malignant epithelioid vascular tumors of bone, which have metastatic potential and need to be treated more aggressively. ( info)

6/451. Intravascular angioleiomyoma.

    A 59-year-old man presented with a 6-year history of a 2-cm tender nodule on his left lateral elbow. Excisional biopsy led to the diagnosis of angioleiomyoma. However, unlike typical angioleiomyoma which demonstrate extravascular extension, this lesion demonstrated purely intravascular growth. ( info)

7/451. Intravascular lymphomatosis of the brain: a diagnostic problem.

    Intravascular lymphomatosis (of B- or T-cell origin) is a rare lymphoproliferative disorder characterised by neoplastic proliferation of lymphoid cells within the lumen of capillaries, small veins and arteries with no or minimal involvement of the parenchyma. Its predilection sites are the skin and the brain. We studied a 44-year-old man who presented with a 2 year history of unexplained LDH elevation followed by a neurological syndrome without systemic involvement. brain biopsy showed an intravascular lymphoma of the B-cell lineage. This report illustrates the diagnostic challenge of this rare disorder with a grim prognosis. ( info)

8/451. Intracavernous teratoma in a school-aged child.

    An eight-year-old boy presented with left eye pain, photophobia, proptosis, third nerve paresis and decreased visual acuity. magnetic resonance imaging revealed a nonenhancing mass filling the cavernous sinus. Using an extradural fronto-orbitozygomatic approach, the cavernous sinus was approached laterally, and a teratoma was removed from within the cavernous sinus. This is the first case of a truly intracavernous teratoma in a child and the fourth case of a teratoma reported in the cavernous sinus region overall. This report outlines the diagnosis and treatment of this unusual cavernous sinus tumor. ( info)

9/451. Combined surgical and medical approach to intravenous leiomyomatosis with cardiac extension.

    Intravenous leiomyomatosis with cardiac extension is a rare entity. The case of a 49-year-old patient is described: she was operated on for intracaval intra-atrial leiomyomatosis. After an incomplete procedure (the tumour appeared not totally resectable), the patient was treated for a period of three years with a GnRH-analogue, whereafter the patient was doing clinically well and the tumour, although it regained some growth, was in a stable situation. This new strategy seems of certain importance to the surgeon, as it carries an alternative to a high-risk reoperation. To our knowledge, this is the first description of such a combined therapeutical approach. ( info)

10/451. Stereotactic radiosurgery for cavernous sinus cavernous hemangioma--case report.

    A 40-year-old female presented with cavernous sinus cavernous hemangioma manifesting as left abducens and trigeminal nerve pareses. magnetic resonance imaging revealed a left cavernous sinus tumor. The tumor was partially removed. Histological examination of the specimen confirmed cavernous hemangioma. radiosurgery was performed using the gamma knife. The tumor markedly decreased in size after radiosurgery and morbidity was avoided. cavernous sinus cavernous hemangiomas may be difficult to treat surgically due to intraoperative bleeding and cranial nerve injury. Stereotactic radiosurgery can be used either as an adjunct treatment to craniotomy, or as the primary treatment for small cavernous sinus cavernous hemangioma. ( info)
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