Cases reported "Splenic Neoplasms"

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1/27. Splenic lymphoma with villous lymphocytes presenting as leucocytoclastic vasculitis.

    We describe a 69-year-old woman who presented with purpura on the legs. Examination of a blood film revealed a homogenous population of abnormal lymphoid cells with villous projections. The immunophenotype was consistent with a diagnosis of splenic lymphoma with villous lymphocytes (SLVL). A type II (IgM-IgG) cryoglobulin was detected in the serum. Renal biopsy demonstrated a membranoproliferative type I glomerulonephritis with intraluminal IgG and IgM deposits, and a skin biopsy showed a leucocytoclastic vasculitis with outlining of the vessels by IgG and IgM. vasculitis has been reported in association with a number of haematological malignancies, most frequently with hairy cell leukaemia. To our knowledge this is the first report of SLVL presenting with cryoglobulinaemic vasculitis.
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2/27. Isolated diffuse hemangiomatosis of the spleen with Kasabach-Merritt-like syndrome.

    AIMS: Diffuse haemangiomatosis of the spleen is a rare benign vascular condition occurring as a manifestation of systemic angiomatosis or, less commonly, confined to the spleen. It is sometimes accompanied by severe disturbance of blood coagulation. The goal of this study was to characterize an additional case of isolated diffuse haemangiomatosis of the spleen and to determine the histogenesis of this lesion which remains obscure. methods AND RESULTS: We describe a case of isolated diffuse haemangiomatosis of the spleen in which histological and immunohistological findings suggested the possibility of a malformative tumour-like lesion. The pathological cavernous vessels were distributed randomly through the red pulp, without continuity with sinuses. The endothelial cells expressed vimentin, factor viii related antigen and CD34, but not CD8. Some cells lining the sinus lumen expressed CD68, lysozyme and myeloperoxidase. In addition, trabecular veins presented with intimal thickening. These results allow making a diagnosis between diffuse haemangiomatosis and other tumours/tumour-like lesions of the spleen, especially littoral cell angioma, splenoma and peliosis. CONCLUSION: If diffuse haemangiomatosis is usually classified as a benign proliferation of endothelial cells, we suggest that diffuse haemangiomatosis, when confined to the spleen, could be a tumour-like vascular lesion. In this hypothesis, the aetiology may be hamartomatous or malformative as is suspected in arterio-venous haemangioma of the lower extremities. The histogenesis is still questionable and no definitive proof in favour of one or the other hypothesis has been reported.
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3/27. color Doppler sonographic findings in splenic hamartoma.

    We present the gray-scale and color Doppler sonographic findings in a case of a splenic hamartoma in a 40-year-old man. Gray-scale sonograms showed a 2 x 2 cm, hypoechoic splenic mass that was homogeneous without evidence of cystic change or calcification. color Doppler sonograms showed multiple radial blood-flow signals inside the mass, and spectral analysis confirmed arterial and venous flow. Arteriograms showed multiple small, hypervascular masses with fine tumor vessels and tumor stains within the spleen. Histologic analysis following a splenectomy showed dilated vessels and congestion consistent with the color Doppler sonographic findings.
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4/27. Intravascular occlusive therapy: use of interventional radiology in cancer patients.

    Selective transcatheter intravascular occlusion in the treatment of cancer patients is a valuable extension of interventional diagnostic radiology. Intra-arterial embolization may be performed with various substances, including autologous clot, autologous subcutaneous tissue, Gelfoam, and stainless steel coil. Clinical applications in cancer patients include control of gastrointestinal and genitourinary hemorrhage, preoperative reduction of tumor vascularity, control of local symptoms, and therapeutic reduction of tumor bulk. The technique has been used for preoperative and palliative treatment of neoplasms of the head and neck, kidney, liver, spleen, and soft tissue and bone. Transcatheter intravascular occlusion should be performed only by radiologists experienced in angiographic techniques. Inadvertent occlusion of a normal vessel and thromboembolism are possible complications.
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5/27. Littoral cell angioma as a rare cause of splenomegaly.

    A 58-year-old, otherwise healthy man presented with a sudden onset of watery diarrhea. A pseudomembranous colitis due to antibiotics was identified as the cause of the diarrhea. Enlargement of the spleen was detected during the evaluation. The enlarged, plump spleen (20 cm long, 7.1 cm wide) had multiple nodules that differed in size from 1 to 8 cm. Neither clinical nor other symptoms of an underlying malignant disease could be detected. Because the signs were of little diagnostic value we arranged a splenectomy, which showed a littoral cell angioma (LCA) to be the cause of splenomegaly. In addition to the case report, we have reviewed the literature, clinical manifestations, differential diagnosis, special gross and microscopic pathological findings, and the location of this benign vessel tumor in the pathology of the spleen.
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6/27. Splenic angiosarcoma and iron deficiency anemia in a 43-year-old man.

    thrombocytopenia and microcytic anemia are two laboratory findings that alone or together suggest an underlying disease process. Both are found throughout particular age groups and have broad differential diagnoses. Angiosarcomas are rare neoplasms from the lining of blood vessels. Primary splenic angiosarcoma is an even rarer neoplasm, first reported in the late 1870s. We report a case of primary splenic angiosarcoma in a patient with thrombocytopenia, microcytic anemia, and splenomegaly.
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7/27. Necrotic cutaneous lesions induced by hypereosinophilic syndrome secondary to a T-cell lymphoma.

    Cutaneous lesions of hypereosinophilic syndrome (HES) may display various presentations. These are important to recognize to allow early diagnosis. We report an unusual case of a young man with HES secondary to a splenic T-cell lymphoma, revealed by diffuse necrotic cutaneous lesions. Later on, brain and heart infarctions developed, leading to the patient's death. Analysis of skin biopsy specimens showed occluded dermal vessels surrounded by activated eosinophils. Because a complete analysis of hemostasis was unremarkable, these findings suggest that dermal vessel thrombosis was the direct consequence of the migration of activated eosinophils. Cutaneous lesions of HES are protean and probably reflect the differences between the lesional events induced in situ by activated eosinophils.
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8/27. Multiple lymphangioendothelioma of the spleen in a 13-year-old girl.

    A 13-year-old girl had a history of an upper-quadrant abdominal mass for about six months. On exploratory laparotomy, multiple discrete large nodules were seen in the spleen. Histologically, the spleen had the unusual appearance of lymphangioma, in that there was a tremendous proliferatiion of endothelial cells that formed either papillary projections in the lumina of the cavernous lymphatic vessels or a solid mass of cells without the lumina. The changes were suggestive of sarcomatous transformation, but the evidence was not conclusive. A term "lymphangioendothelioma" is proposed for this multinodular tumor.
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9/27. Splenic lymphangioma with papillary endothelial proliferation: a case report and review of the literature.

    A 76-year-old man complained of difficulty breathing. A solitary mass was found in the spleen by ultrasonography and the tumor was excised. Grossly, the tumor was 3.9 x 2.9 cm in size, solid and brownish in color. A stellate scar-like fibrosis was observed in the center of the tumor. Histologically, the tumor consisted of the proliferation of irregular and small lymph vessel-like spaces, with sclerotic change in the center. The lymph vessel-like spaces showed papillary projections of the lining cells. The lumen contained amorphous proteinaceous fluid. Immunohistochemically, the lining cells of lymph vessel-like spaces were positive for endothelial markers (CD31, CD34, factor viii-related antigen), and bound ulex europaeus agglutinin-1. The tumor was diagnosed as splenic lymphangioma, but its appearance was rather unusual for a typical splenic lymphangioma because of the presence of papillary endothelial proliferation and scar-like fibrosis. Splenic lymphangioma with papillary endothelial proliferation is uncommon, and there have been only four cases reported.
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10/27. Primary large cell lymphoma of the splenic sinuses: a variant of angiotropic B-cell lymphoma (neoplastic angioendotheliomatosis)?

    A case of large cell lymphoma of B-cell lineage originating in the splenic sinuses is described. In addition to widening the spectrum of primary malignant lymphomas of the spleen, this case raises the possibility that variants of angiotropic large cell lymphomas may exist that do not involve blood vessels but do involve the spleen and lymph node sinuses.
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