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1/8. A case of intravascular large B-cell lymphoma mimicking erythema nodosum: the importance of multiple skin biopsies.

    BACKGROUND: Intravascular lymphoma is a rare disease characterized by the proliferation of neoplastic monuclear cells within the lumens of small blood vessels. The neoplastic cells are usually of B-cell origin, and rarely of T-cell or histiocytic origin. Although this clinicopathological entity of lymphoma has not been listed in general pathological classifications such as REAL classification or the Working Formulation, it is recently in the WHO classification scheme, which is essentially an updated REAL scheme, and the EORTC classification scheme. methods: In this report, a 62-year-old woman with intravascular large B-cell lymphoma was observed by clinical, histopathological, immunohistochemical and molecular methods. RESULTS: A 62-year-old woman presented with large erythematous macules on the bilateral thighs and lower legs. The lesions were accompanied with hard, tender, intradermal or subcutaneous nodules mimicking erythema nodosum. Histopathological examination in the first biopsy revealed non-specific panniculitis compatible with erythema nodosum. The second biopsy revealed emboli of atypical lymphocytes within many of the dilated and proliferated vessels in the deep dermis and subcutaneous tissue. These cells were positive for L-26 and kappa light chain, and negative for lambda light chain, factor viii-related antigen, CD30, CD34, CD68 and UCHL-1. These findings confirmed the diagnosis of intravascular large B-cell lymphoma. A laboratory examination showed a high level of LDH and abnormal cells in the bone marrow. An MRI of the brain and computed tomographic (CT) scans of the chest and abdomen revealed no evidence of malignancy. Before the treatment, the size of the nodules decreased spontaneously by about 50% in one month and significantly in two months. Although combination chemotherapy, which consisted of CHOP, brought her partial remission, she experienced neurological symptoms 6 months after the initial treatment and died of brain metastasis 9 months after the treatment. CONCLUSIONS: This is a unique case for two following reasons: 1) the first biopsy revealed non-specific findings compatible with erythema nodosum; and 2) before the treatment, the nodules regressed spontaneously. Dermatologists should take multiple skin biopsies for EN lesions with the non-specific histopathological findings not to refute the existence of this disease.
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2/8. Intravascular myopericytoma.

    BACKGROUND: Myopericytoma is a benign tumor composed of cells that show apparent differentiation towards putative perivascular myoid cells called myopericytes. It arises most commonly in the dermis or subcutaneous tissue of the extremities in adults. methods: We describe a myopericytoma that was unusual in its intravascular location. RESULTS: A 54-year-old man presented with a 10-year history of a painful slowly growing 1.5-cm nodule in the subcutaneous tissue of the thigh. Histologic examination of the excised lesion showed that is was entirely contained within the lumen of a vein. It was composed of a proliferation of myoid-appearing spindle cells, which were arranged in a striking concentric pattern around numerous blood vessels, in a manner that accentuated the vessel walls. This pattern is characteristic of myopericytoma. In some areas, fascicles of spindle cells, embedded in a myxoid stroma, bulged into the lumina of lesional vessels, reminiscent of myofibroma/myofibromatosis. Lesional spindle cells were diffusely positive for smooth muscle actin, focally positive for CD34 and were negative for desmin, cytokeratin, S100 protein, HMB-45 and CD31. CONCLUSION: This case illustrates that myopericytoma can be entirely intravascular in its location.
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3/8. Fulminant intravascular lymphoma presenting as fever of unknown origin.

    Intravascular large cell lymphoma (IVLL) is a rare neoplasm characterized by the proliferation of malignant lymphoid cells within the lumens of small to medium-sized blood vessels. The central nervous system, skin, and endocrine systems are most commonly involved. IVLL may disseminate to the heart, pancreas, liver, spleen, kidneys, and adrenal glands. We report a 52-year-old patient who was admitted for fever of unknown origin for 3 weeks, jaundice and abnormal liver function tests. Fever, high levels of bilirubin, severe anemia, thrombocytopenia, and a very fulminant course characterized the clinical picture. Although bone marrow involvement is quite rare, the diagnosis of IVLL in this patient was done by bone marrow biopsy. The patient was treated with CHOP protocol and received the first course but the aggressive disease led to the patient's death.
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4/8. Intravascular glomus tumour: a previously undescribed phenomenon.

    We report the first case of an intravascular glomus tumour, which was located in the right forearm of a 40-year-old male. Microscopically the lesion originated from the wall of a vein and protruded into the lumen of the affected blood vessel. The tumour cells were characterized immunohistochemically by the presence of vimentin, actin and myosin. Within the tumour, small nerves, immunopositive for S-100 protein and neurofilaments, could be identified. Histogenetically, the tumour is thought to derive from intramural epithelioid cells of the venous part of an arteriovenous anastomosis.
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5/8. An immunohistochemical study on "neoplastic angioendotheliosis": demonstration of B lymphocyte markers in the neoplastic cells.

    Frozen cerebral and renal tissue sections of an autopsied "neoplastic angioendotheliosis (NAE)" case were investigated immunohistochemically using monoclonal and heterologous antibodies to lymphocyte, monocyte, endothelial, epithelial and histiocytic antigens. In both tissues, positive stainings for surface immunoglobulin (sIg) mu and chi, but not lambda, were observed in most of the neoplastic cells. These cells were also positive for other B cell markers (BA-1, Leu-12 and HLA-DR). No distinct staining was observed in the neoplastic cells with antibodies to T lymphocyte (OKT-11 and Leu-1) or monocyte (OKM-1) markers. Positive stainings were observed only in some small round lymphoid cells which were distributed sporadically in and around blood vessels and were considered to be reactive. No positive staining was observed in the neoplastic cells with antibodies to endothelial (factor viii), epithelial (cytokeratin) or histiocytic (lysozyme) antigens. Thus, our NAE case was shown to be of monoclonal B cell lymphoma in nature.
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6/8. angiolymphoid hyperplasia with eosinophilia involving skeletal muscle.

    A vascular tumour involving the trapezius muscle is described. In addition to proliferating blood vessels with perithelial cuffing, there was a distinctive stroma of lymphoid aggregates, plasmacytoid cells, some mast cells and large numbers of eosinophils, many with a perivascular distribution. This solely intramuscular lesion was considered to be an example of angiolymphoid hyperplasia with eosinophilia. The aetiology and pathogenesis are discussed.
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7/8. An autopsy case of intravascular malignant lymphoma presenting with intracranial B-cell type malignant lymphoma.

    This report concerns a 79-year-old man with intravascular malignant lymphoma who was admitted to our hospital for slight right side hemiparesis. Radiological examinations showed a mass in the left parietal lobe, and a brain biopsy revealed a B-cell type malignant lymphoma. The tumor could not be detected on magnetic resonance images following focal radiotherapy, but the patient died of acute progressive pneumonia about 3 months after the onset of symptoms. An autopsy was performed. Microscopic examinations disclosed proliferation of neoplastic cells in the small and medium-sized blood vessels of the adrenal glands, liver, spleen, pancreas, kidneys and epididymis. A diagnosis of intravascular malignant lymphoma was established on the bases of these autopsy findings.
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8/8. The clear cell variant of epithelioid intravenous leiomyomatosis of the uterus: report of a case.

    A case of very rare uterine clear cell epithelioid leiomyomatosis is reported. The patient presented with a recurrent pelvic mass after hysterectomy 2 years earlier. The recurrent tumor was located mainly in the vessels of the broad ligament as worm-like plugs. The reviewed hysterectomy specimen showed a typical multi-lobulated intramural mass together with worm-like plugs within the myometrial vessels. Computer tomography exhibited metastatic nodules in both lungs. According to an histological examination, all the tumor cells were of epithelioid type and contained abundant clear cytoplasm. Characteristic large, thick-walled blood vessels were observed. Immunohistochemical staining and ultrastructural examination supported smooth-muscle origin in this case. Electron microscopic study revealed that the clear cytoplasm was attributed to the presence of numerous dilated mitochondria that had lost their cristae. The patient is still alive with the disease 28 months after surgery.
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