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1/7. magnetic resonance imaging of lymphomatoid granulomatosis: punctate and linear enhancement preceding hemorrhage.

    lymphomatoid granulomatosis (LG) is an angiocentric and angiodestructive lymphoproliferative disease that mainly involves the lung. Thirty percent of patients with LG have neurological symptoms. We report serial changes in MRI of a patient with LG of the brain. Postcontrast MRI demonstrated multiple punctate and linear areas that preceded hemorrhage, as indicated by hypointensity with surrounding hyperintensity on subsequent T2-weighted images. The diagnostic value of these lesions observed with contrast MR images is discussed. We consider that enhancing areas correspond to walls of small vessels affected by LG.
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2/7. Fine needle aspiration diagnosis of lymphomatoid granulomatosis. A case report.

    The diagnosis of lymphomatoid granulomatosis (LG) of the lung depends on obtaining adequate histologic material to demonstrate the characteristic angioinvasive, polymorphous, lymphoid infiltrate and normally requires an open lung biopsy. Fine needle aspiration biopsy (FNAB), if only smeared directly, does not allow an assessment of the lymphoid infiltrate in relation to blood vessels. However, we report a case diagnosed by FNAB in which the specimen was processed by an alternative method that allows cell blocks to be made from all visible particles. Percutaneous FNAB of a nodular pulmonary infiltrate was performed after bronchoscopy and transbronchial needle biopsy failed to yield a diagnosis. The FNAB specimen was placed in 50% alcohol and submitted for processing. The specimen was then filtered through a fine sieve, and all visible tissue was embedded in bacteriologic agar and processed as a standard surgical specimen. The filtrate was processed as standard fluid cytology. The atypical, angioinvasive, lymphoid infiltrate was clearly demonstrated on the cell blocks, and the diagnosis of LG was made. This diagnosis was confirmed by subsequent open lung and skin biopsies.
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3/7. Inflammatory sarcomatoid carcinoma: a case report and discussion of a malignant tumor with benign appearance.

    Inflammatory sarcomatoid carcinoma is an aggressive tumor with an unusually benign appearance. We report the case of a 65-year-old man with a history of inoperable poorly differentiated carcinoma of the right lung, for which he had received chemoradiotherapy. A new solitary mass was discovered 4 years later in the left lung on surveillance computed tomography. The patient underwent thoracotomy with a wedge biopsy on which frozen section was performed. The nodule was vaguely granulomatous and associated with a mixed inflammatory infiltrate and a deceptively bland spindle cell proliferation. Results of immunoperoxidase studies, however, showed that the nodule contained neoplastic cells with an epithelial phenotype that were invading the pulmonary vessels. These are features of the rare inflammatory sarcomatoid carcinoma. In contrast to sarcomatoid carcinomas, this case highlights the deceptively benign appearance of inflammatory sarcomatoid carcinoma. This leads us to concur with the recommendation to exercise caution when attempting the diagnosis of apparently benign lesions on intraoperative frozen section in patients with high clinical suspicion of malignancy.
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4/7. lymphomatoid granulomatosis: a rare mimicker of vasculitis.

    lymphomatoid granulomatosis (LG) is a rare Epstein-Barr virus-associated lymphoproliferative disorder, with a propensity for blood vessel destruction. Although it most commonly affects the lung, it can affect multiple extrapulmonary sites (i.e., skin, nervous system, gastrointestinal tract, liver, spleen, kidney, and heart). Since LG often mimics systemic vasculitis, it presents a diagnostic clinical challenge. We describe a case of LG with no pulmonary involvement, which was thought to be systemic vasculitis on the basis of multiorgan involvement and biopsy findings.
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5/7. lymphomatoid granulomatosis.

    The clinical and pathologic appearance of seven patients with lymphomatoid granulomatosis who had skin lesions when first seen is reviewed. Six patients subsequently developed systemic disease. Although the gross morphology of the skin lesions is variable, the pathology is distinctive. An adequate deep biopsy shows the characteristic lymphohistiocytic infiltrate with variable numbers of atypical cells. Angiodestruction is less evident in the skin compared to other organs. The infiltrate surrounds and invades not only vessels but also nerves and epidermal appendages. The skin biopsy specimen can be differentiated from the lymphomatous infiltrates and Wegener's granulomatosis. Two of the patients who developed systemic disease were diagnosed by skin biopsy but clinicians failed to institute therapy, preferring to wait for other organ involvement. In addition, two patients developed lymphoma, one of which was confirmed at autopsy and one on subcutaneous and bone marrow biopsy 5 years after the initial skin diagnosis. lymphomatoid granulomatosis can be diagnosed by performing a skin biopsy. Appropriate chemotherapy may result in a high percentage of complete remissions and therefore the dermatopathologist can play an important role in the early diagnosis of this potentially fatal disease.
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6/7. Microvascular injury in lymphomatoid granulomatosis involving the skin. An ultrastructural study.

    An electron microscopic study of a cutaneous lesion of lymphomatoid granulomatosis taken from a patient with pulmonary involvement was performed. Microvascular alterations ranged from mild, degenerative changes to vessel necrosis. Less severe changes included enlarged endothelial cells, with margination of small lymphoid cells in affected vessels. Luminal occlusion by necrotic endothelial cell fragments and fibrin was associated with thickening and reduplication of the basal lamina and an angiocentric, inflammatory infiltrate in severely affected vessels. The most frequently observed cells in the infiltrate were cleaved and noncleaved lymphocytes that exhibited notable cytolysis and degenerative changes in cytoplasmic organelles. We conclude that lymphomatoid granulomatosis involving the skin is a disorder in which substantial numbers of cleaved and noncleaved lymphoid cells participate, eventuating in small-vessel necrosis and occlusion and repeated endothelial cell regeneration.
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7/7. Magnetic resonance appearance of cerebral lymphomatoid granulomatosis.

    lymphomatoid granulomatosis is an angiocentric lymphoproliferative process that involves the lungs. In a 52-year-old-man with lymphomatoid granulomatosis who presented with encephalopathy, magnetic resonance imaging (MRI) of the brain demonstrated unusual multiple areas of enhancement that were both punctate and linear. These findings may be relatively specific for inflammation of deep cerebral vessels and have implications for MRI findings in other inflammatory cerebrovascular disorders.
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