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1/13. life-threatening giant mediastinal goiter: a surgical challenge.

    Mediastinal goiter is a well known benign disease, usually resectable through a cervical approach with minimal morbidity and mortality. Only occasionally a median sternotomy or a lateral thoracotomy may be required. The present case is worthy of presentation because of the exceptional dimension of the disease and the surgical challenge that it presented. In a 72-year-old woman a large intrathoracic goiter of the right thorax caused a severe dyspnoea due to an important contralateral mediastinal shift with compression of the lung, superior vena cava system and trachea. At surgical exploration, through a cervico-sternotomic approach, the mediastinal structures dislocation and the strong adherences between the anomalous neovascularized capsula of the mass and the surrounding structures, complicated the surgical dissection. An accidental lesion of the innominate artery required its reimplantation on the ascending aorta. An immense mass, was finally removed and pathological examination revealed a rare case of neovascularized, pseudosarcomatoid capsula among a benign hyperplastic proliferation. In spite of its benign nature, a giant goiter caused a life-threatening compression of the respiratory tract and lung parenchyma in this patient. The dimension of the lesions, the mediastinal anatomy alterations and the severe intraoperative haemorrhage represented major technical difficulties during surgical resection.
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2/13. Inflammatory pulmonary nodules in Kawasaki disease.

    Symptomatic pulmonary manifestations of Kawasaki disease (KD) are uncommon. However, epidemiologic, radiologic, and histologic studies have indicated that respiratory symptoms and findings occur in KD and suggest that the KD agent may have a respiratory portal of entry. We report on three young infants with KD who developed pulmonary nodules, in addition to coronary artery aneurysms. Two patients had pathologic specimens available, one from biopsy and the other from autopsy. The nodules had predominantly mononuclear cell infiltrates, which were within the lung parenchyma and infiltrating vessel walls. Immunohistochemical studies of the nodules, using antibodies to common leukocyte antigen (LCA) and factor viii-related antigen, confirmed the inflammatory nature of the lesions and showed capillary proliferation. IgA plasma-cell infiltration was observed in the nodule, consistent with previous KD findings of IgA plasma-cell infiltration in the vessel walls, kidneys, pancreas, and upper respiratory tract. The two patients with nonfatal KD were treated with intravenous immunoglobulin and aspirin, with resolution of the nodules. We propose that when pulmonary involvement occurs in KD, it ranges from subclinical interstitial micronodular infiltrates to larger inflammatory pulmonary nodules. These pulmonary infiltrates and nodules likely reflect the host response to the etiologic agent of KD, and may resolve with the disease process. Recognition of this pulmonary complication of KD may enable cautious observation of such lesions for spontaneous resolution.
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3/13. Aggressive giant fibroepithelial lesion with unusual vascular stroma--a case report.

    The stroma of fibroadenoma and phyllodes tumor usually consists of fibroblastic proliferation. Rarely the stroma contains bundles of smooth muscle. Pseudoangiomatous hyperplasia of the mammary stroma has been described in fibroadenomas. However, true benign vascular stroma has not been reported. We report a case of a 34-year-old Chinese woman who presented with a large mass occupying the entire left breast. Left mastectomy was performed and showed a large, well-circumscribed, lobulated, rubbery-firm tumor measuring 13 x 10 x 6 cm. Microscopic examination revealed a fibroepithelial tumor formed by an organoid pattern of ductal structures with a very striking stromal appearance composed of extensive vascular proliferation and that demonstrated strong immunoreactivity for CD31, CD34, and factor viii. Ultrastructural examination revealed intercellular junctions, basal lamina, pinocytotic vesicles, and weibel-palade bodies in the cells lining the vascular spaces, confirming their endothelial nature. These findings rule out the diagnosis of pseudoangiomatous hyperplasia. The patient developed local recurrence a year later, and the resection showed malignant phyllodes tumor with ductal carcinoma in situ.The extensive vascular stroma noted in the primary tumor may have played a role in the malignant transformation of the epithelial and stromal components in this tumor.
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4/13. Spindle cell hemangioendothelioma exhibits the ultrastructural features of reactive vascular proliferation rather than of angiosarcoma.

    A patient with spindle cell hemangioendotheliomas was followed from 1964 to the present time, allowing the authors the opportunity to examine the lesions in the early, mature, and old phases. Organizing thrombi of different stages associated with slit-like vascular proliferation were always observed, whereas cavernous vascular spaces predominated as the lesions became older. Each spindle cell hemangioendothelioma initially developed relatively rapidly and was sometimes painful but then persisted as a silent nodule for decades. Transmission and scanning electron microscopic studies revealed that endothelial cells tended to digitate into the slit-like proliferating channels, became attached to other cells by means of tight junctions, and thus obstructed the channels at sites where thrombi developed repeatedly. The vascular spaces, ranging in nature from slit-like to cavernous, were outlined further by a relatively sparse mantle of ramified or dendritic interstitial cells that corresponded to spindle cells. Most of the cells appeared simply to be fibroblasts, but they developed the features of pericytes when they were close to the endothelial lining of well-developed vascular lumens. Large vascular spaces and phleboliths were surrounded by smooth muscle cells. Approximately 20% of the interstitial cells were dendritic macrophages characterized by phagocytic activity, presence of many lysosomes, and factor xiiia expression. The long and characteristic clinical course, the histologic evidence that thrombosis and its organization was continually occurring within the lesions, and the ultrastructural finding that spindle cell hemangioendotheliomas were composed of different microvascular segments from capillaries to veins, suggest that spindle cell hemangioendotheliomas may develop from a cycle of recanalization after thrombosis that occurs repeatedly because of the unique endothelial growth that was noted. This is in contrast with the previous conception that they were low-grade angiosarcomas.
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5/13. Bilateral extensive vascular calcification of the breast associated with coagulative necrosis: a calciphylaxis-like syndrome.

    A case of asynchronous bilateral breast necrosis in a 40-year-old woman with untreated chronic hypercholesteraemia is described. Mastectomies were performed, and histological examination revealed bilateral breast calciphylaxis characterized by extensive vascular calcification with coagulative necrosis. breast calciphylaxis is a rare disease clinically characterized by progressive tissular necrosis with secondary cutaneous ulceration and by vascular calcification and thrombosis. However, the nature of the vascular calcification has remained poorly understood up to now, owing to the absence of any precise mineralogical examination. In this case the mineral deposits were analysed for the first time: only hydroxyapatite was found. The etiology is discussed, and the high cholesterol rate of the patient is implicated.
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6/13. Spontaneous development of bilateral subdural hematomas in an infant with benign infantile hydrocephalus: color Doppler assessment of vessels traversing extra-axial spaces.

    We present an infant with macrocrania, who initially demonstrated prominent extra-axial fluid collections on sonography of the brain, compatible with benign infantile hydrocephalus (BIH). Because of increasing macrocrania, a follow-up sonogram of the brain was performed; it revealed progressive enlargement of the extra-axial spaces, which now had echogenic debris. color Doppler US showed bridging veins traversing these extra-axial spaces, so it was initially thought that these spaces were subarachnoid in nature (positive cortical vein sign). However, an arachnoid membrane was identified superior to the cortex, and there was compression of true cortical vessels beneath this dural membrane. An MRI of the brain showed the extra-axial spaces to represent bilateral subdural hematomas. The pathogenesis of spontaneous development of the subdural hematomas, in the setting of BIH, is discussed. We also emphasize that visualizing traversing bridging veins through extra-axial spaces does not necessarily imply that these spaces are subarachnoid in origin.
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7/13. Diffuse drusen and associated complications.

    An eye of a patient with an unusual form of diffuse drusen was studied by light and electron microscopy. Examination disclosed marked diffuse and nodular thickening of the inner portion of Bruch's membrane by vesicular and curvilinear membranous profiles and basement membrane-like material that was apparently derived from the retinal pigment epithelium. Deposition of this abnormal material suggests a diffuse abnormality of the retinal pigment epithelium and predisposes to serous detachment of the retinal pigment epithelium, choroidal neovascularization, hemorrhagic detachment of the retinal pigment epithelium, and disciform scarring. The features of this case are similar to previously reported cases of diffuse drusen, but differ in the early age of onset, intensity of changes, and, in part, by the nature of the abnormal material observed.
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8/13. choroidal neovascularization associated with malignant melanoma: a case report.

    Subretinal neovascularization observed over an elevated, pigmented choroidal lesion is usually considered evidence in favour of the benign nature of that lesion, a nevus. Histologic verification of malignant melanoma with choroidal neovascularization has never appeared in the literature. In this report, we present a case of subretinal neovascularization documented by fundus photography, fluorescein angiography, and histology in an eye containing a malignant choroidal melanoma.
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9/13. amyloidosis of the vitreous. Fluorescein angiographic findings and association with neovascularization.

    A case of primary systemic amyloidosis with bilateral asymmetric involvement of the vitreous was followed up throughout a 45-month span, during which two vitrectomy procedures were done on one eye. The progressive nature of this disease was documented by serial fluorescein angiograms and fundus photography. The amyloid seemed to be derived from the retina, at small multifocal areas over arterioles and venules that were otherwise clinically and angiographically normal in appearance. follow-up studies suggested that these focal deposits were actually preretinal, since they disappeared after posterior vitrectomy. Peripheral neovascularization was visible in this case, which has not previously been reported. amyloidosis of the vitreous can mimic numerous other diseases. vitrectomy can be effective in restoring visual acuity, although recurrence may be rapid. The diagnosis is readily made by pathologic examination of the vitreous aspirate.
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10/13. Surgical removal of an extrafoveal fibrotic choroidal neovascular membrane with foveal serous detachment in age-related macular degeneration.

    BACKGROUND: Visual recovery after submacular surgery for age-related macular degeneration (AMD) has been very limited. methods: A patient with an extrafoveal fibrotic choroidal neovascular membrane from AMD had an overlying serous foveal detachment with the fibrotic tissue elevating the foveal retina. Photocoagulation of the neovascular membrane was not recommended because of its nonpigmented, fibrotic nature. The membrane was surgically excised. RESULTS: Preoperative and postoperative visual acuity and central 30 degrees visual fields were followed. visual acuity improved from 20/200 to 20/25, and a preoperative central scotoma resolved completely 18 months after surgical excision of the extrafoveal fibrotic neovascular membrane. There were no intraoperative or postoperative complications. CONCLUSION: This type of patient may represent a distinct subset of patients with AMD amenable to subretinal surgery who could potentially have good recovery of vision.
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