Cases reported "Calcinosis"

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1/88. High-resolution computed tomography of pulmonary alveolar microlithiasis.

    Pulmonary alveolar microlithiasis (PAM) is a rare disease. It has been reported predominantly in turkey. We report a case of PAM with characteristic high-resolution computed tomography (CT) findings. A 45-year-old Taiwanese woman had progressive difficulty in breathing for 7 years. Her chest radiographs showed diffuse high-density micronodules and reticular lines that obliterated the bronchovascular bundles and the margin of the heart and diaphragm. The micronodules were scattered throughout both lung fields with basal predominance. Examination of a transbronchial lung biopsy specimen showed PAM. High-resolution CT showed a unique and characteristic calcified reticular pattern and thickening of the interlobular septa of the lung parenchyma, with predominant basal and peripheral lung distribution. Reticulonodular changes of the interlobular septa and intralobular interstitial lines associated with subpleural air cysts and paraseptal emphysema were evident. These high-resolution CT findings are pathognomonic for PAM. Thus, lung biopsy may be avoided in the presence of this characteristic finding.
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2/88. Giant unruptured aneurysm of the thoracic aorta--a case report.

    An asymptomatic 88-year-old woman underwent a screening medical examination. The chest x-ray film showed a large mediastinal mass with calcification. Both chest computed tomography and nuclear magnetic resonance imaging revealed an unruptured aortic aneurysm, predominantly affecting the ascending aorta and the proximal part of the aortic arch. Its maximum diameter was 10.5 cm. An ascending aortic aneurysm more than 10 cm in diameter is very rare. She died of acute pulmonary embolism unrelated to the aneurysm, and autopsy indicated that the etiology of the aneurysm was atherosclerotic degeneration. Retrospectively, the natural progression of the aneurysm was able to be followed on a series of chest x-ray films obtained over 18 years.
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3/88. carpal tunnel syndrome caused by an idiopathic calcified mass.

    This is a case report of carpal tunnel syndrome caused by an idiopathic calcareous lesion within the carpal canal. The median nerve was trapped between the transverse carpal ligament and the calcified mass. The mass was predominantly composed of calcium phosphate. Surgical release of the transverse carpal ligament and removal of the calcareous mass relieved the symptoms.
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4/88. Inflammatory pseudotumor of the spleen: a case report.

    We report on an inflammatory pseudotumor of the spleen. A 72-year-old woman visited our hospital complaining of nausea. physical examination and laboratory investigations were unremarkable. ultrasonography, computed tomography, magnetic resonance imaging and angiography showed a hypovascular splenic mass measuring about 5 cm in diameter with a calcification in the center of the lesion. splenectomy was performed. The removed spleen, weighing 145 g, contained a tan-white, circumscribed mass, measuring 6.2 x 5.5 x 5.3 cm. Histologically, the splenic mass was composed of an admixture of inflammatory cellular elements, predominantly plasma cells and lymphocytes with hyalinization, fibrosis, lymph follicles and multinuclear giant cells, suggestive of a inflammatory pseudotumor. The patient is currently alive and asymptomatic, 24 months after surgery. Inflammatory pseudotumors of the spleen are extremely rare and only 39 cases have been reported in the medical literature.
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5/88. Calcifying fibrous pseudotumor involving the neck of a five-week-old infant. Presence of factor xiiia in the lesional cells.

    Calcifying fibrous pseudotumor is an uncommon lesion characterized by hyalinized collagen, psammomatous or dystrophic calcifications, and a predominantly lymphoplasmacytic infiltrate. Although the pathogenesis is unclear, a possible relationship with other inflammatory "pseudotumors" has been proposed. We describe the pathology of two right neck calcifying fibrous pseudotumors present in a five-week-old female infant. The masses had many of the pathologic features of calcifying fibrous pseudotumor. The presence of a florid, mixed infiltrate, and the occurrence of more than one lesion in the same patient, favor the proposal that calcifying fibrous pseudotumor may be a sclerosing end stage of inflammatory myofibroblastic tumor. However, the presence of a previously undescribed participation of factor xiiia-positive cells suggests that the tumor may be of dermal dendrocyte origin.
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6/88. Meningioangiomatosis with a predominant fibrocalcifying component.

    A case of meningioangiomatosis, resected from the parietal lobe in a 31-year-old female is presented. Macroscopically, the lesion was composed of five calcified nodules embedded within hardened elastic tissue. Histologically, cortical and subcortical calcified masses were found surrounded by a palisade of spindle and/or oval cells. In adjacent nervous tissue many pathological microvessels were observed and some were ensheathed by a perivascular proliferation of spindle cells. Moreover, gliosis with Rosenthal fibers and prominent connective tissue elements were observed. Immunohistochemical analysis based on monoclonal antibodies was performed. The spindle cells both within the palisades and the perivascular proliferations were vimentin and usually epithelial membrane antigenpositive. The possible pathogenesis of meningioangiomatosis is discussed and the role of angiogenesis within this lesion emphasized.
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7/88. Calcifying epithelial odontogenic tumor with intracranial extension: report of a case and review of the literature.

    The calcifying epithelial odontogenic tumor (CEOT) is a rare benign neoplasm, possibly of stratum intermedium origin and occurring predominantly in the mandible of adults. The treatment varies, depending on its size, location, and histology. A case of an advanced CEOT arising in the maxilla with intracranial extension is reported. The report is supplemented by a review of the literature.
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8/88. Lethal form of chondrodysplasia punctata with normal plasmalogen and cholesterol biosynthesis.

    We present a male autopsied case of chondrodysplasia punctata with abnormal face, symmetrical proximal limb shortness, severe psychomotor developmental delay, respiratory muscle weakness, and death at the age of 2 years. Although his clinical manifestations were similar to those of rhizomelic chondrodysplasia punctata (RCDP), biochemical studies using skin fibroblasts did not document the peroxisomal dysfunction described in RCDP. In addition, the sterol profile, for which abnormalities have recently been reported in cases of X-linked dominant form chondrodysplasia punctata (CDPX2), was normal both in the liver and in the fibroblasts. This patient may represent a new lethal form of chondrodysplasia punctata.
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9/88. Idiopathic hypoparathyroidism with intracranial calcifications and dominant skin manifestations.

    Presented here is an unusual case of a 41-year-old man with idiopathic hypoparathyroidism strongly connected with dermatological, ophthalmological and neurological disorders. Since the age of 4 he had been treated ineffectively for mycosis resulting in complete baldness and atrophic nail plate changes. At the age of 35 he was diagnosed with idiopathic hypoparathyroidism. He underwent surgery twice due to bilateral cataract. CT scans of the head demonstrated numerous symmetrically located calcifications in both frontal lobes, subcortical nuclei, the paraventricular region, brain fornix, and both cerebellar hemispheres. The neuropsychological examination demonstrated the occurrence of psychosensory disorders under the form of hallucinations accompanied by a sense of fear and anxiety. It seems essential to investigate the parathyroid gland for any sign of pathology, especially amongst patients with severe dermatological and/or neuropsychological symptoms. early diagnosis and treatment of patients with hypoparathyroidism may prevent the development of many serious complications or at least result in marked improvement of neurological manifestations.
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10/88. Ectopic cardiac calcification associated with hyperparathyroidism in a boy with hypophosphatemic rickets.

    An adolescent with hypophosphatemic rickets developed cardiac calcifications in the absence of hypercalcemia or elevation of the phosphocalcic product (the product of the total serum calcium and phosphorus concentrations). Cardiac calcifications led to aortic and mitral valve dysfunction, myocardial calcification, and arrhythmia. hyperparathyroidism probably played a significant role in the development of this complication, which emphasizes the necessity for intermittent assessment of parathyroid status in individuals receiving medical therapy for hypophosphatemic rickets.
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