Cases reported "Calcinosis"

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1/194. Mesenteric lipodystrophy with fever of unknown origin and mesenteric calcifications.

    Mesenteric lipodystrophy (ML) is a rare condition characterized by a nonspecific inflammatory process that involves the root of the mesentery in a lipoma-like lesion. Presenting features of ML include abdominal pain, weight loss, nausea, vomiting, diarrhea, and constipation. This case illustrates that ML should be included in the differential diagnosis of patients with fever of unknown origin and mesenteric calcifications.
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2/194. Calcifying fibrous pseudotumor (CFPT): a patient presenting with multiple pleural lesions.

    Calcifying fibrous pseudotumor (CFPT) is a rare pseudotumoral lesion initially described in the subcutaneous and deep soft tissues. More recently, 3 cases were reported in the pleura. We report an unusual presentation associating miliary pleural lesions with a single soft tissue lesion in a 29-year old, asymptomatic female patient. The multitude of pleural lesions prevented complete excision. Although the present case confirms an indolent process, the pathogenesis and long-term prognosis remain undetermined.
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3/194. 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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4/194. dna ploidy alterations detected during dedifferentiation of periosteal chondrosarcoma.

    dna ploidy of a case with dedifferentiated periosteal chondrosarcoma was analyzed by dna cytofluorometry. The diagnosis of primary periosteal chondrosarcoma was made on the basis of the radiographic and histological findings. At 4 years after marginal resection, the tumor recurred locally and metastasized to various organs. The patient died of disease 2 years later. Histologically, there were two components, chondrosarcoma and malignant fibrous histiocytoma, in the recurrent and metastatic tumors. dna ploidy analysis of multiple samples revealed that the primary lesion was composed of many diploid cells with some tetraploid and octaploid cells, whereas in the dedifferentiated area, there were many aneuploid cells which were not recognized in any area of the primary tumors. This case illustrated that dna ploidy alteration of euploidy to aneuploidy is closely correlated with the process of dedifferentiation in chondrosarcoma.
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5/194. Fibro-osseous lesions of the central nervous system: report of four cases and literature review.

    Fibro-osseous lesions, also reported as calcifying pseudoneoplasms of the neural axis, are uncommon lesions of the CNS. We report four additional cases: two extraaxial and two intraaxial, in patients ages 33, 47, 49, and 59 years at presentation. Fibro-osseous lesions involving the CNS demonstrate variable proportions of fibrous stroma, bone, palisading spindle to epithelioid to multinucleated cells in association with a highly distinctive, perhaps pathognomonic, chondromyxoid-like matrix often distributed in a nodular pattern. This histopathologically distinctive lesion can be seen in many regions of the neuraxis, often with a dural association, and most commonly along the vertebral column. It appears to be a slow-growing lesion and, with wide excision, the prognosis is excellent. The etiology remains unclear, but the preponderance of data favors a reactive rather than neoplastic process. If this putative pseudotumor is not recognized histopathologically, a neoplastic or infectious differential might result in inappropriate investigations and potentially harmful therapies.
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6/194. Corneal stromal calcification after topical steroid-phosphate therapy.

    Secondary corneal calcification involving the full thickness of the stroma is a rare potential complication of severe dry eye conditions, recurrent corneal ulcerations, chronic ocular inflammation, or multiple surgical procedures. We describe on a patient with unusual, hitherto unreported calcareous degeneration of the corneal stroma after topical steroid-phosphate therapy for chronic keratoconjunctivitis after stevens-johnson syndrome. The patient's serum levels of calcium and phosphorus were normal. Histopathologic and electron microscopic examination of the corneal button revealed mainly intracellularly located crystalline calcium deposits throughout all layers of the corneal stroma but sparing the Bowman layer. Energy-dispersive x-ray analysis confirmed the presence of calcium phosphate. The calcium deposits were closely associated with intracellular and pericellular accumulations of glycosaminoglycans. Our findings indicate that corneal stromal calcification may develop after topical steroid-phosphate medication, and suggest a possible role of alterations in the glycosaminoglycan metabolism of stromal keratocytes in the calcification process.
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7/194. Neonatal asteroid hyalosis.

    Asteroid hyalosis, noted in 0.83% of routine eye examinations, is uncommon in younger patients and is more frequently seen in patients more than 60 years old. It has been considered to be related to an aging process, and when it occurs in younger patients, ocular disease is typically associated. We report a 4-week-old patient with down syndrome and bilateral congenital cataracts who had unilateral asteroid hyalosis.
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8/194. The styloid or "Eagle" syndrome: an unexpected consequence.

    The development and pathogenesis of the elongated styloid process with a calcified stylohyoid ligament are reviewed and discussed. A case report is presented to demonstrate that, although the related symptoms may be mild, unless the patient is aware of the cause of the discomfort, a "cancerophobia" may cause great emotional distress.
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9/194. Development of a calcifying fibrous pseudotumour within a lesion of Castleman disease, hyaline-vascular subtype.

    A nine year old boy with localised Castleman disease of the hyaline-vascular subtype developed a calcifying fibrous pseudotumour. This pathological association does not appear to have been described before. In this case, the development of this very unusual soft tissue tumour-like process was thought to be related to a previous fine needle aspiration biopsy, which was performed because of lymphadenopathy localised to the right inguinal area. This case provides further evidence of the reactive nature of calcifying fibrous pseudotumour and also broadens the pathological spectrum of the stromal cell proliferation that occasionally supervenes within lesions of Castleman disease, hyaline-vascular type.
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10/194. Ulcerated dystrophic calcinosis cutis secondary to localised linear scleroderma.

    Dystrophic calcinosis is a more common form of calcinosis cutis; calcium salts are deposited secondary to local inflammation, tissue damage and degeneration. Various conditions can cause dystrophic calcinosis, including connective tissue disease, infection, inflammatory processes, chronic venous stasis, cutaneous neoplasm and trauma. We report a case of ulcerated cutaneous calcinosis associated with a localised linear scleroderma or morphea. Healing of the ulcerations took place after four months of treatment with colchicine 1 mg per day.
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