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1/6. Bilateral orbital metastases from a neuroendocrine tumor.

    When neuroendocrine tumors metastasize to the orbit, they usually do so as solitary lesions, sometimes involving an extraocular muscle. A 70-year-old woman with a known neuroendocrine tumor had bilateral painful proptosis, orbital soft tissue swelling, and ophthalmoplegia. Imaging showed masses within all the extraocular muscles. Orbital biopsy disclosed metastatic neuroendocrine tumor cells within the connective tissue.
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ranking = 1
keywords = connective
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2/6. Phosphaturic mesenchymal tumor: a case report.

    osteomalacia induced by tumor is a rare phenomenon in which the resection of tumor is followed by dramatic amelioration of clinical signs and symptoms. We hereby report a case of a 66 years old male who presented with features of osteomalacia in which the characteristic clinical presentation was associated with the phosphaturic mesenchymal tumor, mixed connective tissue variant. The case is reported for its rarity.
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ranking = 5.6639678268743
keywords = mixed connective, connective
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3/6. A new type of connective tissue nevus: isolated exophytic elastoma.

    An unusual case of isolated exophytic elastic tissue nevi in the scrotal region of a 64-year-old man is described. The histological and ultrastructural findings were those of abundant abnormal elastic fibers and increased reticulin in the dermis. This hamartomatous lesion possesses clinical and histological features previously undescribed in connective tissue nevi.
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ranking = 5
keywords = connective
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4/6. Hypophosphatemic oncogenic osteomalacia: report of three new cases.

    Three cases of connective tissue tumors causing hypophosphatemic osteomalacia are reported and the literature is reviewed. In two of our patients the tumors were completely excised with total disappearance of the symptoms. In one case a total excision was not possible and the symptoms of this patient have not completely disappeared. The substance responsible for the syndrome has not been identified yet, but probably interferes with vitamin d renal hydroxylation, thus causing osteomalacia. As more than 30 per cent of cases of this condition have been reported in the last 5 years, it is suggested that these tumors are more frequent than previously believed.
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ranking = 1
keywords = connective
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5/6. Linear nevi, hemihypertrophy, connective tissue hamartomas, and unusual neoplasms in children.

    The association of cutaneous nevi, hemihypertrophy, and connective tissue overgrowth has been described in many patients. We report long-term follow-up in two boys with such association who in addition developed testicular neoplasms. One patient also had progressive spinal cord compression as a result of stenosis of the spinal canal, and required amputation of a severely deformed limb. The importance of careful, long-term management in such patients is emphasized.
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ranking = 5
keywords = connective
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6/6. Oncogenic osteomalacia caused by a phosphaturic mesenchymal tumor of the oral cavity: a case report.

    We report a case of oncogenic osteomalacia associated with a phosphaturic mesenchymal tumor in a 31-year-old woman. She was presented with severe generalized bone and muscle pain and was restricted to bed. She lost 20 cm in height over the 8 years since she had first noticed a pain in her thigh. A walnut-sized, hard, soft tissue tumor was found very easily beside her lower molar teeth Radiologic examination revealed a remarkable decrease in bone density and multiple pathologic fractures of spine, femur and phalangeal bones. Severe hypophosphatemia, hyperphosphaturia, low plasma 1,25-dihydroxyvitamin D3 level and high plasma PTH level were disclosed at presentation. Histomorphometric examination revealed an extensive area of unmineralized osteoid and little mineralizing activity. A pharmacologic dose of 1 alpha-hydroxyvitamin D3 or or 1,25-dihydroxyvitamin D3 slightly increased the serum phosphate level and renal tubular reabsorption of phosphate, and slightly decreased plasma PTH level without any symptomatic improvement. Histologic examination of the tumor revealed a mixed connective tissue tumor that consisted of central woven bones and surrounding primitive spindle cells with prominent vascularities. After removal of the tumor, all biochemical, hormonal and radiologic abnormalities disappeared with remarkable symptomatic improvement.
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ranking = 5.6639678268743
keywords = mixed connective, connective
(Clic here for more details about this article)


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