Cases reported "Xanthomatosis"

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1/109. Xanthogranulomatous oophoritis--a case report.

    This is a case of a 25 year old unmarried women who presented with intermittent fever and lower abdominal pain. laparotomy revealed a large cystic left sided tuboovarian mass adherent to surrounding structures and containing foul smelling fluid. microscopy showed extensive replacement of the ovary by a chronic inflammatory exudate composed predominantly of foamy macrophages.
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keywords = macrophage
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2/109. Determination of extent and activity with radionuclide imaging in erdheim-chester disease.

    erdheim-chester disease usually involves the diaphyseal and metaphyseal regions of tubular bones and various visceral organs. A 56-year-old woman presented with the histologically confirmed diagnosis of erdheim-chester disease. A Tc-99m MDP bone scan revealed the entire extent of the skeletal disease and showed unusual involvement of the epiphyses and axial skeleton. In addition to MRI, a Ga-67 citrate scan including SPECT showed extensive soft-tissue infiltration of different organs. Both Tc-99m MDP and Ga-67 scintigraphy are useful tools in determining the distribution of this rare disease.
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ranking = 0.0015031943804067
keywords = bone
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3/109. Extensive intracranial xanthoma associated with type II hyperlipidemia.

    Xanthomas are associated with a spectrum of medical conditions, most commonly disorders of lipid storage and lipid metabolism. They occur primarily in the subcutaneous tissues, especially along the achilles tendon and the extensor tendons of the hands. Intracranial xanthomas are extremely rare. We present a case of an extensive xanthoma of the temporal bone in a patient with hyperlipidemia.
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ranking = 0.00075159719020333
keywords = bone
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4/109. Xanthoma of the temporal bone: case report.

    OBJECTIVE AND IMPORTANCE: Xanthomas of the cranium that are not accompanied by endocrine or metabolic abnormalities are extremely rare. It is very important to understand the pathological features of this disease, for differential diagnosis from other diseases. CLINICAL PRESENTATION: A 62-year-old woman presented to the hospital with headaches. Cranial x-rays revealed bone destruction in the right temporal bone, with osteosclerosis in the area surrounding the lesion. In computed tomographic scans, the bone cortex was intact and the diploe was dilated because of the large mass of the lesion. T1-weighted magnetic resonance imaging findings were heterogeneous, with areas of hypo- and isointensity; T2-weighted magnetic resonance imaging findings were also heterogeneous, with areas of hypo- and hyperintensity. INTERVENTION: Total removal of the tumor was performed, followed by cranioplasty using artificial bone. diagnosis: Histologically, lipid-containing foamy cells were dense and a cholesterin granuloma was observed. There was no reason to infer other diseases, and a diagnosis of xanthoma of the temporal bone was reached. CONCLUSION: Xanthomatous lesions are observed in various pathological conditions, including malignant diseases. Xanthomas are benign lesions and the prognoses for patients with these lesions are satisfactory, even after partial excision. Therefore, it is quite important to distinguish xanthomas from other diseases that produce xanthomatous lesions.
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ranking = 0.00676437471183
keywords = bone
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5/109. The role of bone scintigraphy in patients with erdheim-chester disease.

    erdheim-chester disease (ECD) is a rare disorder that has been reported fewer than 60 times in the literature. Although clinical findings seem to be specific at first sight, histologic classification remains unclear. It has not been decided whether ECD is part of the spectrum of histiocytoses or whether it may be a lipid storage disorder or even a primary macrophage cell disorder, although it does show a distinct histologic pattern. However, the clinical appearance alone shows several typical features, rendering the diagnosis very probable if present. This article illustrates the importance of bone scanning in ECD, because the scintigraphic pattern of involved skeletal sites may in themselves lead to the diagnosis. Several differential diagnoses are considered. The importance of bone scintigraphy as an imaging method in patients with an unclear diagnosis is discussed, as exemplary in ECD, as is its role for the detection of sites of skeletal involvement in other diseases.
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ranking = 1.0045095831412
keywords = macrophage, bone
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6/109. Xanthoma of the temporal bone: a unique case of this rare condition.

    Xanthoma of the temporal bone is extremely rare; we describe only the fourteenth reported case. Our case is further remarkable because it is the first report of such an occurrence in a patient with familial type III hyperlipoproteinemia. Moreover, while otalgia, infection, hearing loss, and tinnitus were the most common initial symptoms in the previous 13 cases, our patient reported only diplopia, vertigo, and unstable gait. The patient underwent a simple mastoidectomy and debulking, and his diplopia, vertigo, and unstable gait resolved.
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ranking = 0.0037579859510166
keywords = bone
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7/109. Xanthoma of bone in a normolipidemic child: report of one case.

    Xanthoma invasion of the bone is a very rare disease especially in normolipidemic children. Bone erosion can be found in patients with this disease. However, due to the similarity of the symptoms of xanthoma with many other diseases including malignancy, the other diseases may initially be to be suggested and xanthoma may not even be considered. In this paper, we present an 8-year-old normolipidemic male child with a parietal bone xanthoma proved using tissue diagnosis. The clinical, radiographic and histological findings are also reviewed.
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ranking = 0.00450958314122
keywords = bone
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8/109. Xanthogranulomatous salpingitis and oophoritis: a case report and review of the literature.

    A case of xanthogranulomatous salpingitis and oophoritis in a 47-year-old woman is presented. Xanthogranulomatous inflammation is an uncommon form of chronic inflammation that is destructive to affected organs; it is characterized by the presence of lipid-filled macrophages with admixed lymphocytes, plasma cells, and neutrophils. Only a few cases of xanthogranulomatous salpingitis and oophoritis have been reported to date. The case presented here is associated with escherichia coli infection, endometriosis, and an intrauterine device.
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keywords = macrophage
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9/109. Nodular and bullous cutaneous mastocytosis of the xanthelasmoid type: case report.

    Severe generalized nodular and bullous mastocytosis of the xanthelasmoid type is described in a 7-month-old boy. Reddish to yellowish-brown xanthelasmoid papules and nodules first developed in the inguinal region a few weeks after birth and then progressively spread to cover nearly the entire body surface. There was severe pruritus and recurrent episodes of blistering. The diagnosis of cutaneous mastocytosis of the xanthelasmoid type with subepidermal bullae was confirmed by skin biopsies showing solid and deeply penetrating infiltrates of metachromatic mast cells under light and electron microscopy. Systemic involvement of other organs, however, was excluded by bone scintigraphy, abdominal ultrasound, bone marrow aspiration and echocardiography. The extensive skin involvement was reflected in highly elevated urinary levels of histamine (263.4 microg L(-1)) and its metabolite N-methylimidazole acetic acid (20.8 mg L(-1)). The patient was systematically well and received only symptomatic treatment. Over a period of 1 year, the condition gradually improved, and the skin lesions began to flatten and regress.
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ranking = 0.0015031943804067
keywords = bone
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10/109. Post-traumatic fibro-osseous lesion of the ribs and scapula (sclerosing xanthofibroma).

    Fibrous lesions of bone may be difficult to classify. Their etiology is controversial, most being considered to be developmental lesions rather than true neoplasms. We describe a patient with post-traumatic fibro-osseous lesion of the ribs, also known as sclerosing xanthofibroma; a lesion believed to be a reactive response to intramedullary hemorrhage following chest wall trauma. Clinically, the lesions in our patient were thought to be metastatic disease because of their multifocal appearance. An extensive clinical and laboratory metastatic evaluation with surgical resection of one of the lesions using intraoperative gamma probe was undertaken. In addition to the multiple rib involvement, our case also showed a lesion in the scapula. The pathologic and radiologic features of this entity are reviewed along with the summary of the differential diagnosis.
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ranking = 0.00075159719020333
keywords = bone
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