Cases reported "Erdheim-Chester Disease"

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1/23. A case of erdheim-chester disease with bilateral orbital involvement.

    PURPOSE: To describe a case of erdheim-chester disease with bilateral orbital involvement. methods: A 43-year-old female with bilateral proptosis was presented. Its clinical features, image findings, pathological character and therapeutic effect were evaluated. RESULTS: CT demonstrated bilateral, diffuse orbital mass. Histopathologic assessment revealed a diffuse xanthogranulomatous process with clusters of lipidladen histocytes. Numerous Touton giant cells were scattered throughout the lesion. Renal and heart failure happened during a 6-year follow-up period. Long bones roentgenogram demonstrated diffuse symmetrical sclerosis with extensive, lytic lesions. Systemic administration of corticosteroids, chemotherapy, immunoglobulin and traditional Chinese medicine showed good therapeutic result. CONCLUSIONS: An administration of systemic corticosteroids, chemotherapy, immunoglobin and traditional Chinese medicine can control erdheim-chester disease. Further exploration of its pathogenesis and collection of useful clinical data are required.
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keywords = bone
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2/23. Biochemical markers of bone turnover, serum levels of interleukin-6/interleukin-6 soluble receptor and bisphosphonate treatment in erdheim-chester disease.

    erdheim-chester disease (ECD) is a rare non-Langherans form of histiocytosis characterized radiologically by symmetrical sclerosis of the metaphysis and the diaphysis of long tubular bones. macrophages are potent interleukin-6 (IL-6) producers and elevated IL-6 serum levels have been described in pathological conditions characterized by increased bone resorption. In a patient with ECD, during the acute phase of the disease we found high serum levels of IL-6 and IL-6 soluble receptor (sIL-6R) and high levels of bone turnover markers. After 5 years of combination therapy with oral prednisone and intravenous clodronate a significant reduction in the above mentioned biological parameters was seen. We suggest that the systemic disorders present in ECD could be related to the high serum levels of IL-6 and sIL-6R. We also propose the use of bisphosphonates in the clinical management of ECD.
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3/23. erdheim-chester disease.

    A 38-year-old man presented with numerous dermal nodules, similar to xanthoma disseminatum, that were histologically consistent with his diagnosis of erdheim-chester disease, a non-Langerhans cell histiocytosis. Other cutaneous manifestations of this disease include eyelid xanthelasma, pretibial dermopathy and pigmented lesions of the lips and buccal mucosa. The histological diagnosis of erdheim-chester disease was originally made on the patient's retroperitoneal tissue, obtained at a laparotomy for surgical treatment of a presumed phaeochromocytoma, and confirmed by the pathognomonic long bone X-ray findings of this disease.
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4/23. erdheim-chester disease: a unique presentation with liver involvement and vertebral osteolytic lesions.

    erdheim-chester disease is a very rare xanthogranulomatous, non-Langerhans cell systemic histiocytosis with an unknown etiology and pathogenesis. Histologically, it is characterized by a diffuse infiltration with large, foamy histiocytes, rare Touton-like giant cells, lymphocytic aggregates, and fibrosis. The histiocytes differ from the Langerhans cell group in ontogenesis, immunohistochemistry (positive for CD68 and negative for CD1a and S100 protein), and ultrastructural appearance (lack of Birbeck granules). Although most of the cases have symmetric osteosclerosis of the long bones, an involvement of the axial skeleton has also been described. Extraskeletal lesions are present in more than 50% of the patients and may involve the retroperitoneal space, lungs, kidneys, brain, retro-orbital space, and heart. This study presents the case of a patient with erdheim-chester disease with vertebral destruction and, for the first time, to our knowledge, involvement of the liver. The diagnosis is based on radiologic, histologic, immunohistochemical, and ultrastructural findings.
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5/23. Extracerebral subdural manifestation of Chester-Erdheim disease associated with a giant adenoma of the pituitary.

    Chester-Erdheim disease is a rare non-Langerhans histiocytosis, affecting within the CNS mainly the neurohypophyseal unit, the retrobulbar space and the parenchyma of cerebellum, cerebrum and brainstem. Here we present a case of a 55-year-old woman who developed an exophthalmus, edema and dyspnea, finally leading to death 4 months post admission to the hospital. A cMRI showed a retrobulbar fibrosis, a tumor in the sella turcica, and further tumor formation expanding from the pons to the spinal cord, but without involvement of the dural sheet. autopsy revealed multiple tumors attached to the pituitary gland, the tentorium, and the brainstem as well as a diffuse thickening of the dura. Histologically, the tumor tissue consisted of densely packed lipid-laden foamy macrophages positive for CD68 and intervening fibrillary cords. Interestingly, tumor cells did not infiltrate/affect the parenchyma but showed a strictly extracerebral/ subdural location. In addition, sections of the pituitary tumor revealed a chromophobe giant adenoma of the pituitary gland. As to our knowledge this is the first detailed description of an exceptional case of intracranial CED presenting with strictly extracerebral/subdural tumor masses accompanied by a giant adenoma of the pituitary gland.
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ranking = 20.751766563713
keywords = macrophage
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6/23. erdheim-chester disease mimicking multiple meningiomas syndrome.

    We describe a rare case of non-Langerhans histiocytosis, consistent with erdheim-chester disease (ECD), which presented with lesions resembling multiple meningiomas. The patient was initially evaluated for migraine headaches. Initial MR imaging demonstrated a parasellar mass and a second mass near the torcula considered to represent meningiomas. Within 1 year, he developed bilateral orbital lesions surrounding both optic nerves, which were also considered meningiomas. biopsy of one orbital mass revealed a non-Langerhans histiocytosis. Subsequently, soft tissue masses, a pericardial effusion, and bone lesions consistent with ECD were identified.
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7/23. Improvement of erdheim-chester disease in two patients by sequential treatment with vinblastine and mycophenolate mofetil.

    erdheim-chester disease (ECD) is a rare non-Langerhans' form of histiocytosis with a plethora of different clinical manifestations owing to multiple organ involvement. We report two patients who presented initially with different clinical symptoms. The presenting complaint of the first patient was bone pain, predominantly in the legs, whereas in the other patient the initial symptoms were related to obstruction of both ureters, as in idiopathic retroperitoneal fibrosis. Ultimately, ECD was diagnosed in both patients by the occurrence of both pathognomonic manifestations, the histologic presence of non-Langerhans' histiocytes in bone biopsies, and osteosclerotic lesions of the long bones. Because the extraosseous manifestations progressed and a single application of corticosteroids was ineffective, sequential treatment with vinblastine and mycophenolate mofetil, together with prednisolone, was started. At follow-up respectively 15 and 16 months after the start of treatment a beneficial effect was noted in both patients. These cases illustrate the clinical spectrum of ECD, detail the pathognomonic manifestations of this rare disease, emphasize the need to consider ECD as an uncommon but important differential diagnosis in patients with arthralgias or systemic fibrosis, and give the first evidence for a new treatment option.
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keywords = bone
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8/23. Positron emission tomography/computed tomography of a rare xanthogranulomatous process: erdheim-chester disease.

    erdheim-chester disease (ECD) is a disseminated xanthogranulomatous infiltrative disease of unknown etiology due to infiltration of different organs and bones by foamy histiocytes. A 37-year-old male with cerebral and periorbital lesions was diagnosed with this rare disease and was evaluated with magnetic resonance imaging (MRI) and 2-deoxy-2-[(18)F]fluoro-D-glucose (FDG) with positron emission tomography/computed tomography (PET/CT) imaging at baseline and following therapy. FDG-PET imaging allowed accurate evaluation of the extent of the disease at baseline, as well as assessment of response to therapy.
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keywords = bone
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9/23. erdheim-chester disease: MR imaging, anatomic, and histopathologic correlation of orbital involvement.

    erdheim-chester disease (ECD) is a rare form of histiocytosis of unknown origin characterized by tissue infiltration by lipid-laden histiocytes. Typically, the diaphyseal and metaphyseal portions of the tubular bones are affected, leading to a characteristic radiographic pattern of bone sclerosis. Orbital involvement is not infrequent and is manifested by exophthalmos and periorbital xanthomatous lesions, with associated visual problems. This case report documents imaging and pathologic findings in a patient with ECD with extensive orbital involvement.
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keywords = bone
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10/23. Myocardial involvement in erdheim-chester disease.

    erdheim-chester disease (lipoid granulomatosis) is a rare type II (non-Langerhans cell) histiocytosis with systemic manifestations. The disease causes nonspecific disturbances in the function of multiple extraosseous organs, most commonly the eyes, lungs, pituitary gland, and kidneys. diagnosis is usually made on the basis of radiologic evidence of cortical expansion of long bones. While the osseous and systemic changes have been well documented in the current literature, pathologic changes in the myocardium have not been well characterized since Erdheim and Chester's first description of this disease in 1930. In the 2 autopsy cases from Dartmouth-Hitchcock Medical Center (lebanon, NH) reported in the present study, myocardial involvement was severe and had contributed significantly to the patients' morbidity and death. We describe the autopsy results and correlate them with Erdheim's original descriptions of this disease. In neither of our cases was bony involvement characteristic of the disease, and the diagnosis was made postmortem on the basis of soft tissue findings at autopsy.
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keywords = bone
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