1/20. Spontaneous regression of orbital Langerhans cell granulomatosis in a three-year-old girl.PURPOSE: To report a case of spontaneous regression of orbital Langerhans cell granulomatosis. METHOD: Case report. A 3-year-old girl was initially examined with a 5-week history of slowly progressive blepharoptosis and periorbital swelling of the left eye. RESULTS: Computed tomographic scan showed a mass in the left orbit eroding into the left frontal bone; fine-needle aspiration confirmed diagnosis of Langerhans cell granulomatosis. After initial biopsy, the patient was treated by close observation alone. Six months after initial examination, the monostotic lesion had completely resolved. CONCLUSIONS: In some cases of monostotic Langerhans cell granulomatosis, initial biopsy followed by observation alone may allow for the spontaneous regression of the lesion. This conservative approach to treatment is an important therapeutic option that may spare the patient the adverse effects of surgical resection, radiation, or chemotherapy.- - - - - - - - - - ranking = 1keywords = granulomatosis (Clic here for more details about this article) |
2/20. pulmonary veno-occlusive disease in pulmonary Langerhans' cell granulomatosis.This report describes unusual clinical and pathological findings in a 29-yr-old female with pulmonary Langerhans' cell granulomatosis (LCG). During a 7-yr clinical course her condition deteriorated despite corticosteroid therapy, and she died of respiratory failure and pulmonary hypertension. At autopsy, there were widespread pulmonary veno-occlusive disease (PVOD) lesions as well as abundant advanced and healed lesions of pulmonary LCG composed of multiple cysts and stellate fibrosis. The present case demonstrates that pulmonary Langerhans' cell granulomatosis should be considered as a possible cause of pulmonary veno-occlusive disease.- - - - - - - - - - ranking = 0.85714285714286keywords = granulomatosis (Clic here for more details about this article) |
3/20. Langerhans' cell granulomatosis in an adult: a 22-year follow up.A 57-year-old patient with Langerhans' cell granulomatosis (LCG) is presented. During 22 years of treatment, there were five relapsing infiltrations at different sites of the skull bones, which were treated by surgery, local radiotherapy, and chemotherapy. During the last relapse, the right temporal bone was infiltrated by granuloma and the petrous bone was destroyed with an intimate spread to the internal carotid artery. After two palliative surgical resections and ineffective radiotherapy, 12 cycles of chemotherapy (vinblastine plus prednisolone) were applied and a clinical remission of the disease was achieved. Special attention is paid to the complexity of diagnosis and choice of therapy. It is concluded that the behavior of LCG may change with time, and assume an aggressive form of the disease. Chemotherapy is the treatment of choice for this type of multifocal malignant form of LCG. prognosis of the disease is unpredictable.- - - - - - - - - - ranking = 0.71428571428571keywords = granulomatosis (Clic here for more details about this article) |
4/20. Langerhans cell granulomatosis: a case report of polyostotic manifestation in the jaw.We report the case of a 43-year-old woman who suffered from a polyostotic form of Langerhans cell granulomatosis (LCG) in the mandible and maxilla. The course of the disease was followed for more than 14 years. The disease's progression finally required subtotal resection of the mandible and reconstruction with a microvascular fibula graft. The literature is reviewed for diagnostic and treatment concepts based on the different stages of LCG.- - - - - - - - - - ranking = 0.71428571428571keywords = granulomatosis (Clic here for more details about this article) |
5/20. hypercalcemia in Langerhans' cell granulomatosis with elevated 1,25 dihydroxyvitamin D (calcitriol) level.A 48-year-old woman presented with a history of premature menopause, polyuria, polydipsia, fever, and diffuse bony tenderness. Her evaluation revealed central diabetes insipidus, hypothalamic amenorrhea, an elevated free calcium on multiple occasions with an elevated 1,25 dihydroxyvitamin D level, and osteoporosis by densitometry. Skeletal series revealed multiple lytic lesions involving the long bones. The diagnosis of Langerhans' cell granulomatosis was made. She was treated with hormone replacement therapy, radiotherapy, and vinblastine, with a dramatic improvement in her pain and a near normalization of her free calcium. Whereas hypercalcemia has been described in several granulomatous disorders and is secondary to unregulated extrarenal production of 1,25 dihydroxyvitamin D, it is, however, extremely rare in Langerhans' cell granulomatosis. This is the first case report of Langerhans' cell granulomatosis with hypercalcemia and documented elevated increased 1,25 dihydroxyvitamin D level that responded to the treatment of her primary disease.- - - - - - - - - - ranking = 1keywords = granulomatosis (Clic here for more details about this article) |
6/20. Langerhans cell granulomatosis manifested as pigmented villonodular synovitis.We report an unusual case of Langerhans cell granulomatosis (LCG) manifested as a villous synovial proliferation in a 38-year-old female jogger. One year after the onset of joint symptoms, she had a classical LCG presentation with skin and visceral lymph node involvement. review of the literature revealed only one case of synovial shoulder joint tenosynovitis associated with LCG in a middle-aged woman. Ours is the first reported case presenting clinically in the synovium of the hip joint as pigmented villonodular synovitis. Histiocytic/dendritic proliferations involving the synovial tissues are not uncommon. These lesions as well as the rare multicentric reticulohistiocytosis (MRH), a systemic monocytoid/histiocytic disorder with multinucleated giant cells, polyarthritis, and papulonodular skin lesions, should be considered in the differential diagnosis. Clinical and pathologic features will distinguish LCG from MRH.- - - - - - - - - - ranking = 0.71428571428571keywords = granulomatosis (Clic here for more details about this article) |
7/20. Pulmonary Langerhans cell granulomatosis with extrapulmonary involvement.Pulmonary Langerhans cell granulomatosis is defined as an abnormal langerhans cells infiltration in the lungs. Multifocal involvement is unusual, however. We report 3 cases of pulmonary Langerhans cell granulomatosis combined with either rib, thyroid or hypothalamus involvement.- - - - - - - - - - ranking = 0.85714285714286keywords = granulomatosis (Clic here for more details about this article) |
8/20. Signal intensity changes on MRI during the healing process of spinal Langerhans cell granulomatosis: report of two cases.Two patients with spinal Langerhans cell granulomatosis were followed by magnetic resonance imaging, and the observed signal changes were detailed. Both patients had no neurologic abnormalities but back pain. They were treated by immobilization with a brace and bed rest. Their back pain disappeared after about a month. At the first visit, the tumorous lesions involving vertebrae showed mainly low signal intensity on T1-weighted imaging (T1WI) and high intensity on T2-weighted imaging (T2WI). They were clearly enhanced by gadolinium diethylenetriaminepentaacetic acid (Gd-DTPA). Their original intensities changed to iso-intensities on both T1WI and T2WI without enhancement by Gd-DTPA after a year. These signal changes might indicate the healing stage of the involved vertebrae and recommend the removal of the spinal brace.- - - - - - - - - - ranking = 0.71428571428571keywords = granulomatosis (Clic here for more details about this article) |
9/20. An intracranial mass lesion in systemic xanthogranulomatosis: case report.The authors describe a 42-year-old woman with systemic xanthogranulomatosis and bilateral intraorbital tumors, who subsequently developed multiple lesions of the intracranial dura mater, spinal cord, retroperitoneum, pericardium, and mediastinum. Systemic xanthogranulomatosis is histologically similar to systemic Weber-Christian disease, except for the absence of subcutaneous panniculitis. Immunohistochemical studies suggest that this clinical entity can be differentiated from histiocytosis X, because foamy cells in systemic xanthogranulomatosis demonstrate macrophages but not T-zone histiocyte markers. Differentiation of this disease from other intracranial xanthogranulomas and treatment are discussed.- - - - - - - - - - ranking = 1keywords = granulomatosis (Clic here for more details about this article) |
10/20. Lymph node interdigitating cell granuloma associated with non-Hodgkin's malignant lymphoma. A case report and review of the literature.We report a new case of histiocytic proliferation, which histologically resembles histiocytosis X, in a lymph node affected by non-Hodgkin's malignant lymphoma. This brings the total number of such reported cases to 12. histiocytosis X cells, with folded nuclei, expressed S100 protein and an antigen recognized by anti-CD1 monoclonal antibodies. Ultrastructural study did not show any Birbeck granules and demonstrated a morphology similar to that of interdigitating cells. In the absence of Birbeck granules, the term 'Langerhans' cell granulomatosis' is not correct and should be replaced by either 'interdigitating cell granuloma' according to immunohistochemistry and ultrastructure or 'histiocytosis X-like granuloma' according to optical morphology. The fact that some cells with folded nuclei were positive for lysozyme argues in favor of the existence of transitional cells between histiocytes hnd interdigitating cells. The 11 other reported cases were reviewed. In 6 cases, this type of granuloma was associated with B cell lymphoma. In 3 cases the lymphoma was also probably of B cell type. In 2 cases, no information could be found. We could speculate that these histiocytosis X-like lesions are reactive, resulting from immune disturbances due to the lymphoma and/or the treatment.- - - - - - - - - - ranking = 0.14285714285714keywords = granulomatosis (Clic here for more details about this article) |
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