1/18. Multiple intracranial juvenile xanthogranulomas. Case report.The authors report on an 11-year-old boy in whom proptosis of the eye caused by a benign intraosseous xanthofibroma of the left orbital wall became clinically apparent at the age of 4 years. Two years later he developed bilateral papilledema, at which time computerized tomography and magnetic resonance studies revealed multiple enhancing intracranial lesions. The largest mass was located in the left middle fossa; other lesions were located at the tentorium cerebelli, in both lateral ventricles, near the superior sagittal sinus, and extracranially near the left jugular vein. The mass in the left middle fossa was resected and diagnosed as juvenile xanthogranuloma (JXG). Thirty months later, the patient again became symptomatic, exhibiting behavioral abnormalities and a decrease in mental powers. At that time, the two remaining lesions in both lateral ventricles had grown enough to cause trapping of the temporal horns and raised intracranial pressure. These lesions were successively resected and histopathologically confirmed to be JXGs. However, resection of the second intraventricular lesion was complicated by postoperative bilateral amaurosis, presumably caused by postdecompression optic neuropathy. According to a review of the literature, fewer than 20 patients with JXG involving the central nervous system have been reported. The patient described in this report is the first in whom multiple intracranial JXGs developed in the absence of cutaneous manifestations. Although JXGs are biologically benign lesions, the treatment of patients with multifocal and/or progressive intracranial manifestations is problematic.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/18. September 2000: 15 year old girl with intracranial lesion.The September COM: We reported the case of a 15 year old white girl with solitary brain cortical lesion clinically mimicking meningioma. Microscopic examination revealed histiocytic lesion with foamy and giant cells predominately of Touton type. Subsequent clinical examination did not reveal any similar lesion in the skin or in other location. Expression of S-100 protein in histiocytes is unusual finding, but has been reported recently in juvenile xanthogranulomas (JXG). The only specific ultra-structural finding differing JXG from histiocytic neoplasm of Langerhans' cells histiocytosis type seems to be Birbeck granules.- - - - - - - - - - ranking = 0.0098856658155721keywords = brain (Clic here for more details about this article) |
3/18. Isolated cervical juvenile xanthogranuloma in childhood.STUDY DESIGN: This is a report of an exceptional case of isolated cervical juvenile xanthogranuloma in a child. OBJECTIVES: This case report draws attention to the fact that isolated xanthogranuloma of the central nervous system should be considered among possible diagnosis of subdural extramedullary spinal masses in children and young adults. SUMMARY AND BACKGROUND DATA: Isolated juvenile xanthogranuloma of the central nervous system is extremely rare. When located in the spinal canal it behaves like any extramedullary mass-occupying lesion. MRI depicts the tumor's association with adjacent structures. In cases in which a subtotal surgical removal was possible, radiotherapy has been indicated. methods: A three-year-old girl presented severe pain in the right shoulder and spastic tetraparesis. The MRI showed an intradural extramedullary mass homogeneously enhancing after DTPA-gadolinium infusion. Complete surgical removal of the tumor was performed through open-door laminoplasty. RESULTS: The child was pain free immediately after the surgical removal of the tumor. A gradual complete recovery of the neurologic deficits followed. Open-door laminoplasty provided sufficient operative space, and it minimized the impact on the growing spinal column. CONCLUSIONS: Isolated juvenile xanthogranuloma does not show any predilections of localization inside the central nervous system. Both intracranial and spinal juvenile xanthogranulomas appear isointense in MRI and enhance homogeneously with gadolinium. Whenever possible, total surgical removal alone seems to be curative. Otherwise, a subtotal removal of the tumor might be followed by radiotherapy. Immunohistochemical tests ensure the diagnosis.- - - - - - - - - - ranking = 3keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/18. Spectroscopic increase in choline signal is a nonspecific marker for differentiation of infective/inflammatory from neoplastic lesions of the brain.We report in vivo proton magnetic resonance (MR) spectroscopic findings in three benign infective/inflammatory lesions (one case each of tuberculoma, fungal granuloma, and xanthogranuloma), which showed high choline along with the presence of lipid/lactate, a feature characteristically described in neoplastic lesions. Histopathology of the lesions showed inflammatory cellular infiltrates with areas of necrosis/caseation. The spectroscopic-visible increased choline resonance in these lesions is probably the result of cellularity. We conclude that increased choline, along with the presence of lipid/lactate is a nonspecific finding and may not be of much value in the differentiation of neoplastic from nonneoplastic infective/inflammatory intracranial mass lesions.- - - - - - - - - - ranking = 0.039542663262288keywords = brain (Clic here for more details about this article) |
5/18. Giant orbital and intracranial xanthogranuloma--a short report.Xanthogranuloma are known to arise in the paranasal sinus or orbit. They may also arise primarily in the brain. Those arising from the sinuses or orbit might involve the intracranial cavity to some extent. But an extensive involvement of the cranial compartment is very rare. This report describes one such case.- - - - - - - - - - ranking = 0.0098856658155721keywords = brain (Clic here for more details about this article) |
6/18. Bilateral multifocal uveal juvenile xanthogranuloma in a young boy with systemic disease.BACKGROUND: Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that could occasionally produce diffuse systemic disease in young children, but associated posterior uveal lesions are very uncommon. methods: Case report of an 8-month-old boy with macrocephaly who presented with chronic subdural haematoma and intracranial hypertension. A combination of nodular skin lesions and bilateral yellowish choroidal infiltration with limited retinal involvement suggested juvenile xanthogranuloma. RESULTS: The diagnosis was confirmed by a skin biopsy, and oral corticosteroid therapy was introduced. Progression of the disease to involve an anterior uveitis with hypopyon and numerous other systemic lesions, including the central nervous system, lung, liver, spleen, kidney and testis, was also suggestive of Letterer-Siwe disease. histiocytes were negative for Langerhans cell markers (S-100 and CD1a) and positive for macrophage marker (CD68). Electron microscopy failed to show Birbeck granules. Ocular lesions regressed under prolonged corticosteroid treatment, but resurgence of the other lesions required chemotherapy with vinblastine. CONCLUSION: In this atypical systemic variant of juvenile xanthogranuloma with bilateral uveal involvement, the immunohistochemical and ultrastructural findings were crucial in distinguishing juvenile xanthogranuloma from Langerhans cell histiocytosis.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/18. Primary cerebral non-Langerhans cell histiocytosis: MRI and differential diagnosis.We report a young woman with primary cerebral non-Langerhans cell histiocytosis of the juvenile xanthogranuloma family. The clinical course was complicated by extensive infiltration of cranial nerves and meninges and epi- and intramedullary spinal dissemination. Whereas the cutaneous form of juvenile xanthogranuloma is usually benign and self-limited, central nervous system involvement is associated with high morbidity and mortality and might therefore be considered a separate clinical entity.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/18. Multicentric parenchymal xanthogranuloma in a child: case report and review of the literature.OBJECTIVE AND IMPORTANCE: Xanthogranulomas of the central nervous system are rare, and asymptomatic lesions are often identified in autopsies. We report the first case of a multicentric, cystic, intraparenchymal xanthogranuloma, involving both the supra- and infratentorial compartments, in a 16-month-old girl. CLINICAL PRESENTATION: The clinical presentation consisted of a focal motor seizure in an otherwise healthy infant. INTERVENTION: Gross total removal of the infratentorial lesion was accomplished via a midline suboccipital craniotomy. CONCLUSION: Intracranial xanthogranulomas are rare, and limited experience with the diagnosis of these lesions has been reported. Although most cases are asymptomatic, this case involves a symptomatic, multicentric, intraparenchymal xanthoma in a pediatric patient. Our review of the literature provides further insights regarding the clinical, radiological, and pathological behavior of these lesions and examines the available treatment strategies.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/18. Incidental pediatric intraparenchymal xanthogranuloma: case report and review of the literature.Juvenile xanthogranuloma (JXG) is a specialized form of non-Langerhans cell histiocyte proliferation that occurs in children. The majority of cases present as a solitary cutaneous lesion with a predilection for the head and neck region; however, isolated lesions occasionally have been identified in the central nervous system. The cutaneous forms of JXG usually follow a benign course. Other physicians have reported surgery as the first line of treatment in symptomatic patients with accessible lesions. Adjuvant therapies may be indicated for multicentric or surgically inaccessible lesions. The authors describe an unusual case of isolated intraparenchymal JXG in an asymptomatic child with no cutaneous manifestations and provide a review of the literature.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/18. Treatment of severe disseminated juvenile systemic xanthogranuloma with multiple lesions in the central nervous system.Juvenile xanthogranuloma (JXG), one of the most common forms of Langerhans-dendritic cell proliferation in young children, usually presents as spontaneously regressing cutaneous lesions. JXG with systemic (extracutaneous) involvement is a rare histiocytic disorder in which significant morbidity and death may occur. The systemic type, especially combined with multiple central nervous system lesions in young children, has a very poor prognosis. The patient described here presented with disseminated disease including lungs, liver, kidneys, ribs, scalp, and central nervous system. The patient was treated with multiagent chemotherapy based on the Langerhans cell histiocytosis II treatment protocol. The regimen used included an additional intrathecal therapy with methotrexate and prednisolone to control central nervous system lesions. The patient was treated for 28 months and has been in remission for almost 5 years.- - - - - - - - - - ranking = 7keywords = central nervous system, nervous system (Clic here for more details about this article) |
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