1/18. Radiological and clinicopathological features of orbital xanthogranuloma.BACKGROUND: Orbital xanthogranuloma, a diagnosis confirmed histologically, occurs rarely in adults and children. With its characteristic macroscopic appearance the adult form may be associated with a spectrum of biochemical and haematological abnormalities including lymphoproliferative malignancies. METHOD: The clinicopathological features and imaging appearances on computed tomography and magnetic resonance imaging of this condition are described in eight adults and a child. RESULTS: Radiological evidence of proptosis was present in seven patients. In all nine patients an abnormal infiltrative soft tissue mass was seen, with increased fat in six cases. All patients had associated enlargement of extraocular muscles suggestive of infiltration and five had lacrimal gland involvement. Encasement of the optic nerve, bone destruction, and intracranial extension was present only in the child with juvenile xanthogranuloma. Haematological and/or biochemical abnormalities were detected in seven patients and seven patients had other systemic diseases which were considered to have an immune basis. One patient subsequently developed non-Hodgkin's lymphoma. CONCLUSION: The investigation and management of orbital xanthogranulomas requires a multidisciplinary approach even though the diagnosis may be suspected clinically. Imaging delineates the extent of disease and involvement of local structures and may influence the differential diagnosis. The juvenile form may be more locally aggressive, causing bone destruction with consequent intracranial extension.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/18. Intracardiac juvenile xanthogranuloma in a newborn.Juvenile xanthogranuloma (JXG) presents a normolipemic non-langerhans cells histiocytosis. JXG usually presents with cutaneous lesions. Visceral involvement is rare but may affect various organs. Deep JXG differs histologically from the cutaneous form by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. awareness of the possibility of this atypical presentation of JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen). JXG may present with intramuscular lesions only; however, rarely JXG has been reported to affect the heart but not without the typical cutaneous manifestations. We present an unusual case of deep JXG without systemic disease or metabolic abnormalities. To our knowledge, this is a first reported case of intracavitar JXG without skin lesions.- - - - - - - - - - ranking = 0.62639075439574keywords = organ (Clic here for more details about this article) |
3/18. Juvenile xanthogranuloma of peripheral nerve: a report of two cases.As a rule, juvenile xanthogranuloma (JXG) is a cutaneous lesion most often occurring in infancy. An inflammatory process of unknown etiology, it is self-limited and benign in nature. The spectrum of JXG has expanded to include adult examples, multifocal lesions, and ones arising at extracutaneous locations. Although a variety of extracutaneous sites may be affected, few reported lesions have involved cranial or peripheral nerves. Solitary examples have been reported in trigeminal nerve and spinal nerve root; affected individuals were children or adolescents. An optic nerve lesion has also been described. We describe two additional cases of JXG of nerve. One patient developed multiple dorsal nerve root lesions, as well as skin involvement. The other case featured isolated involvement of the left radial nerve. Both patients were adults with no known underlying systemic disorder. These cases further expand the spectrum of extracutaneous JXG, and underscore its consideration in the differential of nerve "tumors."- - - - - - - - - - ranking = 12keywords = nerve (Clic here for more details about this article) |
4/18. Isolated juvenile xanthogranuloma of the subglottis: case report.BACKGROUND: Juvenile xanthogranulomatosis (JXG) is a relatively rare macrophage proliferative disorder. It usually presents as a localized cutaneous lesion but may affect other organs. Until now it has never been described in the subglottic region of the larynx. methods: We report the first case of juvenile xanthogranulomatosis (JXG) in the subglottis in a 3 year old child. RESULTS: The localization in the subglottis caused airway obstruction requiring tracheostomy to secure the airway. On the basis that most cutaneous lesions regress spontaneously the lesion was managed expectantly and regressed over a period of 28 months allowing decannulation of the child. CONCLUSION: JXG should be considered in the differential diagnosis of subglottic lesions. Once the airway has been secured, JXG of the subglottis can be managed conservatively. Long-term follow-up is required because of the possibility of relapse at other sites.- - - - - - - - - - ranking = 0.62639075439574keywords = organ (Clic here for more details about this article) |
5/18. 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 = nerve (Clic here for more details about this article) |
6/18. Langerhans cell histiocytosis and juvenile xanthogranuloma. Two case reports.BACKGROUND: Histiocytoses represent a large, puzzling group of diseases which may involve the skin and other organs. At present, juvenile xanthogranuloma is the disorder most often confused with Langerhans cell histiocytosis. A complex overlap exists between juvenile xanthogranuloma and Langerhans cell histiocytosis, with lesions showing clinical and/or pathological features of both disorders. OBSERVATIONS: We report 2 patients affected by Langerhans cell histiocytosis who, during chemotherapy, presented cutaneous lesions with clinical and histological features of juvenile xanthogranuloma. During the therapy, in both cases, histological examination of new biopsies revealed the presence of Touton giant cells in the dermis with a few histiocytic cells; immunohistochemical staining was negative for CD1a, and no Birbeck granules were seen by ultrastructural examination. RESULTS AND CONCLUSION: A possible explanation for the link between Langerhans cell histiocytosis and juvenile xanthogranuloma regards the lineage development and the relationships of histiocytes. We suggest that chemotherapy can modify the production of cytokines by influencing the conversion or 'maturation' of pathological cells into macrophages or xanthomatous cells and fusing them to form multinucleated giant Touton cells. In our opinion, the modification of the cutaneous lesions during chemotherapy in Langerhans cell histiocytosis patients, as observed in our cases, could be a favorable prognostic factor.- - - - - - - - - - ranking = 0.62639075439574keywords = organ (Clic here for more details about this article) |
7/18. Tracheal juvenile xanthogranuloma in a child.Juvenile xanthogranuloma is the most common form of non-Langerhans cell histiocytosis in children. It usually presents in the form of cutaneous lesions. Occasionally, the disease may affect other organs. Visceral forms are usually associated with cutaneous lesions, and are easily diagnosed. In isolated visceral occurrence, however diagnosis may prove difficult. Having the same spontaneous remission as cutaneous lesions, visceral lesions require treatment only in the case of serious dysfunction. We describe a case of isolated juvenile xanthogranuloma of the trachea, which has not previously been reported in children. The patient presented with serious respiratory symptoms that required immediate intervention.- - - - - - - - - - ranking = 0.62639075439574keywords = organ (Clic here for more details about this article) |
8/18. Successful bone marrow transplantation for life threatening xanthogranuloma disseminatum in neurofibromatosis type-1.A 2-yr and 9-month-old female patient with neurofibromatosis type-1 presented with hepatomegaly, anemia, thrombocytopenia, and croupy cough and diagnosed with xanthogranuloma disseminatum (XD). She failed chemotherapy consisting of steroids, 6-mercaptopurine and methotrexate. A partial response to HLH-94 therapy that included etoposide and cyclosporine A was initially observed. However, she continued to have significant organ dysfunction without further improvement at 6 months of therapy. She then received matched unrelated donor bone marrow transplantation (BMT) following carmustine, etoposide, cytarabine and melphelan conditioning with complete resolution of symptoms. BMT is an option in therapy-resistant, life threatening XD cases.- - - - - - - - - - ranking = 0.62639075439574keywords = organ (Clic here for more details about this article) |
9/18. Chorioretinal involvement and vitreous hemorrhage in a patient with juvenile xanthogranuloma.We describe an unusual presentation of juvenile xanthogranuloma in a 4-year-old girl. Juvenile xanthogranuloma may be associated with posterior manifestations including optic nerve and choroidal granulomas, panuveitis, and vitreous hemorrhage in the absence of the classic anterior segment manifestations.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
10/18. Xanthoma in Meckel's cave. A case report.A case of xanthoma located within Meckel's cave and the semilunar ganglion is described in a patient with a trigeminal nerve deficit. This is the first case of xanthoma in such a location. The distinctive morphological appearance is illustrated and the possible histogenesis is discussed.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
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