Juvenile Xanthogranuloma

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Jihong Sun - One of the best experts on this subject based on the ideXlab platform.

  • neonatal systemic Juvenile Xanthogranuloma with an ominous presentation and successful treatment
    Clinical Medicine Insights: Oncology, 2011
    Co-Authors: Rong Fan, Jihong Sun
    Abstract:

    This case report originated from a case of neonatal multisystemic Juvenile Xanthogranuloma (JXG). The patient presented with blue muffin rush, cervical mass, bone destruction, lung nodule, hepatosplenomegaly, and coagulopathy and was successfully treated with Langerhans cell histiocytosis (LCH) based chemotherapy treatment. Similar cases in literature were reviewed and it seems that JXG, a relatively benign entity, when presented in its systemic form with liver involvement, could have an aggressive course and portend quite poor prognosis. Challenges and special consideration of the diagnosis, treatment, and future case observation are discussed.

Sanjay Behari - One of the best experts on this subject based on the ideXlab platform.

  • solitary Juvenile Xanthogranuloma of the cervical spine in a child a case report and review of literature
    Asian journal of neurosurgery, 2015
    Co-Authors: Kamlesh Singh Bhaisora, A K Jaiswal, Anant Mehrotra, Rabi Narayan Sahu, Arun Srivastava, Sushila Jaiswal, Sanjay Behari
    Abstract:

    The authors present a case of 15 years male child who presented with neck pain and progressive ascending quadriparesis. Magnetic resonance imaging showed lytic mass involving C5 and C6 vertebra with soft tissue extension. Surgical excision of mass done using anterior cervical approach. Postoperatively, patient showed improvement in spasticity and power. Histopathological examination of mass was suggestive of Juvenile Xanthogranuloma (JXG). At 6 months follow-up, patient was improving without any evidence of recurrence. Only 12 cases of JXG of spine have been reported till date including only four cases involving the cervical spine and among these four cases only two were of pediatric age group.

Kenneth L Mcclain - One of the best experts on this subject based on the ideXlab platform.

  • histiocytic diseases of neonates langerhans cell histiocytosis rosai dorfman disease and Juvenile Xanthogranuloma
    Clinics in Perinatology, 2021
    Co-Authors: Kenneth L Mcclain
    Abstract:

    Langerhans cell histiocytosis, Rosai-Dorfman disease, and Juvenile Xanthogranuloma may present at birth or any time afterward. Some patients have minimal skin or lymph node involvement, but others present with life-threatening pulmonary, hepatic, bone marrow, or central nervous system lesions. There is often a delay in diagnosis because of confusing overlap with more common neonatal diseases. Many treatment regimens have been applied to these diseases, but those directed at myeloid cells, such as cytarabine and clofarabine or mutation-targeting inhibitors, are gaining favor. This article provides information on the pathophysiology, clinical presentation, evaluation guidelines, and treatment of these uncommon tumors of neonates.

Rong Fan - One of the best experts on this subject based on the ideXlab platform.

  • neonatal systemic Juvenile Xanthogranuloma with an ominous presentation and successful treatment
    Clinical Medicine Insights: Oncology, 2011
    Co-Authors: Rong Fan, Jihong Sun
    Abstract:

    This case report originated from a case of neonatal multisystemic Juvenile Xanthogranuloma (JXG). The patient presented with blue muffin rush, cervical mass, bone destruction, lung nodule, hepatosplenomegaly, and coagulopathy and was successfully treated with Langerhans cell histiocytosis (LCH) based chemotherapy treatment. Similar cases in literature were reviewed and it seems that JXG, a relatively benign entity, when presented in its systemic form with liver involvement, could have an aggressive course and portend quite poor prognosis. Challenges and special consideration of the diagnosis, treatment, and future case observation are discussed.

Artina Pajaziti - One of the best experts on this subject based on the ideXlab platform.

  • Juvenile Xanthogranuloma: a case report and review of the literature
    BMC research notes, 2014
    Co-Authors: Laura Pajaziti, Syzana Rexhepi Hapçiu, Artina Pajaziti
    Abstract:

    Juvenile Xanthogranuloma is a rare disorder which may be present at birth, or appears in infancy. It can also occur in adults of all ages; appears with lesions that may be solitary or multiple nodules several millimeters in diameter. The predilection sites are head and neck, but it may occur on the extremities and trunk also. There can also be involved internal organs such as lung, kidney, gastrointestinal tract, etc. The most frequent extracutaneous location is the eye. We report a case of Juvenile Xanthogranuloma in a male child with onset in the fourth month of life. He presented with a nodule 8 millimeters in diameter, tan-orange in color, ulcerated in the centre, located on the left corner of the left eye. A biopsy without total excision was performed. After the biopsy, the nodule enlarged to 1.5 cm in diameter and became haemorrhagic. The histologic evaluation and immunohistochemistry analysis resulted in the diagnosis of Juvenile Xanthogranuloma. For aesthetic reasons the nodule was removed by surgical resection. Juvenile Xanthogranuloma is on a spectrum of histiocytic disorders, which is necessary to differentiate from maligniances in childhood by biopsy.