Cases reported "Xanthogranuloma, Juvenile"

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1/9. Verruciform xanthoma associated with squamous cell carcinoma.

    Verruciform xanthoma (VX) is a rare lesion of unknown etiology that is typically solitary and predominantly located within the oral cavity. Less commonly, they arise on the skin, with the majority of cases occurring in anogenital sites. They can be confused clinically with verruca vulgaris, condyloma, leukoplakia, verrucous carcinoma, and squamous cell carcinoma. Histologic features include acanthosis with uniform elongation of the rete ridges and xanthomatous cells that lie in and are typically confined to the papillary dermis. Although epidermal atypia is not a characteristic finding, we describe an unusual case of VX that has features of both VX and squamous cell carcinoma. In addition, there was a VX with typical histologic characteristics located at a separate site in the same patient. This case is also the first to our knowledge to be reported on the neck and axilla and is the third case associated with cutaneous graft versus host disease secondary to bone marrow transplant for acute lymphoblastic leukemia.
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ranking = 1
keywords = leukemia
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2/9. Juvenile xanthogranuloma of the nasal cavity.

    Juvenile xanthogranuloma (JXG) is the most common non-Langerhans histiocytosis, but it rarely occurs extracutaneously. It sometimes presents with associated neurofibromatosis and Juvenile chronic myalogenous leukemia. We present a case of nasal JXG and discuss the histological characteristics, the classification, and the management.
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ranking = 1
keywords = leukemia
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3/9. Juvenile myelomonocytic leukemia presenting with features of hemophagocytic lymphohistiocytosis in association with neurofibromatosis and juvenile xanthogranulomas.

    An association exists among neurofibromatosis 1 (NF1), juvenile xanthogranulomas (JXGs), and juvenile myelomonocytic leukemia (JMML). The authors describe a patient with the triple association of JXG, NF1, and JMML initially presenting with features of hemophagocytic lymphohistiocytosis (HLH). An 18-month old boy had multiple cutaneous and gastrointestinal JXG and NF1. At 3 years of age he developed anemia, thrombocytopenia, and hepatosplenomegaly. A bone marrow biopsy revealed features of HLH. Despite chemotherapy, he went on to develop JMML, which proved fatal.
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ranking = 3649.0240991601
keywords = juvenile myelomonocytic leukemia, juvenile myelomonocytic, myelomonocytic leukemia, myelomonocytic, leukemia
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4/9. Prolonged severe pancytopenia preceding the cutaneous lesions of juvenile xanthogranuloma.

    We report a case of juvenile xanthogranuloma (JXG) having progressive pancytopenia for 6 months until the proliferating skin lesions. A 2-month-old infant presented recurrent fever, anemia, and hepatosplenomegaly mimicking hemophagocytic lymphohistiocytosis (HLH) or juvenile myelomonocytic leukemia (JMML). At 8 months of age, the biopsy of a growing papule on the elbow made the diagnosis. bone marrow (BM) specimens showed clustering foamy cells including hemophagocytosis by histiocytes. Treatment with etoposide followed by vinblastine plus prednisolone (PSL) therapy improved the disease. Although JXG is a benign non-Langerhans cell histiocytosis, the multisystem-visceral form should be considered as a potential aggressive disease when associated with BM failure in early infancy.
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ranking = 3353.6040911245
keywords = juvenile myelomonocytic leukemia, juvenile myelomonocytic, myelomonocytic leukemia, myelomonocytic, leukemia
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5/9. Juvenile chronic granulocytic leukemia, juvenile xanthogranulomas, and neurofibromatosis. Case report and review of the literature.

    A 22-month-old boy had xanthomatous skin lesions, neurofibromatosis, and chronic granulocytic leukemia. Histologic examination of the xanthomatous skin lesions disclosed juvenile xanthogranulomas. Twenty-three previously published cases of this association are reviewed.
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ranking = 5
keywords = leukemia
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6/9. Management of plexiform neurofibroma of the larynx.

    We report the ninth case of plexiform neurofibroma of the larynx, which occurred in a 2-year-old with multiple cafe au lait spots and obstructive sleep apnea. In discussing this clinical problem, we have attempted to make the following points. A patient's having more than six cafe au lait spots of greater than 1.5-cm diameter is diagnostic of von Recklinghausen's disease. It is much more difficult to completely excise plexiform neurofibroma than nonplexiform neurofibroma. The association of juvenile xanthogranuloma with von Recklinghausen's disease may be a risk factor for the later development of leukemia.
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keywords = leukemia
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7/9. association of juvenile xanthogranuloma with juvenile myeloid leukemia.

    Multiple cutaneous xanthomas developed in a patient at the age of 10 months, and juvenile chronic myeloid leukemia (JCML) developed at the age of 30 months. The xanthomas were histopathologically consistent with a diagnosis of juvenile xanthogranuloma (JXG). A review of other cases of JCML with JXG indicates that the cutaneous lesions have many clinical and histopathologic similarities to sporadic JXG but are more often multiple or papular and confluent. In addition to JXG, a few children with JCML also have multiple cafe-au-lait spots and a family history of neurofibromatosis.
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ranking = 5
keywords = leukemia
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8/9. Juvenile xanthogranuloma and acute leukemia: a case report.

    Juvenile xanthogranuloma (JXG) is a rare benign disease of the skin. It is seen in combination with juvenile chronic myelo-monocytic leukemia (JCML) and/or neurofibromatosis type 1 (NF1). The association with acute lymphoblastic leukemia is hardly mentioned in the literature. A case report of this rare combination is described and a review of the literature is given.
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ranking = 6
keywords = leukemia
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9/9. Sporadic acute lymphocytic leukemia arising in a patient with neurofibromatosis and xanthogranulomatosis.

    We present a case report of a child who developed acute lymphoblastic leukemia, neurofibromatosis, optic glioma, and xanthogranulomatosis. This complex is unusual, not previously described, and appears to be a coincidence of different diseases. The importance of this case is that it may offer a clue to the genetic base of neurofibrosis syndromes including leukemic associations.
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ranking = 5
keywords = leukemia
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