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Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG) family lesions, and Rosai-Dorfman-Destombes disease (RDD) are now classified by the World Health Organization (WHO) under the heading of histiocytic/dendritic cell neoplasms. Each disease may manifest as a focal lesion, as multiple lesions, or as a widespread aggressive systemic disease with visceral organ involvement. Erdheim-Chester disease (ECD) is a rare systemic disease process of adults with limited cases in children.
View Article and Find Full Text PDFHistiocytic neoplasms: a brief review and differential diagnosis.
J Clin Exp Hematop
September 2024
Department of Cell Pathology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
Article Synopsis
- - Histiocytic neoplasms (HNs) encompass various diseases like juvenile xanthogranuloma and Erdheim-Chester disease, which are recognized in the WHO's latest classification.
- - These diseases have unique clinical and histological characteristics, but they share a common feature of histiocytic cell proliferation, leading to similarities in appearance.
- - The review summarizes clinical data, molecular aspects, histological features, and immunophenotyping of HNs, while also addressing the need for differentiating them from other similar conditions.
FEATURES OF DIAGNOSIS AND ADVERSE COURSE OF NEONATAL JUVENILE XANTHOGRANULOMA: CASE REPORT.
Exp Oncol
December 2022
Danylo Halytsky Lviv National Medical University, Lviv 79010, Ukraine.
The work describes a case of rare neonatal systemic juvenile xanthogranuloma with an initial damage of the scalp, limbs, back and abdomen, multiple damages of the parenchyma of both lungs, spleen and liver with the development of a severe form of congenital cholestatic hepatitis. The diagnosis was established on the basis of histopathological and immunohistochemical examination of the skin nodules. The child on the background of therapy under the Langerhans cell histiocytosis III program achieved a partial response, which was manifested by a reduction of granulomatous formations on the skin, elimination of liver failure, but retained hepatosplenomegaly, specific lesions of the lung parenchyma, liver, and left kidney.
View Article and Find Full Text PDFJuvenile and adult xanthogranuloma: A 30-year single-center experience and review of the disorder and its relationship to other histiocytoses.
Ann Diagn Pathol
June 2022
Lauren V. Ackerman Laboratory of Surgical Pathology, Barnes Jewish/St. Louis Children's Hospitals, Washington University Medical Center, St. Louis, MO, USA. Electronic address:
Background: Juvenile xanthogranuloma (JXG) is the most common type of non-Langerhans cell histiocytosis whose cell of origin, etiology and pathogenesis are not fully understood. We aimed to provide an update on histopathologic and immunophenotypic profile of this well-characterized entity whose relationship to the other histiocytoses has received renewed attention in light of recent molecular genetic studies.
Materials And Methods: A retrospective review of all the cases with the pathologic diagnosis of "xanthogranuloma" was performed on our archives from 1989 to 2019.
Symmetrical Facial Giant Plaque-Type Juvenile Xanthogranuloma: Case Report and Review of the Literature.
Case Rep Dermatol
July 2021
Department of Dermatology, University Hospital Basel, Basel, Switzerland.
Juvenile xanthogranuloma (JXG) is the most common type of non-Langerhans cell histiocytosis. JXG is a rare benign tumor, which may be present at birth or develop later. The classical form of JXG is characterized by a red-yellowish benign papule or nodule with predilection sites on the head, neck, and trunk, although lesions can appear on extremities or extracutaneous sites.
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