1,301 results match your criteria Juvenile Xanthogranuloma Nevoxanthoendothelioma

Cyclin D1 expression and molecular genetic findings in periocular histiocytoses and neoplasms of macrophage-dendritic cell lineage.

Am J Ophthalmol 2022 May 17. Epub 2022 May 17.

Department of Pathology, Wills Eye Hospital, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, PA, USA; Department of Ophthalmology, Wills Eye Hospital, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, PA, USA.

Purpose: Frequent activating mutations in the mitogen-activated protein kinase (MAPK) pathway genes have been identified in histiocytoses. MAPK signaling consistently upregulates Cyclin D1. The goal of this study was to determine whether Cyclin D1 expression by immunohistochemistry is a useful diagnostic marker for periocular histiocytoses and to further characterize their genetic basis. Read More

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Juvenile Xanthogranuloma as Differential Diagnosis of a Vulvar Mass: A Case Report.

European J Pediatr Surg Rep 2022 Jan 19;10(1):e25-e29. Epub 2022 Apr 19.

Department of Pediatric Surgery, Hospital General Universitario Gregorio Marañón, Madrid, Spain.

Vulvar masses in children are an unusual finding but their differential diagnosis is extensive. In case of solid masses, rhabdomyosarcoma (RMS) must always be considered due to the fact that it is the most common tumor in external genitals during childhood. However, RMS has a radiological appearance very similar to juvenile xanthogranuloma (JXG). Read More

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January 2022

What is the Role of Different Macrophage Subsets in the Evolution of Juvenile Xanthogranulomas?

Appl Immunohistochem Mol Morphol 2022 Apr 18. Epub 2022 Apr 18.

Departments ofPathology.

There are several activated forms of macrophages: 2 main groups are designated M1 and M2. While M1 macrophages have proinflammatory, bactericidal, and phagocytic functions and are the dominant phenotype observed in the early stages of inflammation, M2 macrophages are involved in constructive processes such as tissue repair; they play a role in wound healing and are required for revascularization and re-epithelialization. Juvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytosis. Read More

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Giant Juvenile Xanthogranuloma of face in an adult: A rare complexity.

J Stomatol Oral Maxillofac Surg 2022 Apr 6. Epub 2022 Apr 6.

Department of Oral & Maxillofacial Surgery, Faculty of Oral and Dental Medicine, Cairo University, Egypt.

Juvenile xanthogranuloma, a form of non-Langerhans cell histiocytosis can be defined as the proliferation of cells with macrophage like characteristics. It has been described as a benign, asymptomatic and common self-healing disorder of non-Langerhans cell histiocytosis (LCH), affecting mostly infants, children and rarely adults. We have documented a case of a 40 year old male who presented to us with extensive insidious papulonodulous growth over the face. Read More

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Juvenile and adult xanthogranuloma: A 30-year single-center experience and review of the disorder and its relationship to other histiocytoses.

Ann Diagn Pathol 2022 Jun 30;58:151940. Epub 2022 Mar 30.

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. Read More

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Juvenile Xanthogranuloma: A Comparative Immunohistochemical Study of Factor XIIIa, CD11c, and CD4.

Am J Dermatopathol 2022 Mar 22. Epub 2022 Mar 22.

Lauren V. Ackerman Laboratory of Surgical Pathology, Barnes/St. Louis Children's Hospitals, Washington University Medical Center, St. Louis, MO.

Abstract: Juvenile xanthogranuloma is a group C and L non-Langerhans cell histiocytosis, and its cell of origin is still debatable. The expression of CD11c, a more recently described macrophage marker, and CD4 have not been studied comprehensively. This study aimed to expand immunophenotypic profile and hence our understanding of the origin of these lesions. Read More

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Intralesional glucocorticoid treatment of an isolated intracranial juvenile xanthogranuloma: a case report.

Childs Nerv Syst 2022 Mar 18. Epub 2022 Mar 18.

Department of Neurosurgery, School of Medicine & Public Health, University of Wisconsin, Madison, WI, USA.

Juvenile xanthogranuloma is a type of non-Langerhans cell histiocytic process that appears primarily in children and is described as a benign lesion. Although they typically present as a cutaneous lesion, it can also present in other areas including within the central nervous system. We report a 6-month-old infant who presented with seizure-like activity who was found to have a single intracranial mass within the right temporal area on magnetic resonance imaging of the head. Read More

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Solitary juvenile xanthogranuloma of temporal bone: a case report.

Shu-Ni Wang Ji Lu

BMC Pediatr 2022 02 12;22(1):87. Epub 2022 Feb 12.

The Department of Radiology, The First College of Clinical Medical Science of China Three Gorges University and Yichang Central People's Hospital, Yichang, 443000, Hubei, China.

Background: Juvenile xanthogranuloma (JXG) is a kind of non-Langerhans cell histiocytosis, usually with skin lesions as the main manifestation. It rarely occurs in other tissues or organs and even more rarely is it found in the skull. Here, we report a case of xanthogranuloma derived from the temporal bone that was not present in any other parts of the body. Read More

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February 2022

What Is a Juvenile Xanthogranuloma?


Pediatr Dermatol 2022 Jan;39(1):119-120

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January 2022

Role of FDG PET/CT in the Evaluation of Therapy Response in Systemic Juvenile Xanthogranuloma.

Clin Nucl Med 2022 May;47(5):e395-e396

From the Department of Nuclear Medicine, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Abstract: Juvenile xanthogranuloma, a rare type of non-Langerhans cell histiocytosis, is mostly seen in childhood and adolescence and generally manifests as widespread skin lesions. It rarely shows systemic involvement. Although the cutaneous form is often self-limited, systemic form is quite aggressive. Read More

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On the knowledge of solitary juvenile xanthogranuloma of the eyelid: a case series and literature review.

Graefes Arch Clin Exp Ophthalmol 2022 Jan 27. Epub 2022 Jan 27.

State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Guangdong Provincial Clinical Research Center for Ocular Diseases, Guangzhou, 510060, China.

Purpose: Solitary eyelid juvenile xanthogranuloma (JXG) is extremely rare, and there is limited literature on its clinical features and treatment outcomes. Here, we present a case series and comprehensive review of the literature on patients with isolated eyelid JXG.

Methods: We systematically extracted data from our institution's records of isolated eyelid JXG cases and conducted a search for additional cases from the literature utilising the PubMed, Wanfang, and Chinese National Knowledge Infrastructure (CNKI) databases. Read More

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January 2022

Firm, enlarging papule on an infant's proximal thigh.

JAAD Case Rep 2022 Jan 10;19:78-80. Epub 2021 Nov 10.

Department of Dermatology, Baylor College of Medicine, Houston, Texas.

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January 2022

Cutaneous histiocytoses in children.

Histopathology 2022 Jan;80(1):196-215

Department of Pathology, Ambroise Paré Hospital, AP-HP, Boulogne, France.

Cutaneous histiocytoses constitute a heterogeneous group of diseases characterised by the cutaneous accumulation of cells with the cytological and phenotypic features of macrophages or dendritic cells. The clinical spectrum ranges from self-resolving, skin-limited conditions to severe, multiorgan disease with a high morbidity rate. Until recently, cutaneous histiocytoses were classified according to the immunophenotype of the pathological cells, with differentiation between Langerhans cell histiocytosis (LCH) [CD1a+, CD207 (langerin)+] and non-Langerhans cell histiocytosis (CD68+, CD163+, CD1a-, CD207-). Read More

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January 2022

Large Congenital Juvenile Xanthogranuloma in a Two-Month-Old Girl, a Case of Non-Langerhans Cell Histiocytosis.

Acta Med Acad 2021 08;50(2):304-305

Department of Pediatric Surgery, University General Hospital of Alexandroupoli, School of Medicine, Democritus University of Thrace, Alexandroupoli, Greece.

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Pediatric liver failure with massive sinusoidal infiltration of histiocytes.

J Clin Exp Hematop 2022 Mar 26;62(1):25-34. Epub 2021 Nov 26.

Department of Clinical Research, Saitama Children's Medical Center, Saitama, Japan.

Histiocytic neoplasms, such as Langerhans cell histiocytosis (LCH) and disseminated juvenile xanthogranuloma (JXG), can involve the liver and sometimes cause liver failure. We aimed to classify non-LCH histiocytic proliferating disorders that do not exhibit typical disseminated JXG histology. We examined four pediatric patients who presented with liver failure and splenomegaly. Read More

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Juvenile xanthogranulomas in Asian children.

Dermatol Ther 2022 02 4;35(2):e15224. Epub 2021 Dec 4.

Dermatology Service, KK Women's and Children's Hospital, Singapore, Singapore.

Juvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytic disorder. It can rarely be associated with systemic involvement. There is a paucity of literature on JXG in Asian children. Read More

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February 2022

Utility of Cyclin D1 Immunostaining in Cutaneous Xanthogranuloma.

Am J Dermatopathol 2021 Dec;43(12):e141-e145

Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH.

Abstract: Mitogen-activated protein kinase (MAPK)/extracellular signal-regulated kinase pathway activation has recently been found to be associated with almost all Langerhans cell histiocytosis cases. In BRAF V600E mutation-positive cases, this activation is seen as a downstream activation. In addition, cyclin D1 is a downstream target of the MAPK pathway. Read More

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December 2021

Multicentric reticulohistiocytosis with dermatomyositis-like features: A case report with dermoscopy and reflectance confocal microscopy findings.

J Cutan Pathol 2022 Apr 12;49(4):388-392. Epub 2021 Dec 12.

Dermatology Department, Rutgers University, Robert Wood Johnson Medical Center, Somerset, New Jersey, USA.

Multicentric reticulohistiocytosis (MRH) is an insidious-onset, non-Langerhans-cell histiocytosis (NLCH) affecting the joints and skin. Early diagnosis is important to prevent destructive arthritis and disease-related complications. Reflectance confocal microscopy (RCM) is a technique that allows the visualization of the epidermis and superficial dermis noninvasively on a horizontal plane with quasi-histologic images of the skin. Read More

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Neoadjuvant intralesional methotrexate for juvenile xanthogranuloma in an adult.

Dermatol Ther 2022 01 16;35(1):e15200. Epub 2021 Nov 16.

Department of Dermatology, University Clinic of Navarra, School of Medicine, University of Navarra, Pamplona, Spain.

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January 2022

[Atypical inflammmatory presentation of an iris juvenile xanthogranuloma in a 10-month-old girl].

J Fr Ophtalmol 2020 Oct 23. Epub 2020 Oct 23.

Service d'ophtalmologie, Hôtel-Dieu, CHU de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes cedex 1, France.

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October 2020

Oral juvenile xanthogranuloma: An unusual presentation in an adult patient and a systematic analysis of published cases.

Oral Surg Oral Med Oral Pathol Oral Radiol 2022 01 8;133(1):42-49. Epub 2021 Oct 8.

Head and Neck Department, Fondazione Policlinico Universitario A Gemelli IRCCS, Roma, Università Cattolica del Sacro Cuore, Rome, Italy.

Objective: The purpose of this study was to describe an unusual case of intraoral juvenile xanthogranuloma (JXG) and to perform a systematic review to investigate the available literature regarding oral JXGs.

Study Design: We present a new case of oral JXG arising in a 36-year-old Italian woman and conducted a systematic literature review in PubMed, Web of Science, and Scopus, according to the PRISMA guidelines.

Results: Our review of the English-language literature yielded 34 cases of oral JXG, which included our case report. Read More

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January 2022

Ophthalmic histiocytic lesions: a baseline demographic and clinicopathological study of 28 cases from two eye centers.

Int Ophthalmol 2022 Apr 2;42(4):1221-1232. Epub 2021 Nov 2.

Pathology and Laboratory Medicine Department, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia.

Purpose: Ophthalmic histiocytic lesions comprise a heterogeneous rare group of disorders that are characterized by an abnormal proliferation of histiocytes and may affect all age groups of both sexes. The aim of this study was to highlight the basic demographic, clinical, and histopathological characteristics of this rare group of diseases in ophthalmic practice, which has not been previously studied in this area. Only individual cases have been previously reported. Read More

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Skin anomalies in acromegalic patients (Review of the practical aspects).

Exp Ther Med 2021 Nov 20;22(5):1330. Epub 2021 Sep 20.

Department of Endocrinology, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania.

Acromegaly is a hormonal disorder which occurs as the result of growth hormone (GH) and insulin growth factor 1 (IGF-1) over-secretion; both hormones are related to skin anomalies. The skin acts as a large endocrine organ, hosting GH receptors in every cell while IGF-1 receptors are expressed only in keratinocytes. This review is a literature review of skin anomalies found in acromegaly, either related to the disease itself or associated with related complications such as secondary diabetes mellitus, or involving associated conditions such as genetic syndromes. Read More

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November 2021

Histiocytic disorders.

Nat Rev Dis Primers 2021 10 7;7(1):73. Epub 2021 Oct 7.

Texas Children's Cancer Center, Department of Paediatrics, Baylor College of Medicine, Houston, TX, USA.

The historic term 'histiocytosis' meaning 'tissue cell' is used as a unifying concept for diseases characterized by pathogenic myeloid cells that share histological features with macrophages or dendritic cells. These cells may arise from the embryonic yolk sac, fetal liver or postnatal bone marrow. Prior classification schemes align disease designation with terminal phenotype: for example, Langerhans cell histiocytosis (LCH) shares CD207 antigen with physiological epidermal Langerhans cells. Read More

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October 2021

Adult xanthogranuloma on penis: a case report.

Int J Dermatol 2022 Mar 30;61(3):e103-e105. Epub 2021 Sep 30.

Department of Dermatology, Chongqing Hospital of Traditional Chinese Medicine, Chongqing, China.

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Clustered papulonodular lesions in an infant.

Clin Exp Dermatol 2022 Jan 29;47(1):216-219. Epub 2021 Sep 29.

Department of Dermatology, Venereology and Leprology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

Non-Langerhans cell histiocytosis is a collective term encompassing a vast group of benign proliferative disorders of histiocytes, macrophages and dendritic cells that do not meet the criteria of Langerhans cell histiocytosis. We describe a case of juvenile xanthogranuloma with an unusual clustered distribution. Read More

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January 2022

Refractory Extracutaneous Juvenile Xanthogranuloma With Multiple Intracranial Nodular Lesions Successfully Treated With 2-Chlorodeoxyadenosine.

J Pediatr Hematol Oncol 2022 Apr;44(3):e823-e825

Department of Pediatrics, Children's Cancer Center, National Center for Child Health and Development, Tokyo, Japan.

Juvenile xanthogranulomatosis (JXG) is a rare histiocytic disease that is usually limited to the skin, but some JXG cases involve other organs. JXG involving the central nervous system (CNS) is rare and its treatment is inadequate. The optimum treatment for refractory JXG involving the CNS remains unknown. Read More

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Diagnostic challenges in fine-needle aspiration of a jaw lesion.

Diagn Cytopathol 2021 Oct 31;49(10):1141-1143. Epub 2021 Aug 31.

Department of Pathology, University of Tennessee Graduate School of Medicine, Knoxville, Tennessee, USA.

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October 2021

What to Look Out for in a Newborn with Multiple Papulonodular Skin Lesions at Birth.

Dermatopathology (Basel) 2021 Aug 17;8(3):390-417. Epub 2021 Aug 17.

Department of Dermatology, Necker-Enfants Malades Hospital, APHP, 75015 Paris, France.

Multiple papulonodular skin lesions at birth can indicate the presence of various benign and malignant disorders. Although the lesions' clinical aspect (color and consistency, in particular) may steer the clinician towards one disorder or another (infantile myofibromatosis, xanthogranuloma, or metastatic neuroblastoma), the diagnosis can only be confirmed by the histopathologic assessment of a biopsy. In neonates, a rapid but accurate diagnosis is critical because skin lesions may be the first manifestation of a malignant disorder like leukemia cutis or metastatic neuroblastoma. Read More

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