1,119 results match your criteria Juvenile Xanthogranuloma Nevoxanthoendothelioma


Solitary juvenile xanthogranuloma in the spine pretreated with neoadjuvant denosumab therapy followed by surgical resection in a 5-year-old child: case report and literature review.

Eur Spine J 2018 Jun 6. Epub 2018 Jun 6.

Department of Orthopedics and Traumatology, Unit of Musculoskeletal Surgery, Tampere University Hospital, Tampere, Finland.

Purpose: We present a case report that describes neoadjuvant denosumab therapy initiated in a child with a solitary giant cell-rich juvenile xanthogranuloma tumor involving the spine, and review the current literature.

Methods: A giant cell-rich histiocytic lesion involving the 11th thoracic vertebral body was identified in a healthy 5-year-old girl with persistent back and pelvic pain for several months. Imaging examinations and an open biopsy were performed to obtain a definite pathologic diagnosis. Read More

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June 2018
2 Reads

Rare childhood hybrid histiocytosis of the central nervous system-diagnosed by stereotactic brain biopsy with marked treatment response to clofarabine.

Childs Nerv Syst 2018 Jun 5. Epub 2018 Jun 5.

Department of Neurosurgery, Rigshospitalet University Hospital, Blegdamsvej 9, 2100, Copenhagen, Denmark.

Histiocytosis is a heterogeneous group of disease entities, comprised by two main categories, namely Langerhans and non-Langerhans cell histiocytoses. Central nervous system involvement in histiocytosis is considered very rare and is often secondary to affection of anatomically related bone structures and/or multi-organ disease. We present a never-before described case of rare childhood histiocytosis with hybrid features of Langerhans cell histiocytosis and juvenile xanthogranuloma confined to the central nervous system in a 2- and a half-year-old boy with distinct treatment response to clofarabine. Read More

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June 2018
2 Reads

Emperipolesis and S100 expression may be seen in cutaneous xanthogranulomas: A multi-institutional observation.

J Cutan Pathol 2018 May 24. Epub 2018 May 24.

Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire.

Cutaneous Rosai-Dorfman disease (RDD) can be difficult to distinguish from other non-Langerhans cell histiocytoses, particularly xanthogranuloma (XG). Pathologists use S100 immunoreactivity, abundant plasma cells, and the presence of emperipolesis to distinguish RDD from XG. However, S100 expression has been reported in XG and, in practice, we have occasionally observed emperipolesis in cases that were otherwise clinically and pathologically consistent with XG. Read More

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May 2018
2 Reads

Intracerebral juvenile xanthogranuloma: an infrequent involvement of hystiocitosis in children.

Childs Nerv Syst 2018 May 25. Epub 2018 May 25.

Department of Neurosurgery, Hospital Universitario Donostia, San Sebastián, Guipúzcoa, Spain.

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May 2018
1 Read

Giant Juvenile Xanthogranuloma: Case Report, Literature Review, and Algorithm for Classification.

J Cutan Med Surg 2018 May 1:1203475418777734. Epub 2018 May 1.

1 Division of Dermatology, Department of Medicine, University of Calgary, Calgary, AB, Canada.

Juvenile xanthogranuloma (JXG) is a member of the non-Langerhans cell group of proliferative disorders of mononuclear phagocytes. JXG is a benign tumour of histiocytic cells. Classic JXG is divided into 2 main clinical subtypes: dome-shaped papules (<0. Read More

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Juvenile Xanthogranuloma in a Pediatric Patient with Langerhans Cell Histiocytosis.

Ocul Oncol Pathol 2018 Apr 4;4(3):141-144. Epub 2017 Oct 4.

Department of Ophthalmology, Baylor College of Medicine, Houston, TX, USA.

Purpose: To report a case of juvenile xanthogranuloma that simulated a chalazion and to discuss the association between juvenile xanthogranuloma and Langerhans cell histiocytosis.

Method: Case report and review of literature.

Results: A 13-year-old boy with a prior history of Langerhans cell histiocytosis was referred to our clinic for a possible chalazion. Read More

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April 2018
3 Reads

Expression of the transcription factor ZBTB46 distinguishes human histiocytic disorders of classical dendritic cell origin.

Mod Pathol 2018 May 9. Epub 2018 May 9.

Department of Pathology, Stanford University School of Medicine, Stanford, CA, 94305, USA.

Distinguishing classical dendritic cells from other myeloid cell types is complicated by the shared expression of cell surface markers. ZBTB46 is a zinc finger and BTB domain-containing transcription factor, which is expressed by dendritic cells and committed dendritic cell precursors, but not by plasmacytoid dendritic cells, monocytes, macrophages, or other immune cell populations. In this study, we demonstrate that expression of ZBTB46 identifies human dendritic cell neoplasms. Read More

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Extracutaneous variant of juvenile xanthogranuloma in an adult patient associated to follicular lymphoma.

Med Clin (Barc) 2018 Apr 20. Epub 2018 Apr 20.

Anatomía Patológica, Hospital Infanta Margarita, Cabra, Córdoba, España.

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Dermoscopy in the diagnosis of juvenile xanthogranuloma.

An Bras Dermatol 2018 Jan-Feb;93(1):138-140

Dermatology Department at Hospital Santa Casa de Curitiba - Curitiba (PR), Brazil.

Juvenile xanthogranuloma is the most common form of non-Langerhans cell histiocytosis. It manifests clinically as a red-yellow papule, usually showing spontaneous remission. The diagnosis is based on clinical and histological findings. Read More

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April 2018
2 Reads

Oral juvenile xanthogranuloma in a child: Clinical, histological and immunohistochemical profile of a rare entity.

J Cutan Pathol 2018 Jul 9;45(7):515-521. Epub 2018 May 9.

Oral Pathology Section, Universidad Autónoma de Guadalajara (UAG), Zapopan, Mexico.

Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis (non-LCH) affecting normolipemic infants and children most frequently in the first year of life, often showing spontaneous regression within 3 to 6 years. Classic JXG is characterized by a yellowish asymptomatic papule or nodule, often located in the skin of the head, neck and upper trunk. Oral JXG has been reported, but is rare. Read More

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July 2018
2 Reads

Cutaneous Hematolymphoid and Histiocytic Proliferations in Children.

Pediatr Dev Pathol 2018 Mar-Apr;21(2):208-251

2 Lauren V. Ackerman Laboratory of Surgical Pathology, St. Louis Children's Hospital and Dermatopathology, Washington University Medical Center, St. Louis, Missouri.

This article focuses on cutaneous hematopoietic neoplasms that are more likely to be encountered in the pediatric age-group and includes both lymphoproliferative and histiocytic disorders. The cutaneous hematologic disorders in children have a different epidemiologic profile to what is seen during adulthood. Although mycosis fungoides is the most frequent form of cutaneous lymphoma in adults, it is very rare in children. Read More

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April 2018
3 Reads

[Juvenile xanthogranuloma of the eye].

Vestn Oftalmol 2018 ;134(1):89-96

Kemerovo Regional Clinical Ophthalmological Hospital, Oktyabrsky pr., 22А, Kemerovo, Russian Federation, 650066.

Juvenile xanthogranuloma (JX) is a benign growth of fibrohistiocytoma range that mainly affects children. The most frequent localization of the tumor is skin integument. Non-cutaneous forms of JX are found in 5-10% of cases; they are manifested as deep lesions of soft tissues, involvement of various organs (oropharynx, lungs, liver, spleen, pericardium, gastrointestinal tract, central nervous system, bone marrow) including the organ of vision. Read More

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January 2018
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Pediatric Case of Xanthogranuloma in the Sellar Region Presenting a Visual Disturbance Successfully Treated with Endoscopic Endonasal Surgery.

World Neurosurg 2018 Jun 7;114:22-26. Epub 2018 Mar 7.

Department of Neurosurgery, Nara Medical University, Nara, Japan.

Background: Xanthomatous pituitary diseases rarely occur in childhood. We report a rare pediatric case of a xanthogranuloma that developed in the sellar region, resulting in a visual disturbance that was treated successfully with endoscopic endonasal surgery.

Case Description: A 13-year-old boy came to us with a headache and visual disturbance that occurred 1 month prior. Read More

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June 2018
2 Reads

Solitary Intraspinal Juvenile Xanthogranuloma in an Infant.

Asian J Neurosurg 2018 Jan-Mar;13(1):172-175

Department of Pathology, Topiwala National Medical College and B. Y. L. Nair Hospital, Mumbai, Maharashtra, India.

Juvenile xanthogranuloma (JXG) is a benign, non-Langerhans cell histiocytic proliferative disorder. We report a case of solitary JXG in an infant presenting as an intraspinal mass. Awareness of this mode of presentation is very important as subsequent prognosis differs from other tumors at the same location. Read More

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Risk of intraocular and other extracutaneous involvement in patients with cutaneous juvenile xanthogranuloma.

Pediatr Dermatol 2018 May 28;35(3):329-335. Epub 2018 Feb 28.

Division of Dermatology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.

Background/objectives: Cutaneous juvenile xanthogranuloma is an uncommon, usually benign disease affecting infants and young children. Ocular and other systemic involvement have been reported, but their incidence is unclear, and the utility of routine screening is not well established. Our aim was to characterize the risk of ocular and systemic complications in children with cutaneous juvenile xanthogranuloma. Read More

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May 2018
1 Read

Congenital juvenile xanthogranuloma with ulceration: a pediatric case report.

Dermatol Online J 2017 Jul 15;23(7). Epub 2017 Jul 15.

Children's Mercy Hospital, Kansas City, Missouri.

Congenital juvenile xanthogranuloma (JXG) is an uncommon diagnosis and even more rarely presents with ulceration. We report such a case in a two-week-old girl. Biopsy was performed to rule out any concerning entities. Read More

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Juvenile xanthogranuloma involving concurrent iris and skin: Clinical, pathological and molecular pathological evaluations.

Am J Ophthalmol Case Rep 2018 Mar 23;9:10-13. Epub 2017 Sep 23.

Department of Pathology, University Basel, Schönbeinstrasse 40, 4003 Basel, Switzerland.

Purpose: To report a case of juvenile xanthogranuloma involving the iris and skin that clincally was diagnosed with an obvious cutaneous lesion.

Observations: A four month-old girl with hyphema and increased intraocular pressure of the left eye persisting for 2 weeks. A suspicious yellow-brown mass with nodular surface and traversed by irregular vascularization was noted on the inferior iris surface. Read More

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Iris juvenile xanthogranuloma in an infant - spontaneous hyphema and secondary glaucoma.

Rom J Ophthalmol 2017 Jul-Sep;61(3):229-236

Opthalmology Clinic, "Sf. Spiridon" University Hospital, Iași, Romania.

Juvenile xanthogranuloma (JXG) is a benign histiocytic skin disorder mainly encountered during infancy and childhood. Although with multiple potential localizations, less than 1% of the cases exhibit ocular manifestations. Some of these might lead to serious complications, specifically, secondary glaucoma that can result in severe and blinding eye disease. Read More

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February 2018
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Systemic juvenile xanthogranuloma: a case report and brief review.

Clin Exp Dermatol 2018 Feb 10. Epub 2018 Feb 10.

University of Texas Health Science Center at San Antonio, San Antonio, TX, USA.

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February 2018
1 Read

Dermatofibrosarcoma protuberans in pediatric patients: A diagnostic and management challenge.

JAAD Case Rep 2018 Mar 16;4(2):155-158. Epub 2018 Jan 16.

Department of Pediatrics and Medicine, Division of Dermatology, Dell Medical School at the University of Texas at Austin, Austin, Texas.

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Histiocytosis - cutaneous manifestations of hematopoietic neoplasm and non-neoplastic histiocytic proliferations.

J Eur Acad Dermatol Venereol 2018 Jun 31;32(6):926-934. Epub 2018 Jan 31.

Department of Dermatology, University Hospital Zürich, Zürich, Switzerland.

Histiocytoses are rare disorders characterized by the accumulation of cells derived from macrophages, dendritic cells or monocytes in various tissues. There is a broad spectrum of disease manifestations with some subtypes commonly showing skin lesions, while in others, the skin is rarely involved. Here, we describe cutaneous manifestations of histiocytoses belonging to the Langerhans group (L group), the group of cutaneous and mucocutaneous histiocytoses (C group) and the group of Rosai-Dorfman disease (RDD) and related histiocytoses (R group) according to the current classification. Read More

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June 2018
7 Reads

Reply to: "Is the setting sun dermoscopic pattern specific to juvenile xanthogranuloma?"

J Am Acad Dermatol 2018 Feb;78(2):e51-e52

Department of Dermatology, Seoul National University College of Medicine, Seoul, Republic of Korea; Institute of Human-Environment Interface Biology, Seoul National University, Seoul, Republic of Korea. Electronic address:

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February 2018
1 Read

Is the setting sun dermoscopic pattern specific to juvenile xanthogranuloma?

J Am Acad Dermatol 2018 Feb;78(2):e49

Department of Dermatology, Charles Nicolle Hospital, University of Tunis El Manar, Tunis, Tunisia.

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February 2018
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Disseminated juvenile xanthogranuloma occurring after treatment of Langerhans cell histiocytosis: a case report.

Childs Nerv Syst 2018 Apr 5;34(4):765-770. Epub 2017 Dec 5.

Department of Dermatology, Chonnam National University Medical School, Chonnam National University Hwasun Hospital, Jeollanam-do, Republic of Korea.

Case Presentation: An 11-year-old boy presented with a complaint of a painful temporal mass. Brain magnetic resonance imaging (MRI) showed a 3-cm-sized, homogeneously enhancing mass in the greater wing of the left sphenoid bone, which was diagnosed as Langerhans cell histiocytosis (LCH). Chemotherapy with vincristine and prednisolone was performed for 1 year. Read More

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April 2018
6 Reads

A Case of Segmental (Zosteriform) Juvenile Xanthogranuloma.

Ann Dermatol 2017 Dec 30;29(6):806-808. Epub 2017 Oct 30.

Department of Dermatology, Incheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Incheon, Korea.

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December 2017
1 Read

Juvenile xanthogranuloma with angiomatous appearance and a peculiar immunophenotype.

Pediatr Dermatol 2018 Jan 22;35(1):e55-e56. Epub 2017 Nov 22.

Department of Dermatology, Hospital Universitario Fundacion Jiménez Diaz, Universidad Autónoma, Madrid, Spain.

Juvenile xanthogranuloma is the most common form of non-Langerhans cell histiocytosis in childhood. The clinical differential diagnosis of a solitary juvenile xanthogranuloma includes molluscum contagiosum, Spitz nevus, and melanoma. Lesions larger than 2 cm in diameter may be misdiagnosed as hemangiomas, but this is not typical of smaller juvenile xanthogranuloma. Read More

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January 2018
3 Reads

A rare case of intramedullary solitary juvenile xanthogranuloma of the lumbar spine in the adult: a case report.

J Spine Surg 2017 Sep;3(3):504-508

Department of Neurosurgery, S. Maurizio Hospital, Bolzano, Italy.

Solitary juvenile xanthogranuloma (SJX) of the spine is an extremely rare proliferative histiocytic disorder with only few cases reported in literature. We present the first case of intramedullary spinal SJX. A 22-year-old male presented with a nine-month history of progressively worsening sphincteric disturbances and saddle hypoesthesia. Read More

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September 2017
2 Reads

Scalp Lesions in a Pediatric Patient with Hyper IgM Syndrome: Clinical and Histologic Mimicry of Cryptococcus neoformans Infection.

J Pediatr 2018 Jan 28;192:256-258. Epub 2017 Sep 28.

Department of Pediatrics, Columbia University Medical Center, New York, NY; Department of Dermatology, Columbia University Medical Center, New York, NY.

We report a case of cutaneous cryptococcosis due to Cryptococcus neoformans in a pediatric patient with hyper IgM syndrome with scalp lesions that resembled tinea capitis on gross examination and mimicked juvenile xanthogranuloma on histologic examination. This case highlights the importance of considering cutaneous cryptococcosis in patients with hyper IgM syndrome. Read More

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January 2018
3 Reads

Systemic juvenile xanthogranuloma: a case of spontaneous regression of intramedullary spinal cord, cerebral, and cutaneous lesions.

J Neurosurg Pediatr 2017 Dec 29;20(6):556-560. Epub 2017 Sep 29.

Neurosurgery, and.

Juvenile xanthogranuloma (JXG) is a rare disease that belongs to the non-Langerhans cell histiocytoses. It presents a wide clinical spectrum, usually occurs before 5 years of age, and is commonly confined to the skin; however, it can affect multiple sites, including the nervous system, and can lead to severe disorders. Although JXG is a benign disease that usually regresses spontaneously, several curative treatments have been proposed in cases of organ involvement. Read More

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December 2017
15 Reads

Xanthoma-like Skin Changes in an Elderly Woman with a Normal Lipid Profile.

Acta Dermatovenerol Croat 2017 Jul;25(2):167-169

Professor Joanna Maj, MD, PhD, Department and Clinic of Dermatology, Venereology and Allergology, Wroclaw Medical University, Chałubińskiego 1, PL-50-368 Wrocław, Poland;

Dear Editor, An 83-year-old woman developed yellow-brownish infiltrates, nodules, and tumors mimicking xanthomas, mostly involving the periorbital and chest area within three months (Figure 1). She had no abnormalities in serum cholesterol or triglycerides levels. A detailed laboratory analysis revealed the presence of mild monoclonal gammopathy with a presence of immunoglobulin G (IgG) kappa light chains; however, according to hematologist consultation, it did not require medical intervention. Read More

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July 2017
13 Reads

Systemic Juvenile Xanthogranuloma Involving the Bone Marrow, Multiple Bones, and the Skin That Developed During Treatment of Acute Lymphoblastic Leukemia in Remission State.

Pediatr Dev Pathol 2017 Jan 1:1093526617721775. Epub 2017 Jan 1.

1 Department of Pediatrics, 37977 School of Medicine, Ajou University , Suwon, Korea.

Juvenile xanthogranuloma (JXG) is a rare benign disorder classified as non-Langerhans cell histiocytosis, with unclear etiology and pathogenesis. JXG is generally characterized by solitary or multiple cutaneous nodules that resolve spontaneously over a few years. JXG rarely presents as extracutaneous lesions that progress to a symptomatic systemic disorder through multiple organ involvement. Read More

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January 2017
27 Reads

Genetic evaluation of juvenile xanthogranuloma: genomic abnormalities are uncommon in solitary lesions, advanced cases may show more complexity.

Mod Pathol 2017 Sep 28;30(9):1234-1240. Epub 2017 Jul 28.

ARUP Institute for Clinical and Experimental Pathology, Salt Lake City, UT, USA.

Juvenile xanthogranuloma is a rare histiocytic proliferation primarily affecting infants and young children, characterized by aberrant infiltration of histiocyte-derived cells in the skin, soft tissues and more rarely, visceral organs. Juvenile xanthogranuloma is generally considered to be a benign disorder; most lesions are solitary cutaneous nodules that resolve spontaneously without treatment. However, cases with extracutaneous involvement, multiple lesions, and/or systemic disease often require aggressive therapy. Read More

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September 2017
17 Reads

Malignant transformation of multifocal tapioca iris nevus in a child.

J AAPOS 2017 Aug 16;21(4):340-342. Epub 2017 Jun 16.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. Electronic address:

A 31-month-old white boy was evaluated for multiple iris lesions in his left eye, initially suspected to be juvenile xanthogranuloma and later diagnosed as tapioca nevi. Four years later the tumors enlarged. There was invasion throughout the anterior segment into the angle and related secondary elevation in intraocular pressure. Read More

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August 2017
1 Read

Oral Juvenile Xanthogranuloma: Report of Two Cases.

Pediatr Dent 2017 May;39(3):238-240

Associate professor of Oral Pathology, in the School of Dentistry, Rio de Janeiro State University, Rio de Janeiro, Brazil;, Email:

Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that usually occurs in the skin of children. Extracutaneous involvement is rare, and few cases affecting the oral cavity have been reported. The purpose of the present study was to report two cases of oral juvenile xanthogranuloma affecting the lower lip of a 14-year-old girl and the soft palate of a second 14-month-old girl, both present as yellowish papules. Read More

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May 2017
3 Reads

Erdheim-Chester Disease: Comprehensive Review of Molecular Profiling and Therapeutic Advances.

Anticancer Res 2017 06;37(6):2777-2783

Division of Hematology and Oncology, Department of Medicine, George Washington University Medical Center, Washington, DC, U.S.A.

The revised 2016 World Health Organization classification introduced Erdheim-Chester disease (ECD) as a provisional entity within the histiocytic and dendritic cell neoplasms separate from the juvenile xanthogranuloma family based on distinct molecular features. However, evolving knowledge regarding the molecular and genetic aberrations in addition to common clinical features of ECD support the classification of ECD together with Langerhans cell histiocytosis (LCH). Accordingly, ECD can be thought of as an inflammatory myeloid clonal disorder based on the detection of various activating mutations along the mitogen activated protein kinase-extracellular signal regulated kinase (MAPK-ERK) pathway with most notable variant being a valine to a glutamic acid substitution at amino acid 600 in the B-rapidly accelerated fibrosarcoma protein (BRAFV600E). Read More

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June 2017
7 Reads

Multiple adult-onset xanthogranuloma, an uncommon diagnosis.

An Bras Dermatol 2017 Mar-Apr;92(2):294-295

Department of Dermatology and Venereology of the Hospital and University Center of Coimbra (CHUC, EPE) - Coimbra, Portugal.

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November 2017
1 Read

Neonatal glaucoma associated with juvenile xanthogranuloma: Case report.

Arch Soc Esp Oftalmol 2017 Aug 15;92(8):394-397. Epub 2017 May 15.

Servicio de Oftalmología, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, España.

Case Report: The case concerns a 22 day-old male child with juvenile xanthogranuloma (JXG), which manifested as a unilateral glaucoma and with fibrinous haemorrhagic exudate in the anterior chamber affecting the angle of the right eye. Despite a high level of suspicion, the definitive diagnosis was not possible until the infant reached the age of 10 months, after the appearance of the skin lesions typical of this condition and histopathological study of them.

Conclusion: JXG is a rare disease, characterised by yellowish skin lesions on the trunk, neck, or head. Read More

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August 2017
2 Reads

Activating MAPK1 (ERK2) mutation in an aggressive case of disseminated juvenile xanthogranuloma.

Oncotarget 2017 Jul;8(28):46065-46070

Texas Children's Cancer Center, Texas Children's Hospital, Houston, TX 77030, USA.

Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder that is usually benign and self-limiting. We present a case of atypical, aggressive JXG harboring a novel mitogen-activated protein kinase (MAPK) pathway mutation in the MAPK1 gene, which encodes mitogen-activated protein kinase 1 or extracellular signal-regulated 2 (ERK2). Our analysis revealed that the mutation results in constitutive ERK activation that is resistant to BRAF or MEK inhibitors but susceptible to an ERK inhibitor. Read More

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July 2017
16 Reads

BRAF V600E mutation in pediatric intracranial and cranial juvenile xanthogranuloma.

Hum Pathol 2017 11 10;69:118-122. Epub 2017 May 10.

Department of Pathology, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand; Chulolongkorn GenePRO Center, Research Affairs, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand. Electronic address:

Juvenile xanthogranuloma (JXG) is a cutaneous form of non-Langerhans cell histiocytosis, primarily affecting children. The lesion is presumed to originate from either macrophages or dermal dendritic cells. JXG can rarely present as an isolated intracranial lesion and, in contrast to the dismal outcome of patients with systemic disease, cranial JXG has been shown to carry a more favorable prognosis. Read More

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November 2017
1 Read

The clinical spectrum of xanthomatous lesions of the eyelids.

Int J Dermatol 2017 Oct 13;56(10):981-992. Epub 2017 May 13.

Pathology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.

Yellowish papules, nodules, or plaques, namely "xanthomatous" lesions, may be seen on the eyelids in the course of various disorders. The prototype is "xanthelasma palpebrarum" (XP) that is localized only to the eyelids and may be associated with hyperlipidemia. On the other hand, different types of normolipemic disorders may also cause xanthomatous eyelid lesions. Read More

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October 2017
4 Reads

A Japanese infant with systemic juvenile xanthogranuloma mimicking severe microangiopathy.

Ann Hematol 2017 Jul 12;96(7):1233-1235. Epub 2017 May 12.

Pediatrics, Nihon University School of Medicine, 30-1 Ohyaguchi-kamicho, Itabashi-ku, Tokyo, 173-8610, Japan.

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July 2017
2 Reads

Hemosiderotic Juvenile Xanthogranuloma.

Am J Dermatopathol 2017 Oct;39(10):773-775

*Department of Cellular Pathology, Hospital El Bierzo, Cellular Pathology, Ponferrada, Spain; †Biomedical Investigation Institute of A Coruña, CellCOM-ST Group, A Coruña, Spain; ‡Department of Cellular Pathology, Hospital de la Reina, Cellular Pathology, Ponferrada, Spain; §Department of Histopathology, Children's Birmingham Hospital, Birmingham, United Kingdom; ¶Department of Histopathology, Birmingham Children's Hospital, Birmingham, United Kingdom; ‖Department of Histopathology, University Children's Hospital, Dublin, Ireland; and **Department of Histopathology, Hospital Niño Jesus, Madrid, Spain.

Juvenile xanthogranuloma is a non-Langerhans cell lesion mostly limited to the skin but occasionally presenting in extracutaneous locations or associated with systemic conditions. Lesions need to be distinguished mainly from dermatofibroma, xanthoma, Langerhans cell histiocytosis, or reticulohistiocytoma. Herein, we present a hemosiderotic variant of juvenile xanthogranuloma in a 12-year-old girl, which we have not found described in literature. Read More

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October 2017
3 Reads

Ultrasound Appearance of Juvenile Xanthogranuloma.

Actas Dermosifiliogr 2017 09 31;108(7):683-685. Epub 2017 Mar 31.

Servicio de Dermatología, Hospital Universitario de Fuenlabrada, Madrid, España.

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September 2017
2 Reads

[Generalized eruptive histiocytosis-Juvenile xanthogranuloma: clinical spectrum in a pediatric patient].

Arch Argent Pediatr 2017 04;115(2):e116-e119

Servicio de Dermatología. Hospital del Niño Jesús. San Miguel de Tucumán.

Both, generalized eruptive histiocytosis and juvenile xanthogranuloma are dendritic histiocytic disorders (also known as non-Langerhans cells histiocytosis) that share clinicopathological and immunohistiochemical characteristics. We present a 3-year-old female patient with skin lesions that were clinically compatible with generalized eruptive histiocytosis, confirmed by histopathological and immunohistochemical studies. During her development the disorder compromised the central nervous system, and surgical intervention of one symptomatic lesion was needed. Read More

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April 2017
1 Read

Cutaneous and Systemic Findings in Mosaic Neurofibromatosis Type 1.

Pediatr Dermatol 2017 May 20;34(3):271-276. Epub 2017 Mar 20.

Department of Dermatology, Hospital Infantil del Niño Jesús, Madrid, Spain.

Background/objectives: Mosaic neurofibromatosis type 1 (MNF1) is a variant of neurofibromatosis type 1 (NF1) in which clinical manifestations are limited to one or several body segments. The objective was to characterize the cutaneous features and associated systemic findings in a cohort of children with MNF1.

Methods: We performed a retrospective study of 40 children diagnosed with MNF1 at the Department of Dermatology, Hospital Infantil Niño Jesús, Madrid, Spain, from January 1, 1986, to October 31, 2015. Read More

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May 2017
3 Reads

A Rare Case of Solitary Giant Congenital Juvenile Xanthogranuloma: A Case Report.

J Cutan Med Surg 2017 May/Jun;21(3):267-269. Epub 2017 Feb 1.

3 Hôpital de Maria, Maria, QC, Canada.

Juvenile xanthogranuloma (JXG) is the most frequent form of non-Langerhans cell histiocytosis. We present a case of giant congenital JXG in a 7-week-old boy, who had a firm and incompressible lesion, measuring 3 × 4 cm in diameter, on his right flank. The clinical appearance of the lesion and the ultrasound results suggested a vascular tumor, such as a hemangioma. Read More

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May 2018
1 Read

A Case with Juvenile Xanthogranuloma and Progressive Nodular Histiocytosis Overlap.

Pediatr Dermatol 2017 Mar;34(2):e102-e103

Department of Dermatology, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.

A patient with overlap between juvenile xanthogranuloma and progressive nodular histiocytosis is presented. He had progressive, superficial, small, yellow-reddish papules and large dermal nodules, consistent with progressive nodular histiocytosis. Histopathology showed distinctive features of juvenile xanthogranuloma. Read More

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March 2017
2 Reads

Severe systemic juvenile xanthogranuloma is an indication for systemic therapy.

Authors:
R Fölster-Holst

Br J Dermatol 2017 02;176(2):302-304

Departments of Dermatology, Venereology and Allergology, University of Schleswig-Holstein (UKSH), Campus Kiel, Kiel, Germany.

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February 2017
1 Read

The relationship between neurofibromatosis type 1, juvenile xanthogranuloma, and malignancy: A retrospective case-control study.

J Am Acad Dermatol 2017 Jun 8;76(6):1084-1087. Epub 2017 Feb 8.

Section of Pediatric Dermatology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address:

Background: Neurofibromatosis type 1 (NF-1) predisposes individuals to the development of benign and malignant tumors. The association of NF-1, juvenile xanthogranuloma (JXG), and juvenile myelomonocytic leukemia has been described in the literature. It is unclear whether JXG alone constitute a risk factor for leukemia or other malignancies in children with NF-1. Read More

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June 2017
17 Reads

[Juvenile xanthogranuloma in a 4-year-old child].

Arch Pediatr 2017 Mar 1;24(3):298-300. Epub 2017 Feb 1.

Université François-Rabelais de Tours, 37000 Tours, France; Service de dermatologie, hôpital Trousseau, CHRU de Tours, avenue de la République, 37044 Tours cedex 9, France.

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March 2017
2 Reads