1,249 results match your criteria Juvenile Xanthogranuloma Nevoxanthoendothelioma

Xanthogranuloma of the external auditory canal-an atypical anatomical manifestation.

J Surg Case Rep 2021 Mar 31;2021(3):rjaa536. Epub 2021 Mar 31.

Department of Otolaryngology Head and Neck Surgery, Modbury Hospital, Adelaide, South Australia, Australia.

Juvenile xanthogranuloma is a proliferative cutaneous manifestation encountered in the paediatric population. Adult cases are uncommon, but have been reported. Lesions are prevalent in the head and neck region, but rarely observed in the external auditory canal. Read More

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Neonatal systemic juvenile Xanthogranuloma with Hydrops diagnosed by Purpura skin biopsy: a case report and literature review.

BMC Pediatr 2021 Apr 6;21(1):161. Epub 2021 Apr 6.

Department of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.

Background: Systemic juvenile xanthogranuloma is a very rare disease typically presents as skin lesions with yellow papules or nodules and is sometimes fatal. We report a case of congenital neonatal systemic juvenile xanthogranuloma with atypical skin appearance that made the diagnosis difficult.

Case Presentation: A preterm Japanese female neonate with prenatally diagnosed fetal hydrops in-utero was born with purpuric lesions involving the trunk and face. Read More

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Musculoskeletal imaging features of non-Langerhans cell histiocytoses.

Skeletal Radiol 2021 Mar 31. Epub 2021 Mar 31.

Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex, HA7 4LP, UK.

The non-Langerhans cell histiocytoses (N-LCH) represent a group of rare diseases with different clinical presentations and imaging features to classical LCH. While there is a long list of entities, only few present with musculoskeletal soft tissue and osseous manifestations alongside the more commonly reported systemic findings. Erdheim-Chester disease (ECD) is typically seen in adults as bilateral and symmetrical long bone osteosclerosis. Read More

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Rare presentation of juvenile xanthogranuloma in the posterior fossa of a toddler.

BMJ Case Rep 2021 Mar 26;14(3). Epub 2021 Mar 26.

Neurosciences and Pediatrics, University of California San Diego, La Jolla, California, USA

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Fatal Disseminated Multiple Intracranial Juvenile Xanthogranuloma without Cutaneous and Other Organ Involvement: A Rare Case Report.

Pediatr Neurosurg 2021 18;56(2):166-170. Epub 2021 Mar 18.

Department of Neurosurgery, Adana City Training and Research Hospital, Adana, Turkey.

Introduction: Juvenile xanthogranuloma (JXG) is a disorder of histiocytic proliferation that affects young children and usually presents as spontaneously regressing cutaneous lesions. JXG with systemic involvement is a rare entity associated with significant morbidity and mortality. Intracranial solitary lesions are uncommon, and when comorbid with multiple lesions of the central nervous system in young children, it has an extremely worse prognosis. Read More

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Rare tumors in pediatric age group: Single center experience from Saudi Arabia.

Rare Tumors 2021 24;13:2036361321997331. Epub 2021 Feb 24.

Division of Pediatric Hematology/Oncology, Pediatric Department, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.

Rare pediatric tumors are heterogeneous group containing a variety of histopathological diseases, they represent approximately 10% of all childhood cancers. These rare tumors had a diversity of histology and clinical behaviors that pose different challenges to the investigators. Exploring different pediatric rare tumors. Read More

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February 2021

Intracranial juvenile xanthogranuloma in an infant.

Childs Nerv Syst 2021 Mar 4. Epub 2021 Mar 4.

Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul, Korea.

Juvenile xanthogranuloma (JXG) is a type of non-Langerhans cell histiocytosis that most commonly manifests as a solitary cutaneous lesion of the head and neck in children. Intracranial JXG is extremely rare. Although it is widely known that JXG skin lesions gradually disappear over time without treatment, treatment guidelines for intracranial JXG have not been established. Read More

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[A toddler with yellow nodules].

Ned Tijdschr Geneeskd 2021 01 14;165. Epub 2021 Jan 14.

Groene Hart Ziekenhuis, afd. Dermatologie, Gouda.

A 1.5 year-old boy is diagnosed with juvenile xanthogranuloma. Juvenile xanthogranuloma is a benign proliferation of histiocytes, which is generally self-limiting. Read More

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January 2021

Histiocytosis and Neoplasms of Macrophage-Dendritic Cell Lineages: Multimodality Imaging with Emphasis on PET/CT.

Radiographics 2021 Mar-Apr;41(2):576-594. Epub 2021 Feb 19.

From the Department of Radiology, Mayo Clinic, 13400 E Shea Blvd, Scottsdale, AZ 85259.

Histiocytosis is a rare inflammatory process characterized by pathologic infiltration and accumulation of cells derived from the monocytic lineage in normal tissue. It encompasses more than 100 different subtypes of disorders that were recently classified into five main groups: Langerhans-related histiocytosis, Rosai-Dorfman histiocytosis, cutaneous and mucocutaneous histiocytosis, malignant histiocytosis, and hemophagocytic lymphohistiocytosis and macrophage activation syndrome. Langerhans cell histiocytosis is the most common histiocytic disorder. Read More

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February 2021

Yellow papules and nodules in an infant.

JAAD Case Rep 2021 Mar 25;9:15-17. Epub 2020 Dec 25.

Dermatology Ward, Military Medical Academy, Sofia, Bulgaria.

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Histiocytic Diseases of Neonates: Langerhans Cell Histiocytosis, Rosai-Dorfman Disease, and Juvenile Xanthogranuloma.

Clin Perinatol 2021 Mar 12;48(1):167-179. Epub 2021 Jan 12.

Baylor College of Medicine, Texas Children's Cancer and Hematology Centers, 6701 Fannin Street, Suite 1510, Houston, TX 77030, USA. Electronic address:

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. Read More

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Bilateral Spontaneous Hyphema in Juvenile Xanthogranuloma: A Case Report.

Case Rep Ophthalmol 2020 Sep-Dec;11(3):668-675. Epub 2020 Dec 21.

King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia.

Juvenile xanthogranuloma (JXG) is an idiopathic granulomatous inflammatory condition that usually affects children. Intraocular involvement, especially bilateral, is rare in JXG. Most patients with ocular lesions are typically infants and usually present with hyphema, iridocyclitis, and secondary glaucoma. Read More

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December 2020

Cutaneous Findings in Neurofibromatosis Type 1.

Cancers (Basel) 2021 Jan 26;13(3). Epub 2021 Jan 26.

Dermatology Unit, University of Campania Luigi Vanvitelli, 80100 Naples, Italy.

Neurofibromatosis type 1 (NF1) is a complex autosomal dominant disorder associated with germline mutations in the NF1 tumor suppressor gene. NF1 belongs to a class of congenital anomaly syndromes called RASopathies, a group of rare genetic conditions caused by mutations in the Ras/mitogen-activated protein kinase pathway. Generally, NF1 patients present with dermatologic manifestations. Read More

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January 2021

Dermoscopic Patterns in Juvenile Xanthogranuloma Based on the Histological Classification.

Jiaosheng Xu Lin Ma

Front Med (Lausanne) 2020 13;7:618946. Epub 2021 Jan 13.

Department of Dermatology, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing, China.

Several dermoscopic features of juvenile xanthogranuloma (JXG) have been previously described in single cases or small case series and need to be further verified in a large sample. We aimed to investigate the dermoscopic patterns of JXG in a large case series and the correlations of these with clinical features of different histopathological subtypes of JXG. Patients who underwent dermoscopic evaluation and had a histopathological diagnosis of JXG were recruited. Read More

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January 2021

Multiple Xanthogranulomas in an Adult Patient with Myelodysplastic Syndrome.

Case Rep Dermatol Med 2020 7;2020:8826715. Epub 2020 Dec 7.

Pathological Anatomy Service, Severo Ochoa University Hospital, Leganés, Madrid, Spain.

Adult multiple xanthogranuloma (XG) is a rare late-onset variant of juvenile XG. It is characterized by the appearance of papules or nodules located preferably on the trunk. A case of a 54-year-old man with myelodysplastic syndrome is presented as a history of interest, who consulted due to the appearance of multiple brownish papules distributed mainly in the trunk. Read More

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December 2020

Solitary Intracranial Juvenile Xanthogranuloma Masquerading as Low-Grade Glioma - A Rare Case Report.

Neurol India 2020 Nov-Dec;68(6):1439-1442

Department of Neurosciences, Apollo Speciality Hospitals, Vanagaram, Chennai, Tamil Nadu, India.

Juvenile xanthogranuloma (JXG) is a rare, benign histiocytic disorder of young children. It is most often present with cutaneous involvement of the head, neck and trunk region. Systemic JXG causes cutaneous lesions with extracutaneous involvements frequently seen in the orbit, liver, spleen, lung, kidney and bones. Read More

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December 2020

Congenital Giant Juvenile Xanthogranuloma in a 3-Month-Old Boy.

J Pediatr 2021 04 14;231:287-288. Epub 2020 Dec 14.

Department of Radiology, AORN Santobono Pausilipon, Pediatric Hospital, Naples, Italy.

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Juvenile xanthogranuloma: retrospective analysis of 44 pediatric cases (single tertiary care center experience).

Int J Dermatol 2021 May 10;60(5):564-569. Epub 2020 Dec 10.

Department of Pathology, Dr. Behcet Uz Children's Research and Training Hospital, Izmir, Turkey.

Background/objective: Juvenile xanthogranuloma (JXG) is a rarely encountered skin disorder, which is characterized by the proliferation of non-Langerhans cell histiocytes. As JXG primarily affects infants and young children, this study aims to describe the epidemiologic, clinical, and histopathologic characteristics of 44 children diagnosed with JXG at a tertiary health care center.

Methods: Fourty-four children with a histopathologic diagnosis of JXG between January 2003 and January 2017 were retrospectively reviewed. Read More

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Isolated endobronchial juvenile xanthogranuloma: a case report.

Contemp Oncol (Pozn) 2020 26;24(3):200-202. Epub 2020 Jul 26.

Department of Paediatric Surgery, Traumatology and Urology, Poznan University of Medical Sciences, Poznań, Poland.

Juvenile xanthogranuloma (JXG) is a rare non-Langerhans cell tumour usually diagnosed during infancy. The lesion is typically located in the skin; however, extracutaneous lesions have been described. The symptoms vary depending on the location and size of the lesion. Read More

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Dig deeper when it does not make sense: Juvenile xanthomas due to sitosterolemia.

JIMD Rep 2020 Nov 20;56(1):34-39. Epub 2020 Aug 20.

Victorian Clinical Genetics Services Murdoch Children's Research Institute Parkville Victoria Australia.

Sitosterolemia is an extremely rare autosomal recessive disease caused by mutations in either or , which encode for a sterol efflux transporter (sterolin) that pumps sterols out into the intestinal lumen or into bile. This leads to progressive accumulation of plant sterols in blood and tissues. Clinical presentation is variable and may include xanthoma, arthritis, thyroid dysfunction, premature atherosclerotic disease, splenomegaly, and hematologic manifestations. Read More

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November 2020

Elastophagocytosis in Juvenile Xanthogranuloma: A Case Report.

Am J Dermatopathol 2020 Nov 16. Epub 2020 Nov 16.

Department of Dermatology, Lebanese American University Medical Center-Rizk Hospital, Beirut, Lebanon.

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November 2020

Dermoscopy of Juvenile Xanthogranuloma.

Dermatology 2020 Oct 19:1-6. Epub 2020 Oct 19.

First Department of Dermatology, Aristotle University, Thessaloniki, Greece.

Background: Dermoscopy is useful for the evaluation of juvenile xanthogranuloma (JXG). The classical "setting sun" pattern is characteristic of JXG, but its sensibility appears to be limited. An extensive description of other dermoscopic findings is not available in the literature. Read More

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October 2020

Diagnosing juvenile xanthogranuloma with reflectance confocal microscopy.

JAAD Case Rep 2020 Oct 25;6(10):975-976. Epub 2020 Jul 25.

Center for Dermatology, Rutgers Robert Wood Johnson Medical School, Somerset, New Jersey.

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October 2020

Imaging features of neonatal systemic juvenile xanthogranuloma: a case report and review of the literature.

J Int Med Res 2020 Sep;48(9):300060520956416

Department of Obstetrics & Gynecology, Washington University, St. Louis, MO, USA.

Juvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytic disorder in children. This report describes the case of a 28-day-old boy that presented with multiple subcutaneous nodular lesions on the trunk and extremities, and multiple red nodular lesions on the scrotum. Magnetic resonance imaging (MRI) of the brain showed a well-demarcated extra-axial dura-based mass that appeared isointense or slightly hyperintense on T1-weighted images, hypointense on T2-weighted images and had intense enhancement on gadolinium-enhanced T1-weighted images. Read More

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September 2020

Isolated Forefoot Juvenile Xanthogranuloma: Unique Case Study and Treatment in a Pediatric Patient.

J Foot Ankle Surg 2020 Nov - Dec;59(6):1301-1305. Epub 2020 Aug 15.

Residency Program Director, Highlands-Presbyterian/St. Luke's Podiatric Medicine and Surgery Residency Program, Denver, CO. Electronic address:

Juvenile xanthogranuloma is a benign histiocytic cell proliferative disorder that occurs in early childhood. The most common presentation occurs within the first 2 years of life with papular or nodular changes to the skin on the head, neck or upper trunk. This case study documents the findings and treatment of a single solitary soft tissue mass in the forefoot of a 17-year-old patient. Read More

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Cutaneous manifestations in neurofibromatosis type 1.

Clin Ter 2020 Sep-Oct;171(5):e371-e377

Dermatologic Clinic, "Sapienza" University of Rome, Rome.

Objective: To better understand the real prevalence of cutaneous manifestations, in Neurofibromatosis type 1.

Materials And Methods: We reviewed all clinical charts of 1102 NF1 patients followed by February 1983 to February 2020 at the "Sapienza" University of Rome, Italy. NF1 patients are seen usually every year by a dermatologist. Read More

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November 2020

Multiple Xanthogranulomas in a Teenager.

Cureus 2020 Aug 2;12(8):e9516. Epub 2020 Aug 2.

Pathology, AmeriPath, Indianapolis, USA.

Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis that typically presents as a solitary lesion in infancy. Multiple lesions, especially in patients over one year of age, are rarely described in the literature. The authors report a case of a 17-year-old female who presented with multiple asymptomatic nodules and plaques. Read More

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Descriptive Analysis of Histiocytic and Dendritic Cell Neoplasms: A Single-Institution Experience.

Yonsei Med J 2020 Sep;61(9):774-779

Department of Pathology, Yonsei University College of Medicine, Severance Hospital, Seoul, Korea.

Purpose: Histiocytic and dendritic cell neoplasms are rare hematologic tumors. This study aimed to describe the epidemiologic features of the entire spectrum of histiocytic and dendritic cell neoplasms, including clinicopathological variables and patient outcomes.

Materials And Methods: We comprehensively reviewed 274 patients who were diagnosed with histiocytic and dendritic neoplasms at Severance Hospital, Seoul, South Korea between 1995 and 2018. Read More

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September 2020

Commentary: Iris nodules in infants and children- A challenging dilemma.

Indian J Ophthalmol 2020 09;68(9):2036

Department of Uveitis and Ocular Immunology, Narayana Nethralaya, Bangalore, Karnataka, India.

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September 2020

Polymerase chain reaction-proven tuberculous anterior segment mass mimicking juvenile xanthogranuloma in a child.

Indian J Ophthalmol 2020 09;68(9):2033-2035

Vision Research Foundation, Sankara Nethralaya, Chennai, Tamil Nadu, India.

Mass lesions arising from the anterior segment in children involving the iris and ciliary body can be of myogenic, neurogenic, or hematogenic/vascular origin. These include nevi, melanomas, adenoma, adenocarcinoma, cysts, metastatic tumours among others. Multiple iris mass lesions due to tuberculosis in children are rare. Read More

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September 2020