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Ken Matsubara, Harushi Mori, Nozomi Hirai, Kumi Yasukawa, Takafumi Honda, Jun-Ichi Takanashi
MRI in a 2-year-old female presenting afebrile seizures and left blepharoptosis revealed multiple well-marginated round-shaped lesions, isointensity to gray matter on T1- and T2-weighted images with homogenously reduced diffusion and diffuse contrast enhancement. MRS revealed elevation of taurine, choline and glutamate, and reduction of N-acetylaspartate. A brain biopsy confirmed a diagnosis of juvenile xanthogranuloma (JXG). JXG should be considered when MR spectroscopy shows elevated taurine and glutamate, which has only previously been reported in medulloblastomas...
November 2016: Brain & Development
Suvradeep Mitra, Saumya Gupta, Prema Menon, K L N Rao, Amanjit Bal
Juvenile xanthogranuloma (JXG) is a histiocytic disorder of childhood that mostly affects the skin although extracutaneous and visceral manifestations are well documented in English literature. The renal involvement is less commonly documented as a manifestation of xanthogranuloma and the reported cases occur in conjunction with cutaneous xanthogranuloma, especially in association with multiple skin lesions. We present a case of isolated renal JXG presenting as a renal mass with perinephric soft tissue and muscle involvement but without any cutaneous manifestation...
July 1, 2016: Fetal and Pediatric Pathology
L Maintz, J Wenzel, M Irnich, H Reinhard, T Bieber
The non-Langerhans cell histiocytosis juvenile xanthogranulomatosis (JXG) is usually a benign disease limited to the skin. Only few cases of systemic disease with ≥2 affected organs and lethal outcome have been reported yet. Treatment is controversial and no standard protocol is available. We report the rare case of a 22-month-old boy presenting multiple erythematous-brownish papules of head, trunk and legs which had developed starting from his sixth' month of life. Additional symptoms were delayed psychomotoric development, hydrocephalus and hepatosplenomegaly...
June 17, 2016: British Journal of Dermatology
Baocheng Wang, Huiming Jin, Yang Zhao, Jie Ma
BACKGROUND: Juvenile xanthogranulomas (JXGs) are uncommon non-Langerhans cell histiocytic proliferations which occur most often in children. Rare cases of intracranial JXGs in children have been reported. The precise treatment strategy for intracranial JXG with high fatality is still unclear. METHOD: We present four cases of intracranial JXG with 2-6 years of follow-up. Review of the previous literature since 1980 revealed another 39 pediatric intracranial JXGs...
July 2016: Acta Neurochirurgica
Hind M Alkatan, Ali A Al-Rajhi
Juvenile xanthogranuloma (JXG) is a benign inflammatory condition of uncertain pathogenesis. It is characterized by skin and ocular involvement - typically in the iris - in children. It has been reported in older age groups and has been also observed to involve other ocular structures such as the cornea and conjunctiva. In this case report, we are presenting an extensive right eye corneal lesion in a 43-year old male which showed the typical histopathological feature of JXG and in association with multiple endocrine neoplasia (type 1)...
January 2016: Saudi Journal of Ophthalmology: Official Journal of the Saudi Ophthalmological Society
G Kontos, S Borooah, A Khan, B W Fleck, S E Coupland
PURPOSE: Adult-onset xanthogranuloma (AOX) of the corneoscleral limbus is a rare inflammatory condition of unknown aetiology. Similar to limbal juvenile xanthogranuloma (JXG), it presents as a growing mass at the corneoscleral junction. Limbal AOX and JXG can lead to sight-threatening complications if not managed in a timely manner. This systematic review summarises the main clinical and histopathological features of limbal AOX/JXG and discusses the management of this uncommon disease...
March 2016: Graefe's Archive for Clinical and Experimental Ophthalmology
Julien Haroche, Oussama Abla
Rosai-Dorfman disease (RDD), juvenile xanthogranuloma (JXG), and Erdheim-Chester disease (ECD) are non-Langerhans cell (non-LCH) disorders arising from either a dendritic or a macrophage cell. RDD is a benign disorder that presents with massive lymphadenopathy, but can have extranodal involvement. In most cases, RDD is self-limited and observation is the standard approach. Treatment is restricted to patients with life-threatening, multiple-relapsing, or autoimmune-associated disease. JXG is a pediatric histiocytosis characterized by xanthomatous skin lesions that usually resolve spontaneously...
2015: Hematology—the Education Program of the American Society of Hematology
Carl E Allen, D Williams Parsons
Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG), and Erdheim-Chester disease (ECD) represent histiocytic disorders with a wide range of clinical manifestations. Until recently, mechanisms of pathogenesis have been speculative and debate has focused on classification of these conditions as reactive versus neoplastic. Genomic studies have been challenged by scarce tissue specimens, as well as heterogeneous nature of the lesions with variable infiltration of pathologic histiocytes. Whole-exome sequencing recently revealed a very low frequency of somatic mutations in LCH, JXG, and ECD compared to other neoplastic disorders...
2015: Hematology—the Education Program of the American Society of Hematology
Su Yuen Ng
A 3-month-old female patient with a giant ulcerated nodule over the back since birth was diagnosed as congenital giant juvenile xanthogranuloma (JXG) based on clinical and histopathological examination. Congenital giant JXG with ulceration at birth is a rare presentation of JXG and commonly misdiagnosed. This case emphasizes the importance of being aware of the myriad presentations of JXG in order to make a correct diagnosis and avoid unnecessary investigations or treatment.
July 2015: Indian Journal of Dermatology
Wasim A Samara, Chloe T L Khoo, Emil Anthony T Say, Jarin Saktanasate, Ralph C Eagle, Jerry A Shields, Carol L Shields
PURPOSE: To report clinical features and treatment outcomes of ocular juvenile xanthogranuloma (JXG). DESIGN: Retrospective case series. PARTICIPANTS: There were 32 tumors in 31 eyes of 30 patients with ocular JXG. METHODS: Review of medical records. MAIN OUTCOME MEASURES: Tumor control, intraocular pressure (IOP), and visual acuity. RESULTS: The mean patient age at presentation was 51 months (median, 15 months; range, 1-443 months)...
October 2015: Ophthalmology
Chinmay Kar, Kapildev Das, Jayanta K Barua
Juvenile xanthogranuloma (JXG) is the most common form of non-Langerhans cell histiocytic lesion, characterized by benign, usually asymptomatic, self-healing yellowish brown papulonodular lesions of skin and other organs in the absence of metabolic disorder. The cells of origin of JXG are dermal dendrocytic cells. Histopathologically there is dermal infiltration of foamy or non-foamy histiocytes with or without giant cell. Immunohistochemistry shows CD68 positivity with CD1a and S-100 negativity of lesional cells although S-100-positive JXG have been reported...
May 2015: Indian Journal of Dermatology
Liangliang Niu, Chaoran Zhang, Fanrong Meng, Rongrong Cai, Yingwen Bi, Yan Wang, Jianjiang Xu
PURPOSE: To report the clinical and histopathologic characteristics and prognoses of three ocular juvenile xanthogranuloma (JXG) cases. CASE REPORTS: Three cases were included in this study. The first case involved a 5-year-old girl with an enlarging yellowish mass at the limbus with corneal involvement. Ultrasound biomicroscopy showed a poorly demarcated mass involving the underlying cornea and sclera. The mass was excised in combination with a lamellar keratoplasty procedure...
June 2015: Optometry and Vision Science: Official Publication of the American Academy of Optometry
Kamlesh Singh Bhaisora, Awadhesh Kumar Jaiswal, Anant Mehrotra, Rabi Narayan Sahu, Arun Srivastava, Sushila Jaiswal, Sanjay Behari
The authors present a case of 15 years male child who presented with neck pain and progressive ascending quadriparesis. Magnetic resonance imaging showed lytic mass involving C5 and C6 vertebra with soft tissue extension. Surgical excision of mass done using anterior cervical approach. Postoperatively, patient showed improvement in spasticity and power. Histopathological examination of mass was suggestive of juvenile xanthogranuloma (JXG). At 6 months follow-up, patient was improving without any evidence of recurrence...
January 2015: Asian Journal of Neurosurgery
Yunlai Zhi, Yuhe Duan, Hong Zhang, Xiaofeng Yin, Tingting Qu, Ge Guan, Lin Su, Qian Dong
Juvenile xanthogranuloma (JXG) is a rare disease that is part of a spectrum of histiocytic dendritic cell disorders. Most patients present with a solitary cutaneous lesion; however, others present with extracutaneous manifestations or even with systemic involvement. We present the first report of an 11-month-old girl in whom was diagnosed a unifocal extracutaneous JXG involving the tibia. Histological and immunohistochemical staining results are presented. A review of the literature on these unusual lesions is conducted, along with discussion of their differential diagnosis and key aspects of the patient's evaluation, management, and pathological diagnosis...
2015: International Journal of Clinical and Experimental Pathology
Devendra Purohit, Amit Kumar Chanduka, Vinod Sharma, Radhey Shyam Mittal, Shashi Singhvi
Juvenile Xanthogranuloma (JXG) is a rare disorder of central nervous system. It rarely produces compressive myelopathy. On reviewing world literature, we could find only nine cases of this disease involving spine and of which only four cases were in adults' i.e., 18 years and above. We are presenting a case of Spinal JXG in an 18-year-old male with thoracic compressive myelopathy presenting as short duration progressive paraparesis. Magnetic Resonance Imaging of Spine showed mass lesion in epidural space compressing cord from behind without any bony involvement at D7 to D10 vertebral segment...
October 2014: Asian Journal of Neurosurgery
Yohei Miyake, Susumu Ito, Mio Tanaka, Yukichi Tanaka
The authors report the case of a large left occipital mass lesion in an 8-month-old boy who presented with seizure. Neuroimaging demonstrated an approximately 5-cm extraaxial tumor, and the patient underwent partial resection. The tumor was strongly attached to the tentorium and falx. In the postoperative course the residual lesion regressed spontaneously, and after 5 years only a slight residual tumor remained along the tentorium. Histopathological examination of the tumor revealed non-Langerhans cell histiocytosis (non-LCH)...
April 2015: Journal of Neurosurgery. Pediatrics
Jennifer Picarsic, R Maarten Egeler, Kudakwashe Chikwava, Kathleen Patterson, Ronald Jaffe
Thymic involvement by Langerhans cell histiocytosis (LCH) has been described mainly in isolated case reports. A description of the histopathologic patterns of LCH proliferations in the thymus, together with therapeutic implications, has not, to our knowledge, been previously addressed. The pathology consultation files at Children's Hospital of Pittsburgh of the University of Pennsylvania Medical Center were reviewed for cases of thymic involvement by LCH. Relevant cases in the literature were also reviewed, and the histopathology and clinical course of those cases were collected...
March 2015: Pediatric and Developmental Pathology
Małgorzata Neska-Matuszewska, Anna Zimny, Krzysztof Kałwak, Marek J Sąsiadek
BACKGROUND: Primary Central Nervous System Lymphomas (PCNSLs) are rare, malignant brain tumors derived from lymphocytes B. Juvenile xanthogranuloma (JXG) is a non-Langerhans histiocytic cell disorder in children which mostly affects the skin. Rare fatalities have been reported in extracutaneous manifestation. Brain magnetic resonance imaging (MRI) is a method of choice in the diagnostics of all neoplastic CNS lesions. Perfusion weighted imaging (PWI) and diffusion weighted imaging (DWI) allow for more detailed analysis of brain tumors including the rate of neoangiogenesis and cellularity...
2015: Polish Journal of Radiology
Nicola Marotta, Alessandro Landi, Cristina Mancarella, Pierluigi Rocco, Andrea Pietrantonio, Gaspare Galati, Antonio Bolognese, Roberto Delfini
Xanthogranulomas (XG) are benign proliferative disorder of histiocytes, a non-Langerhans cell histiocytosis. Whose etiology is unknown. The nature of these lesions is controversial and could be either reactive or neoplastic; the presence of monoclonal cells does, however, favor the second hypothesis. Xanthogranuloma is frequently found in young adults and children (under 20 years old), mainly in the skin. In about 5%-10% of all Juvenile XG (JXG) cases xanthogranuloma are extracutaneous. Within this group, the site most frequently involved is the eye...
January 16, 2015: World Journal of Clinical Cases
Sune R R Jans, Eckhard Schomerus, Anette Bygum
An association between juvenile xanthogranuloma (JXG), neurofibromatosis type 1 (NF1), and juvenile myelomonocytic leukemia (JMML) has been described in the literature but has only been documented in approximately 20 cases. We diagnosed a patient with NF1 at 25 months of age, before any cutaneous stigmata of this disease had appeared, because we decided to screen for the NF1 gene mutation because of his presentation with multiple JXGs and moderate macrocephaly (2.5 standard deviations) at 9 months of age and JMML diagnosed at 20 months of age...
January 2015: Pediatric Dermatology
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