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Neurology India 2023
Topics: Humans; Xanthogranuloma, Juvenile; Cognitive Dysfunction
PubMed: 36861607
DOI: 10.4103/0028-3886.370469 -
Seminars in Cutaneous Medicine and... Sep 1999Juvenile xanthogranuloma (JXG) is a well-recognized benign disorder of infancy and early childhood characterized by yellowish cutaneous nodules that spontaneously... (Review)
Review
Juvenile xanthogranuloma (JXG) is a well-recognized benign disorder of infancy and early childhood characterized by yellowish cutaneous nodules that spontaneously regress over months to years. In the vast majority of children, JXG is limited to the skin and requires no treatment. Over the past two decades, unusual cutaneous and systemic forms of JXG have been increasingly reported. JXGs have been discovered, usually unexpectedly, in every organ system of the body. Correct diagnosis is crucial to prevent unnecessary invasive diagnostic and therapeutic procedures. Unusual clinical and histological variants of JXG often require immunohistochemical studies and/or electron microscopy to establish the diagnosis. Nonlipidized, giant, intramuscular, subcutaneous, and clustered JXG are but some of the variants that are discussed in this article. The immunohistochemistry of JXG, current nosology, and hypotheses regarding the origins of JXG are also reviewed.
Topics: Diagnosis, Differential; Humans; Immunohistochemistry; Skin Diseases; Xanthogranuloma, Juvenile
PubMed: 10468039
DOI: 10.1016/s1085-5629(99)80017-0 -
Dermatologic Therapy Feb 2022Juvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytic disorder. It can rarely be associated with systemic involvement. There is a paucity of...
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. We aim to describe the epidemiology, clinical features, systemic associations, histological features and outcome of a cohort of Asian children with JXG, and review the literature on the condition. We retrospectively reviewed the demographic, clinical and histological data of patients less than 16 years of age, diagnosed with JXG at our tertiary pediatric hospital between January 2002 and April 2019. A total of 147 children with JXG were identified, with a slight male preponderance of 53.1%. The median age of the onset was 15.5 months, with 69.4% presenting before 2 years of age. There was no racial predilection. The most frequently involved site was the head and neck region (44.2%). The majority of patients (76.2%) presented with a solitary lesion. Spontaneous resolution was documented in 57.7% of our patients with mean duration to resolution of 18.8 months. The proportion and speed of resolution did not differ in children with single or multiple lesions. No ophthalmologic complications were detected in our study cohort. JXG in children is generally limited to the skin and is rarely associated with systemic involvement, including the eye. Unless clinically indicated, the results from our study does not support routine screening for juvenile myelomonocytic leukemia, eye or systemic complications, even in the setting of multiple cutaneous JXGs.
Topics: Asian People; Child; Histiocytes; Humans; Infant; Male; Retrospective Studies; Skin; Xanthogranuloma, Juvenile
PubMed: 34820973
DOI: 10.1111/dth.15224 -
Spine Jun 2001This is a report of an exceptional case of isolated cervical juvenile xanthogranuloma in a child. (Review)
Review
STUDY DESIGN
This is a report of an exceptional case of isolated cervical juvenile xanthogranuloma in a child.
OBJECTIVES
This case report draws attention to the fact that isolated xanthogranuloma of the central nervous system should be considered among possible diagnosis of subdural extramedullary spinal masses in children and young adults.
SUMMARY AND BACKGROUND DATA
Isolated juvenile xanthogranuloma of the central nervous system is extremely rare. When located in the spinal canal it behaves like any extramedullary mass-occupying lesion. MRI depicts the tumor's association with adjacent structures. In cases in which a subtotal surgical removal was possible, radiotherapy has been indicated.
METHODS
A three-year-old girl presented severe pain in the right shoulder and spastic tetraparesis. The MRI showed an intradural extramedullary mass homogeneously enhancing after DTPA-gadolinium infusion. Complete surgical removal of the tumor was performed through open-door laminoplasty.
RESULTS
The child was pain free immediately after the surgical removal of the tumor. A gradual complete recovery of the neurologic deficits followed. Open-door laminoplasty provided sufficient operative space, and it minimized the impact on the growing spinal column.
CONCLUSIONS
Isolated juvenile xanthogranuloma does not show any predilections of localization inside the central nervous system. Both intracranial and spinal juvenile xanthogranulomas appear isointense in MRI and enhance homogeneously with gadolinium. Whenever possible, total surgical removal alone seems to be curative. Otherwise, a subtotal removal of the tumor might be followed by radiotherapy. Immunohistochemical tests ensure the diagnosis.
Topics: Cervical Vertebrae; Child, Preschool; Decompression, Surgical; Female; Humans; Magnetic Resonance Imaging; Spinal Diseases; Treatment Outcome; Xanthogranuloma, Juvenile
PubMed: 11426158
DOI: 10.1097/00007632-200106150-00026 -
Child's Nervous System : ChNS :... May 2015Juvenile xanthogranuloma (JXG) is a histiocytic condition in the spectrum of non-Langerhans histiocytosis that preferentially affects children. Rarely this condition can... (Review)
Review
INTRODUCTION
Juvenile xanthogranuloma (JXG) is a histiocytic condition in the spectrum of non-Langerhans histiocytosis that preferentially affects children. Rarely this condition can involve the central nervous system (CNS) with devastating consequences.
METHODS
The authors report the unique case of an 11-year-old child who initially presented with a sellar lesion without evidence of the cutaneous stigmata typical of JXG. She was later discovered to have JXG following initial diagnosis of granulomatous hypophysitis, with development of widespread intracranial disease and subsequent neurological deterioration. She underwent subtotal resection of her sellar lesion followed by whole brain radiation and systemic chemotherapy; however, she succumbed to her disseminated disease within 1 month of the JXG diagnosis.
CONCLUSIONS
This is a rare case of fatal disseminated intracranial JXG without cutaneous manifestations. Additionally, the initial presentation as a sellar lesion is particularly unusual and seldom described in the literature.
Topics: Adolescent; Child; Fatal Outcome; Female; Humans; Pituitary Neoplasms; Xanthogranuloma, Juvenile
PubMed: 25503249
DOI: 10.1007/s00381-014-2604-3 -
Indian Journal of Pediatrics Aug 2021
Topics: Hematologic Neoplasms; Humans; Liver Failure, Acute; Xanthogranuloma, Juvenile
PubMed: 32671637
DOI: 10.1007/s12098-020-03389-7 -
BMC Oral Health Dec 2022Juvenile Xanthogranuloma (JXG) is a non-hereditary, self-limiting disease which is usually presented in infancy or early childhood and in males over females.
BACKGROUND
Juvenile Xanthogranuloma (JXG) is a non-hereditary, self-limiting disease which is usually presented in infancy or early childhood and in males over females.
CASE PRESENTATION
We report a rare case of oral Juvenile Xanthogranuloma with recurrent progressive gingival hyperplasia and concomitant presentation of osteolysis in a 21-year-old adult male with no significant medical history. Patient presented with generalized gingival hyperplasia, osteolysis of the maxilla and mandible, and a round, firm, nodular mass with clear circumference on the left shoulder. Results of gingival tissue biopsy, karyotype, bone marrow biopsy and immunohistochemistry were suggestive of a diagnosis of Juvenile Xanthogranuloma with no association to hematologic malignancy. Unfortunately, patient declined treatment and elected to be transferred back to local hospital for future evaluation.
CONCLUSIONS
Juvenile Xanthogranuloma in adults can have atypical manifestations including generalized gingival hyperplasia and osteolysis of the maxilla and mandible. It should be differentiated between Langerhans cell histiocytosis, Papillon-Lefevre Syndrome, and Pyogenic Granulomas. Despite uncommon incidence, it should be included in differential diagnoses in cases of similar clinical presentations.
Topics: Female; Humans; Adult; Male; Child, Preschool; Young Adult; Xanthogranuloma, Juvenile; Osteolysis; Gingival Hyperplasia; Histiocytosis, Langerhans-Cell; Immunohistochemistry
PubMed: 36529720
DOI: 10.1186/s12903-022-02643-y -
International Journal of Dermatology Jun 1994
Review
Topics: Aged; Cryotherapy; Follow-Up Studies; Humans; Male; Recurrence; Xanthogranuloma, Juvenile
PubMed: 8056480
DOI: 10.1111/j.1365-4362.1994.tb04048.x -
Journal of Pediatric Ophthalmology and... 2021The authors present a case of bilateral painless progressive proptosis. A diagnosis of ocular juvenile xanthogranuloma was made based on clinical manifestations,...
The authors present a case of bilateral painless progressive proptosis. A diagnosis of ocular juvenile xanthogranuloma was made based on clinical manifestations, histopathology, and immunohistochemistrical staining. Genetic testing discovered the BRAF V600E mutation. This patient did not respond to standard chemotherapy; however, he demonstrated regression after anti-BRAF targeted therapy. .
Topics: Child; Eye; Humans; Male; Mutation; Proto-Oncogene Proteins B-raf; Xanthogranuloma, Juvenile
PubMed: 34288768
DOI: 10.3928/01913913-20210416-01 -
Congenital Heart Disease 2013Juvenile xanthogranuloma is a rare histiocytic disorder of childhood mainly affecting skin and rarely deep soft tissues and viscera. We report a 2-month-old infant who... (Review)
Review
Juvenile xanthogranuloma is a rare histiocytic disorder of childhood mainly affecting skin and rarely deep soft tissues and viscera. We report a 2-month-old infant who presented with respiratory distress secondary to a large pericardial effusion associated with an epicardial mass. Excisional biopsy was performed and the mass was diagnosed as juvenile xanthogranuloma. The child is well without evidence of disease 8 months following the excision. The corresponding literature on juvenile xanthogranuloma with cardiac manifestations is reviewed.
Topics: Biopsy; Cardiac Surgical Procedures; Echocardiography; Female; Heart Diseases; Humans; Infant; Magnetic Resonance Imaging; Pericardial Effusion; Respiratory Distress Syndrome, Newborn; Treatment Outcome; Xanthogranuloma, Juvenile
PubMed: 22691125
DOI: 10.1111/j.1747-0803.2012.00688.x