1,409 results match your criteria: "Juvenile Xanthogranuloma Nevoxanthoendothelioma"
Juvenile Xanthogranuloma Involving the Left Femur: A Case Report and Literature Review.
Int Med Case Rep J
December 2024
Department of Orthopedic Oncology Surgery, Shandong Cancer Hospital and Institute Affiliated to Shandong First Medical University and Shandong Academy of Medical Science, Jinan, 250117, People's Republic of China.
Background: Juvenile xanthogranuloma is a rare condition, and femoral involvement is even rarer. We report a case of juvenile xanthogranuloma affecting the femur. To the best of our knowledge, this is the first reported case of femoral juvenile xanthogranuloma in China.
View Article and Find Full Text PDFDeep juvenile xanthogranuloma invading the left tensor fasciae latae muscle: a case report and a literature review.
J Clin Exp Hematop
November 2024
Division of Pediatrics and Perinatology, Department of Multidisciplinary Internal Medicine, School of Medicine, Tottori University Faculty of Medicine, Yonago, Japan.
Article Synopsis
- Juvenile xanthogranuloma (JXG) is a rare benign condition primarily affecting neonates and young children, often presenting as skin lesions, but intramuscular JXG is much less common, accounting for only 0.6% of cases.
- A case involving a 5-month-old girl showed a slow-growing lump in her left thigh, which was diagnosed as deep JXG after imaging, biopsy, and surgical resection.
- Histological findings revealed characteristics typical of JXG, and despite initial concerns about tumor margins, the patient has shown no signs of recurrence over 48 months post-surgery, highlighting the importance of proper diagnosis and monitoring.
Juvenile xanthogranuloma underneath a toenail: an unusual localization.
Br J Dermatol
November 2024
Department of Dermatology and Venerology, Peking University First Hospital, Beijing, China.
Skin Imaging of Juvenile Xanthogranuloma: In Vivo Diagnosis by Line-Field Confocal Optical Coherence Tomography.
Pediatr Dermatol
October 2024
Dermatologia, Dipartimento di Medicina e Chirurgia Traslazionale, Università Cattolica del Sacro Cuore, Rome, Italy.
A 15-year-old boy presented with a yellowish, asymptomatic nodule on the left forearm, which developed in the last year. A diagnosis of juvenile xanthogranuloma was confirmed with histopathology. Line-field confocal optical coherence tomography (LC-OCT), a new skin imaging technique, was performed prior to surgery, showing a strong correspondence with histopathological sections.
View Article and Find Full Text PDFGeneralized Eruptive Histiocytosis or Juvenile Xanthogranuloma: A Clinicopathological Conundrum of Coexistence or Xanthomatization.
Indian Dermatol Online J
July 2024
Department of Dermatology, Venereology and Leprosy, Sri Manakula Vinayagar Medical College and Hospital, Pondicherry, India.
Article Synopsis
- Diseases classified under non-LCH often have similar clinical and histological characteristics, making diagnosis difficult.
- A two-year-old girl exhibited various asymptomatic skin lesions, leading to a provisional diagnosis of generalized eruptive histiocytosis (GEH), which was later found to have overlapping features with juvenile xanthogranuloma (JXG).
- The case highlights the importance of careful differentiation between GEH and JXG since they may require different treatment approaches, demonstrating that clinical observation alone can be misleading due to their similarities.
Histiocytic neoplasms: a brief review and differential diagnosis.
J Clin Exp Hematop
September 2024
Department of Cell Pathology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
Article Synopsis
- - Histiocytic neoplasms (HNs) encompass various diseases like juvenile xanthogranuloma and Erdheim-Chester disease, which are recognized in the WHO's latest classification.
- - These diseases have unique clinical and histological characteristics, but they share a common feature of histiocytic cell proliferation, leading to similarities in appearance.
- - The review summarizes clinical data, molecular aspects, histological features, and immunophenotyping of HNs, while also addressing the need for differentiating them from other similar conditions.
Recurrent CLTC::SYK fusions and CSF1R mutations in juvenile xanthogranuloma of soft tissue.
Blood
December 2024
Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands.
Article Synopsis
- Juvenile xanthogranuloma (JXG) is a type of skin tumor mostly found in children, but some cases occur in other parts of the body, and their genetic causes are not fully understood.* -
- A study analyzed 16 children and 5 adults with extracutaneous JXG using advanced sequencing, finding that numerous genetic alterations, especially related to kinase fusions, were present in most children.* -
- The findings indicate that while surgery is common for treatment, some cases can regress on their own, and specific genetic alterations could help inform better treatment options in the future.*
A 3 -year-old boy presented with a forehead nodular mass, which was excised and confirmed histologically as Juvenile Xanthogranulomma (JXG). It affects children with a predilection for the head and neck region. A relatively rare, benign, histiocytic proliferative cutaneous disorder with a potential for malignancy.
View Article and Find Full Text PDFClinical features and outcomes of 17 children with systemic juvenile xanthogranuloma (sJXG) including five complicated with hemophagocytic lymphohistiocytosis (HLH).
Ann Hematol
August 2024
Hematology Centre, National Key Clinical Discipline of Pediatric Hematology, National Key Discipline of Pediatrics (Capital Medical University), Beijing, 100045, China.
Article Synopsis
- - Juvenile xanthogranuloma (JXG) typically affects the skin, but a rare form called systemic JXG (sJXG) can occur, sometimes linked with serious complications like hemophagocytic lymphohistiocytosis (HLH), particularly in younger children.
- - A study on 17 children with sJXG found that those with HLH exhibited specific symptoms such as fever and rash, and they were treated with corticosteroids and targeted therapy, showing promising outcomes with chemotherapy.
- - The overall survival rates for sJXG patients were similar regardless of whether they had HLH, with certain treatments like LCH-based chemotherapy and dabrafenib proving effective, especially
Serial 18 F-FDG PET/CT Revealing Mixed Histiocytosis in a Pediatric Patient.
Clin Nucl Med
October 2024
From the Department of Nuclear Medicine and PET, The Prince of Wales and Sydney Children's Hospitals, Randwick, Australia.
Article Synopsis
- A 6-month-old boy had soft tissue swelling and CT scans showed multiple bone lesions indicating a serious condition.
- PET/CT scans revealed hypermetabolic lesions in several areas, and a biopsy of one lesion confirmed it was Langerhans cell histiocytosis.
- After chemotherapy, further testing showed another lesion that was diagnosed as juvenile xanthogranuloma, highlighting a rare case of mixed histiocytosis.
Disseminated Juvenile Xanthogranuloma Harboring a GAB2::BRAF fusion Successfully Treated with Trametinib: a Case Report.
Br J Dermatol
August 2024
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Noonan syndrome-like disorder: Case report and review of the literature.
Pediatr Dermatol
November 2024
Department of Paediatrics, University of British Columbia, Vancouver, British Columbia, Canada.
Article Synopsis
- Only about 1% of patients with Noonan syndrome have a condition linked to pathological variants in CBL, known as Noonan syndrome-like disorder (NSLD).
- A case study of a 4-year-old boy with NSLD showed uncommon symptoms like multiple melanocytic nevi and superficial neurofibromas.
- Literature suggests common skin features of NSLD include café-au-lait macules, juvenile xanthogranuloma, and thin hair, indicating that recognizing these symptoms could reduce unnecessary biopsies in other children.
Refractory juvenile xanthogranuloma of the mastoid bone responsive to trametinib.
Pediatr Blood Cancer
October 2024
Division of Pediatric Hematology/Oncology/Bone Marrow Transplant, Department of Pediatrics, Medical College of Wisconsin, Children's Wisconsin, Milwaukee, Wisconsin, USA.
Line-field confocal optical coherence tomography and the diagnosis of cutaneous mastocytoma and juvenile xanthogranuloma.
Int J Dermatol
July 2024
Pediatric Dermatology Unit, Departments of Dermatology & Venereology, University Hospital Lausanne, University of Lausanne, Lausanne, Switzerland.
Advancements in the understanding and management of histiocytic neoplasms.
Blood Res
July 2024
Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, 88, Olympic-ro, 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.
Article Synopsis
- - Histiocytic neoplasms are rare diseases characterized by abnormal growths of macrophages, dendritic cells, and monocytes, with specific types such as Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD), each having different clinical outcomes.
- - Genetic studies indicate that these neoplasms often involve mutations in the MAPK pathway, particularly in genes like BRAF and MAP2K1, suggesting they have a clonal nature.
- - Advances in targeted therapies, including BRAF and MEK inhibitors, offer new treatment options, but challenges such as relapse and side effects highlight the need for further research to improve management and patient outcomes.
Orbital histiocytosis; From A to Z.
Int Ophthalmol
June 2024
Eye Research Center, Farabi Eye Hospital, Tehran University of Medical Sciences, Qazvin Square, Tehran, 1336616351, Iran.
Article Synopsis
- Histiocytosis is a complex disease with varying symptoms and unknown causes, particularly affecting the orbit, eyelids, and eyes, leading to issues like limited eye movement and vision problems.
- A review of 391 articles revealed that while the disease's underlying mechanisms are unclear, specific mutations are common, and diagnosis can differ based on factors like age and disease type.
- Treatments typically involve corticosteroids and surgery, but alternative therapies are being explored, highlighting the need for ongoing research to improve understanding and management of the disease.
Many faces of cutaneous leishmaniasis.
Postgrad Med J
September 2024
Department of Medical Microbiology, Harran University Medical Faculty, Şanlıurfa 63000, Turkey.
Article Synopsis
- This study aimed to identify atypical clinical presentations of cutaneous leishmaniasis (CL) in Şanlıurfa province, encompassing a retrospective analysis of 213 patients with non-standard symptoms out of 1,751 diagnosed cases between 2019 and 2022.
- The prevalence of atypical CL was found to be 12.1%, with common atypical lesions including lupoid, erysipeloid, and impetiginous types, along with many others resembling various skin conditions and diseases.
- The research highlights that CL can mimic a wide range of infectious, inflammatory, and neoplastic disorders, stressing the importance of thorough differential diagnosis for persistent non-healing skin lesions, especially in areas where CL is
Juvenile Xanthogranuloma of the Head and Neck: Imaging Findings in 11 Cases.
J Pediatr Hematol Oncol
August 2024
Radiologie pédiatrique, hôpital Armand Trousseau, Paris.
Article Synopsis
- Juvenile Xanthogranuloma (JXG) is a rare condition primarily affecting infants, often posing diagnostic challenges, especially when lesions are located outside the skin.
- This study involved analyzing the imaging characteristics of 11 patients with JXG in the head and neck using various imaging modalities such as ultrasound, MRI, and CT.
- Key findings suggest that JXG lesions are typically well-defined with specific imaging features, including mild hyper-intensity on T1-weighted MRI, hypo-intensity on T2-weighted MRI, and potential bone involvement, which can help in diagnosing the condition.
Late-onset juvenile xanthogranuloma: a case report.
Int J Dermatol
November 2024
Department of Dermatology, Venereology, and Leprology, College of Medical Sciences and Teaching Hospital, Bharatpur, Nepal.
Multiple juvenile xanthogranuloma.
Dermatol Online J
March 2024
Hospital Universitario San Juan de Alicante, San Juan de Alicante, Alicante, Spain.
Article Synopsis
- - Juvenile xanthogranuloma is the most common type of non-Langerhans cell histiocytosis seen in children, usually appearing as yellowish papules on areas like the head, neck, and upper trunk.
- - While most cases present as solitary lesions, some patients may have multiple lesions, which can sometimes be associated with other health issues.
- - A case study of a 2-month-old girl with 22 papules showcases the importance of monitoring for systemic disease in patients with multiple juvenile xanthogranulomas to ensure proper management.
Article Synopsis
- Juvenile xanthogranuloma (JXG) is a rare, non-cancerous skin condition mainly affecting children, often resolving on its own without treatment.
- In some cases, like the one discussed in the paper, JXG can appear as a single lesion that may require surgical removal if it causes functional issues.
- The case highlighted involves a child whose JXG lesion on the sole led to delays in walking and bearing weight, prompting the need for surgery.
Congenital Juvenile Xanthogranuloma in the Perioral Region: A Case Image.
Head Neck Pathol
April 2024
Department of Oral Diagnosis and Pathology, School of Dentistry, Universidade Federal do Rio de Janeiro, R. Rodolpho Paulo Rocco, N. 325, 1st Floor, Cidade Universitária, Rio de Janeiro, RJ, CEP: 21.941-902, Brazil.
Article Synopsis
- Juvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytosis in children, primarily affecting the skin, especially on the head and neck.
- A case study highlights congenital JXG in a 5-month-old boy, who had a specific nodule above his left upper lip with unique histopathological features.
- The article emphasizes the need for clinicians to recognize the diverse presentations of JXG, particularly congenital cases, for effective treatment.
Pediatric Orbital Juvenile Xanthogranuloma: A Case Series and Review of the Literature.
Ophthalmic Plast Reconstr Surg
September 2024
Department of Ophthalmology, The Children's Hospital at Westmead, Sydney Children's Hospital Network, Westmead.
Article Synopsis
- Juvenile xanthogranuloma (JXG) is a rare pediatric skin condition that usually appears as a single nodule, and orbital JXG is an even rarer form that affects the eye area.
- A review of three cases of orbital JXG revealed a diverse range of symptoms and no clear consensus on the best treatment, though steroids are commonly used.
- The classification of JXG is evolving, and it remains uncertain how orbital JXG fits into this classification, particularly regarding its biological mechanisms compared to cutaneous JXG.
Juvenile xanthogranuloma manifesting with LCH-associated neurodegenerative disease-like radiological findings.
Pediatr Blood Cancer
July 2024
Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Article Synopsis
- The text discusses two cases of juvenile xanthogranuloma (JXG) that presented symptoms resembling neurodegenerative disease (ND) linked with Langerhans cell histiocytosis (LCH).
- One patient experienced worsening neurological symptoms after undergoing chemotherapy, while the other had an unexpected improvement of symptoms after a period of radiological change.
- The findings aim to enhance understanding of JXG-related neurodegeneration and potentially guide future treatment options.
Successful treatment of a preterm infant with congenital juvenile xanthogranuloma presenting as a large intracranial tumor.
Pediatr Blood Cancer
July 2024
Department of Pediatrics, Juntendo University Faculty of Medicine, Bunkyo-ku, Tokyo, Japan.