Study Design: Case report.
Objective: To report a very rare case of juvenile xanthogranuloma (JXG) of the spine in an adult.
Summary Of Background Data: JXG is very rare in the spine, with only five prior reports in infants and children. To the best of our knowledge, this tumor has never been reported in an adult spine.
Methods: The patient is a 47-year-old woman who presented with bowel and bladder incontinence. Magnetic resonance imaging showed a very large lesion arising from the L2 vertebral body, with massive extension into the retroperitoneum with extensive intradural involvement. She had decreased rectal tone, had 4/5 strength in the right hip flexor, and had diminished sensation in her anterior right thigh and perineal region. She was otherwise neurologically intact. After preoperative embolization, a decompressive laminectomy was performed and the tumor was resected through a posterolateral transpedicular approach, followed by stabilization. Because of extensive involvement of retroperitoneum, complete resection was not possible.
Results: After pathologic evaluation of the specimen, a diagnosis of JXG was made. Patient underwent postoperative radiation therapy, and her neurologic examination improved significantly over the next several months.
Conclusions: To the best of our knowledge, this is the first reported case of JXG in an adult spine. Although complete resection of the tumor was not possible, decompression of the dural sac followed by postoperative radiation led to an excellent clinical outcome.
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http://dx.doi.org/10.1097/BRS.0b013e318201b7f5 | DOI Listing |
Pediatr Dermatol
January 2025
Department of Dermatology, University of new Mexico School of Medicine, Albuquerque, New Mexico, USA.
Juvenile xanthogranuloma (JXG) is the most common type of non-Langerhans cell histiocytosis and is characterized by a benign proliferation of dermal dendrocytes. We report two children diagnosed with JXG with reflectance confocal microscopy (RCM), a noninvasive imaging technology that allows for multi-layer visualization of the skin. This report demonstrates the emerging role of RCM as a painless diagnostic aid for JXG and other cutaneous diseases in children.
View Article and Find Full Text PDFAm J Ophthalmol Case Rep
December 2024
Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USA.
Purpose: To report a case of corneoscleral juvenile xanthogranuloma (JXG) with progressive anterior segment involvement refractory to topical steroids.
Observations: A 4-month-old male was referred for a new-onset subconjunctival lesion in the right eye. He was found to have a thickened, yellow corneoscleral lesion and hyphema, presumed to be ocular JXG.
Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG) family lesions, and Rosai-Dorfman-Destombes disease (RDD) are now classified by the World Health Organization (WHO) under the heading of histiocytic/dendritic cell neoplasms. Each disease may manifest as a focal lesion, as multiple lesions, or as a widespread aggressive systemic disease with visceral organ involvement. Erdheim-Chester disease (ECD) is a rare systemic disease process of adults with limited cases in children.
View Article and Find Full Text PDFLife (Basel)
December 2024
Institute of Dermatology, Department of Medical Area, University of Udine, 33100 Udine, Italy.
This literature review aims to comprehensively evaluate the clinical and dermoscopic presentations of common pediatric diseases among children with skin of color (SoC) while also addressing potential variations based on racial backgrounds. This review encompasses various conditions, such as nevi subtypes, viral infections, infestations, and inflammatory dermatoses, as well as hair diseases and abnormal vascular formations, occurring in pediatric populations. Overall, we identified 7 studies on nevi subtypes, 24 studies on skin infections, 6 on inflammatory dermatoses, 10 on hair diseases and disorders, and 14 on miscellaneous disorders that also satisfied our SoC- and race-specific criteria.
View Article and Find Full Text PDFInt 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.
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