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Clinics in Perinatology Mar 2021Langerhans cell histiocytosis, Rosai-Dorfman disease, and juvenile xanthogranuloma may present at birth or any time afterward. Some patients have minimal skin or lymph... (Review)
Review
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. Many treatment regimens have been applied to these diseases, but those directed at myeloid cells, such as cytarabine and clofarabine or mutation-targeting inhibitors, are gaining favor. This article provides information on the pathophysiology, clinical presentation, evaluation guidelines, and treatment of these uncommon tumors of neonates.
Topics: Histiocytosis, Langerhans-Cell; Histiocytosis, Sinus; Humans; Mutation; Skin; Xanthogranuloma, Juvenile
PubMed: 33583503
DOI: 10.1016/j.clp.2020.11.008 -
International Medical Case Reports... 2020Juvenile Xanthogranuloma (JXG) is a relatively uncommon non-Langerhans cell histiocytosis, which often occurs at an early age and is usually asymptomatic. Herein, we...
Juvenile Xanthogranuloma (JXG) is a relatively uncommon non-Langerhans cell histiocytosis, which often occurs at an early age and is usually asymptomatic. Herein, we present the case of a 17-year-old man with numerous asymptomatic yellow-brown papulonodular lesions with a symmetric distribution on upper and lower extremities, face, and trunk, developed over the past 4 years. In the histopathologic examination, histiocytes with a Touton-like appearance were observed in favor of xanthogranuloma. The patient was treated with isotretinoin 20 mg daily for 2 months, which surprisingly led to the progression of lesions and thus was discontinued. Although JXG may cause severe morbidities in some circumstances, it is a self-limiting benign disorder and patients should be assured regarding the benign self-regressive nature of the disease.
PubMed: 32158278
DOI: 10.2147/IMCRJ.S240115 -
Journal of Current Glaucoma Practice 2022To report an ocular juvenile xanthogranuloma (JXG) case presented with buphthalmos, corneal cloudiness, and normal intraocular pressure (IOP) in the neonatal period and...
AIM
To report an ocular juvenile xanthogranuloma (JXG) case presented with buphthalmos, corneal cloudiness, and normal intraocular pressure (IOP) in the neonatal period and treated with Ahmed glaucoma valve (AGV) implantation.
BACKGROUND
JXG is a rare disorder predominantly seen in infants, but the neonatal presentation is extraordinary. Although spontaneous hyphema is a common presenting sign in JXG, buphthalmos and corneal opacity in the neonatal period were reported only in one case, which had high IOP values at presentation.
CASE PRESENTATION
Sixteen-day-old male patient presented with buphthalmos, diffuse corneal clouding, and 11 mm Hg of IOP value in the right eye. IOP increased to 28 mm Hg three weeks later, and spontaneous hyphema developed, which did not respond to antiglaucomatous medications and topical corticosteroids. AGV was implanted, and the IOP decreased to 13 mm Hg postoperatively. In the follow-ups, numerous firm yellowish nodules were noticed on the patient's skin during the examination under general anesthesia. Histopathological examination of the skin nodules was compatible with the diagnosis of JXG. Lens subluxation and phacodonesis were developed during the follow-up and were managed with pars plana lensectomy. After a silent period of 3 months, epithelial ingrowth was determined around the side port entrance. Unfortunately, the ingrowth did not respond to cryotherapy and resulted in phthisis bulbi. Pathological evaluation of the enucleated phthisic eye revealed posterior segment involvement.
CONCLUSION
Ocular JXG can be present with buphthalmos, corneal opacity, and normal IOP values without any skin lesions in the neonatal period. Neonatal presentation of JXG may be associated with limited medical therapy response and aggressive disease course.
CLINICAL SIGNIFICANCE
This case report introduces the second ocular JXG case, which presented with buphthalmos and corneal cloudiness, and the third pathologically proven posterior segment involvement of JXG in the literature.
HOW TO CITE THIS ARTICLE
Dericioglu V, Sevik MO, Eraslan M, Juvenile Xanthogranuloma Presented with Buphthalmos and Corneal Clouding in Neonatal Period: A Case Report. J Curr Glaucoma Pract 2022;16(2):128-131.
PubMed: 36128087
DOI: 10.5005/jp-journals-10078-1369 -
European Spine Journal : Official... Jul 2018We present a case report that describes neoadjuvant denosumab therapy initiated in a child with a solitary giant cell-rich juvenile xanthogranuloma tumor involving the... (Review)
Review
Solitary juvenile xanthogranuloma in the spine pretreated with neoadjuvant denosumab therapy followed by surgical resection in a 5-year-old child: case report and literature review.
PURPOSE
We present a case report that describes neoadjuvant denosumab therapy initiated in a child with a solitary giant cell-rich juvenile xanthogranuloma tumor involving the spine, and review the current literature.
METHODS
A giant cell-rich histiocytic lesion involving the 11th thoracic vertebral body was identified in a healthy 5-year-old girl with persistent back and pelvic pain for several months. Imaging examinations and an open biopsy were performed to obtain a definite pathologic diagnosis. As the tumor appeared to be aggressive in nature, we administered adjuvant therapy with denosumab preoperatively and then performed a total spondylectomy.
RESULTS
Histopathology confirmed that the tumor was juvenile xanthogranuloma. No tumor metastases or recurrence were detected at the 3-year follow-up, and the patient was asymptomatic.
CONCLUSIONS
In giant cell-rich tumors, denosumab is occasionally used as neoadjuvant or adjuvant therapy, especially for tumors in difficult locations or with substantial soft tissue extensions. Rare adverse events in children include skin infections and disruption of calcium homeostasis. Surgical treatment is aimed at removing the tumor and relieving the symptomatic spinal cord compression. Use of denosumab as neoadjuvant therapy for juvenile xanthogranuloma involving the spine has not been reported previously.
Topics: Bone Density Conservation Agents; Child, Preschool; Denosumab; Female; Humans; Neoadjuvant Therapy; Orthopedic Procedures; Spinal Neoplasms; Thoracic Vertebrae; Treatment Outcome; Xanthogranuloma, Juvenile
PubMed: 29876657
DOI: 10.1007/s00586-018-5651-8 -
American Journal of Ophthalmology Case... Mar 2023To report a rare case of an eyelid lesion in an adult, with histological features of juvenile xanthogranuloma (JXG).
PURPOSE
To report a rare case of an eyelid lesion in an adult, with histological features of juvenile xanthogranuloma (JXG).
OBSERVATIONS
Juvenile xanthogranuloma primarily affects the skin of infants and young children. It infrequently can involve the structures of the eye and orbit and rarely occurs in individuals beyond the second decade of life. We present a case of adult onset xanthogranuloma (AXG) involving the eyelid of a 29-year-old female. This lesion required management with multiple treatment modalities.
CONCLUSIONS
This is a rare example of an eyelid xanthogranuloma in an adult. As such, JXG-like lesions should be included as a differential diagnosis for lesions of the eye and orbit in adults. Surgical management may be required if there is no response to intralesional steroids.
PubMed: 36544751
DOI: 10.1016/j.ajoc.2022.101775 -
Indian Pediatrics Jun 2022
Topics: Glottis; Humans; Xanthogranuloma, Juvenile
PubMed: 35695144
DOI: No ID Found -
Revista Paulista de Pediatria : Orgao... 2019To report a rate case of Juvenile xanthogranuloma in a newborn infant.
OBJECTIVE
To report a rate case of Juvenile xanthogranuloma in a newborn infant.
CASE DESCRIPTION
We present the case of a 31-week preterm newborn with multiple skin lesions whose clinical, histological and immunohistochemical findings allowed the diagnosis of juvenile xanthogranuloma. Currently, the patient has nine months-old, and there is no aggravation of the skin lesions or evidence of extra-cutaneous involvement, particularly ophthalmic.
COMMENTS
Juvenile xanthogranuloma is a rare and benign condition, included in the vast group of non-Langerhans histiocytosis. It typically occurs in the pediatric age and may have a neonatal presentation. It affects predominantly the skin, in the form of papules or yellow and/or erythematous nodules and could be asymptomatic, multiple or solitary. Extra-cutaneous involvement, is more common in toddlers and when multiple lesions are present. The eye is the most affected site. We highlight this clinical case by its presentation in the neonatal period and in the form of multiple lesions, which bestows an increased risk of extra-cutaneous involvement, although this has not yet been verified.
Topics: Biopsy; Diagnosis, Differential; Gestational Age; Humans; Immunohistochemistry; Infant; Male; Patient Care; Xanthogranuloma, Juvenile
PubMed: 30810696
DOI: 10.1590/1984-0462/;2019;37;2;00013 -
International Journal of Dermatology May 2024
PubMed: 38773781
DOI: 10.1111/ijd.17263 -
Canadian Association of Radiologists... May 2016Pulmonary opacities/nodules are common findings on computed tomography examinations, which may represent an underlying infections or malignancy. However, not every... (Review)
Review
Pulmonary opacities/nodules are common findings on computed tomography examinations, which may represent an underlying infections or malignancy. However, not every pulmonary nodule or opacity represents malignancy or infection. We present a pictorial essay illustrating common as well as obscure noninfectious, nonmalignant pulmonary lesions. Lesions discussed include organizing pneumonia, Langerhans cell histiocytosis, pulmonary amyloidosis, hyalinizing granuloma, tumourlet (benign localized neuroendocrine cell proliferations), atypical alveolar hyperplasia, inflammatory myofibroblastic tumour, papillary alveolar adenoma, plasma cell granuloma, juvenile xanthogranuloma, and sclerosing hemangiomas. We discuss the clinical presentation, prevalence, radiographic clues, pathology, and diagnostic pitfalls of these rare lesions.
Topics: Humans; Lung; Lung Diseases; Tomography, X-Ray Computed
PubMed: 26690551
DOI: 10.1016/j.carj.2015.07.004 -
Ocular Oncology and Pathology Sep 2018Juvenile xanthogranuloma (JXG) is a rare and benign tumor in infants. A solitary lesion on the eyelid has been reported in patients with JXG. We report a 15-year-old boy...
Juvenile xanthogranuloma (JXG) is a rare and benign tumor in infants. A solitary lesion on the eyelid has been reported in patients with JXG. We report a 15-year-old boy with multiple involvement of JXG on both eyelids. A mass on the left inner canthus was resected because of disturbance of the visual field and a risk of malignancy in terms of central ulceration in the lesion. The mass was examined by light microscopy. The mass had Touton giant cells with a wreath of nuclei surrounded by foamy histiocytes. No malignancy was observed. The mass showed no recurrence after resection.
PubMed: 30320084
DOI: 10.1159/000478101