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Plastic and Reconstructive Surgery.... Jul 2021We treated a patient with juvenile xanthogranuloma on the upper lip. A yellow, elastic, hard tumor on the upper lip was evident from birth, which gradually increased in...
We treated a patient with juvenile xanthogranuloma on the upper lip. A yellow, elastic, hard tumor on the upper lip was evident from birth, which gradually increased in size. The patient was examined at our department at the age of 7 months, at which time the mass extended from the upper lip to the nasal cavity and measured approximately 1 cm. There was a risk that the mass might obstruct the nasal cavity, and an incisional biopsy was conducted to obtain a definitive diagnosis. In histopathological testing, the patient was diagnosed with a juvenile xanthogranuloma. Part of the mass still remains on the upper lip, but has not increased in size during postoperative monitoring. Juvenile xanthogranuloma on the upper lip is extremely rare, and to the best of our knowledge, this is only the fourth case to be reported in the plastic surgery literature in English. In most cases, juvenile xanthogranuloma regresses spontaneously, and unnecessary surgery is to be avoided. The possibility of juvenile xanthogranuloma should always be considered for masses that increase in size in infants and young children, and it is important to reach a definitive diagnosis by skin biopsy.
PubMed: 34290944
DOI: 10.1097/GOX.0000000000003712 -
Asian Journal of Surgery Jun 2023
Topics: Humans; Xanthogranuloma, Juvenile; Magnetic Resonance Imaging
PubMed: 36473813
DOI: 10.1016/j.asjsur.2022.11.121 -
La Clinica Terapeutica 2022Neurofibromatosis type 1 (NF1), is a rare genetic disorder that may involve almost every organ system in the body such as cutaneous, ophthalmologic and central and... (Review)
Review
Juvenile xanthogranuloma in neurofibromatosis type 1. Prevalence and possible correlation with lymphoproliferative diseases: experience of a single center and review of the literature.
Neurofibromatosis type 1 (NF1), is a rare genetic disorder that may involve almost every organ system in the body such as cutaneous, ophthalmologic and central and peripheral nervous system. Cutaneous findings are usually the first sign of the disease. In this study, we investigate the real prevalence of xanthogranulomas juvenile (JXG) and possible correlation with lymphoproliferative diseases. This is a retrospective study conducted on a population with NF1 followed by February 1983 to February 2022 at the "Sapienza" University of Rome, Italy. We investigate the real prevalence of juvenile xanthogranuloma in NF1 and possible correlation with lymphoproliferative diseases. JXG was present in 39 cases (3.1%). JXG is more frequent in NF1 than in the general population while the possible association with lymphoproliferative diseases in NF1 remains controversial.
Topics: Humans; Neurofibromatosis 1; Prevalence; Retrospective Studies; Skin; Xanthogranuloma, Juvenile
PubMed: 35857053
DOI: 10.7417/CT.2022.2445 -
Indian Journal of Dermatology and... 1972
PubMed: 29144315
DOI: No ID Found -
Indian Pediatrics Jun 2022
Topics: Glottis; Humans; Xanthogranuloma, Juvenile
PubMed: 35695144
DOI: No ID Found -
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 -
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 -
The British Journal of Radiology Dec 2016The objectives of this article were: (1) to review common and rare manifestations of systemic and pulmonary Langerhans cell histiocytosis, Rosai-Dorfman disease,... (Review)
Review
The objectives of this article were: (1) to review common and rare manifestations of systemic and pulmonary Langerhans cell histiocytosis, Rosai-Dorfman disease, Erdheim-Chester disease and juvenile xanthogranuloma; (2) to provide the reader with important pathologic, epidemiologic and clinical features of these diseases. The histiocytoses are a diverse group of diseases which typically manifest with multiorgan involvement. Understanding the pathologic, epidemiologic and clinical features of these entities can help the radiologist suggest an accurate diagnosis of histiocytosis when typical imaging features are encountered.
Topics: Cardiovascular Diseases; Diagnostic Imaging; Histiocytosis, Langerhans-Cell; Humans; Thoracic Diseases
PubMed: 27603510
DOI: 10.1259/bjr.20160347 -
Pediatric Investigation Sep 2023
PubMed: 37736360
DOI: 10.1002/ped4.12398 -
AJNR. American Journal of Neuroradiology May 2016Juvenile xanthogranuloma is a non-Langerhans cell histiocytosis primarily affecting children. The purpose of this study was to characterize the imaging features of...
BACKGROUND AND PURPOSE
Juvenile xanthogranuloma is a non-Langerhans cell histiocytosis primarily affecting children. The purpose of this study was to characterize the imaging features of histologically confirmed pediatric head and neck juvenile xanthogranuloma.
MATERIALS AND METHODS
A retrospective review was performed of medical records and imaging of histologically confirmed head and neck juvenile xanthogranuloma.
RESULTS
Ten patients (6 girls, 4 boys) 1 month to 12 years of age were imaged with ultrasound only (n = 1), CT only (n = 2), CT and ultrasound (n = 1), MR imaging only (n = 3), or MR imaging and CT (n = 3). Masses were solitary in 9 patients and multiple in 1. Solitary masses were located in the external auditory canal, infra-auricular region, infratemporal fossa with intracranial extension, frontal scalp, and subperiosteal space eroding the calvaria and along the dura. One patient with disseminated disease had scalp-, calvarial-, and dural-based masses. Clinical presentation included a mass or alteration in function. On sonography, juvenile xanthogranuloma appeared hypoechoic. On contrast-enhanced CT, masses appeared homogeneous and isoattenuating with muscle and sometimes eroded bone. On MR imaging, compared with the cerebral cortex, the masses appeared hyper- or isointense on T1 and hypo- or isointense on T2, had decreased diffusivity, and enhanced homogeneously. Juvenile xanthogranuloma was not included in the differential diagnosis in any case.
CONCLUSIONS
Head and neck juvenile xanthogranuloma has varied manifestations. Mild hyperintensity on T1, hypointensity on T2 compared with the cerebral cortex, decreased diffusivity, and homogeneous enhancement are characteristic. Awareness of these features should prompt radiologists to include juvenile xanthogranuloma in the differential diagnosis of pediatric head and neck masses.
Topics: Child; Child, Preschool; Diagnosis, Differential; Female; Head; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Multimodal Imaging; Neck; Retrospective Studies; Tomography, X-Ray Computed; Ultrasonography; Xanthogranuloma, Juvenile
PubMed: 26744443
DOI: 10.3174/ajnr.A4644