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Archivos Argentinos de Pediatria Feb 2017Juvenile xanthogranuloma is a bening pathology and it represents the most common form of non-Langerhans cell histiocytosis. It is characterized by the presence of...
Juvenile xanthogranuloma is a bening pathology and it represents the most common form of non-Langerhans cell histiocytosis. It is characterized by the presence of papules or firm nodules of a pinkish or yellow-brownish nature, which mainly compromise the skin and, exceptionally, other organs. It is a self-limited entity having a spontaneous regression during the first five years of life. We report the case of a one-month-old patient who presented a congenital tumor in the abdomen, whose histopathology showed the presence of multinucleated giant Touton cells, which are typical of this pathology. We emphasize the rare occurrence of this type of lesion and the importance of the multiple differential diagnosis to be taken into account due to the age of the patient and the characteristics of the lesion.
Topics: Female; Humans; Infant; Xanthogranuloma, Juvenile
PubMed: 28097846
DOI: 10.5546/aap.2017.e9 -
Radiology Case Reports May 2023Juvenile xanthogranuloma (JX) is a non-Langerhans cell histiocytosis. Although precipitating factors remain unclear, it has been described mainly in infancy and early...
Juvenile xanthogranuloma (JX) is a non-Langerhans cell histiocytosis. Although precipitating factors remain unclear, it has been described mainly in infancy and early childhood. The giant variant of JX is a rare form that presents in infancy, measures over 2 cm and tends to involute only partly. Herein, we report a very rare localization of a giant JX in the parotid gland, discovered at age 1 month in an infant of a twin pregnancy and studied with ultrasound and magnetic resonance imaging.
PubMed: 36923389
DOI: 10.1016/j.radcr.2023.01.103 -
Andes Pediatrica : Revista Chilena de... Oct 2023Giant Juvenile Xanthogranuloma (GJXG) corresponds to an infrequent variant of Juvenile Xantho- granuloma (JXG) and is characterized by a lesion larger than 2 cm in...
UNLABELLED
Giant Juvenile Xanthogranuloma (GJXG) corresponds to an infrequent variant of Juvenile Xantho- granuloma (JXG) and is characterized by a lesion larger than 2 cm in diameter. It usually presents as a plaque but infrequently, presents as an ulcerated nodule.
OBJECTIVE
To report two cases of atypical presentation of GJXG, highlighting the importance of considering them in the differential diagnosis of large, ulcerated tumors in infants.
CLINICAL CASES
Case 1: A 4-month-old healthy male infant presented with a rapid and progressive growing left inguinal nodule, present since 2 months of age. At physical examination he presented with a 2.6 cm indurated erythematous nodule with central ulceration. Histological study of an incisional biopsy was compatible with JXG. Ophthalmologic involvement was ruled out. Because of functional impairment and parents worry complete surgical removal was performed. The patient had favorable evolution without local recurrence at 4 years of follow-up. Case 2: A 6-month-old healthy male infant presented with a 2.4 cm scapular crusted nodule of rapid and progressive growth, present since birth. Histological study of an incisional biopsy confirmed JXG. Ophthalmologic involvement was ruled out. After 18 months of periodic clinical follow-up, there was a progressive reduction in size of the lesion.
CONCLUSIONS
The cases presented highlight the importance of considering JXG in the differential diagnosis of large, ulcerated tumors in infants. When encountered to atypical JXG presentations, histologic studies help to confirm the diagnosis. Given the favorable prognosis of this diagnosis, periodic clinical follow-up is advised; in exceptional cases, surgical or ablative treatments may be considered.
Topics: Humans; Infant; Male; Xanthogranuloma, Juvenile; Biopsy; Diagnosis, Differential; Neoplasms
PubMed: 37975698
DOI: 10.32641/andespediatr.v94i5.4673 -
Cancer Control : Journal of the Moffitt... Oct 2014Dendritic and histiocytic cell neoplasms are rare malignancies that make up less than 1% of all neoplasms arising in lymph nodes or soft tissues. These disorders have... (Review)
Review
BACKGROUND
Dendritic and histiocytic cell neoplasms are rare malignancies that make up less than 1% of all neoplasms arising in lymph nodes or soft tissues. These disorders have distinctive disease biology, clinical presentations, pathology, and unique treatment options. Morphology and immunohistochemistry evaluation by a hematopathologist remains key for differentiating between these neoplasms. In this review, we describe tumor biology, clinical features, pathology, and treatment of follicular dendritic cell sarcoma, interdigitating dendritic cell sarcoma, indeterminate dendritic cell sarcoma, histiocytic sarcoma, fibroblastic reticular cell tumors, and disseminated juvenile xanthogranuloma.
METHODS
A literature search for articles published between 1990 and 2013 was undertaken. Articles are reviewed and salient findings are systematically described.
RESULTS
Patients with dendritic cell and histiocytic neoplasms have distinct but variable clinical presentations; however, because many tumors have recently been recognized, their true incidence is uncertain. Although the clinical features can present in many organs, most occur in the lymph nodes or skin. Most cases are unifocal and solitary presentations have good prognoses with surgical resection. The role of adjuvant therapy in these disorders remains unclear. In cases with disseminated disease, prognosis is poor and data on treatment options are limited, although chemotherapy and referral to a tertiary care center should be considered. Excisional biopsy is the preferred method of specimen collection for tissue diagnosis, and immunohistochemistry is the most important diagnostic method for differentiating these disorders from other entities.
CONCLUSIONS
Dendritic cell and histiocytic cell neoplasms are rare hematological disorders with variable clinical presentations and prognoses. Immunohistochemistry remains important for diagnosis. Larger pooled analyses or clinical trials are needed to better understand optimal treatment options in these rare disorders. Whenever possible, patients should be referred to a tertiary care center for disease management.
Topics: Dendritic Cells; Hematologic Neoplasms; Histiocytic Disorders, Malignant; Humans
PubMed: 25310210
DOI: 10.1177/107327481402100405 -
Cancer Cytopathology Apr 2011In pediatric patients, a cutaneous nodule is usually diagnosed by performing an excisional biopsy, but fine-needle cytology (FNC) is a safer and noninvasive diagnostic...
BACKGROUND
In pediatric patients, a cutaneous nodule is usually diagnosed by performing an excisional biopsy, but fine-needle cytology (FNC) is a safer and noninvasive diagnostic method widely used to obtain diagnostic specimens with little stress to the patient. The authors compared the ability of FNC and biopsy to differentiate Langerhans cell histiocytosis (LCH) from juvenile xanthogranuloma (JXG).
METHODS
Correlating cytological results with histological findings, the authors reviewed 27 patients (15 males and 12 females; mean age, 37 months; range, 1 month to 14 years) admitted to the University of Padua Department of Pediatrics from 1998 to 2010.
RESULTS
Cytology smears were adequate in all 27 (100%) patients: 14 (52%) were classified as having JXG, 12 (44%) as having LCH, and 1 (4%) as having a doubtful finding. A biopsy was also performed in 20 of these patients, and in all but 1, the 2 methods were completely concordant.
CONCLUSIONS
FNC is safe and useful in the diagnostic workup of pediatric patients with cutaneous nodules and has no contraindications to its use as the initial diagnostic procedure.
Topics: Adolescent; Antigens, CD; Antigens, Differentiation, Myelomonocytic; Biopsy, Fine-Needle; Child; Child, Preschool; Diagnosis, Differential; Female; Histiocytosis, Langerhans-Cell; Humans; Immunohistochemistry; Infant; Male; Reproducibility of Results; Sensitivity and Specificity; Skin; Xanthogranuloma, Juvenile
PubMed: 21491610
DOI: 10.1002/cncy.20132 -
Skin Research and Technology : Official... Sep 2022Molluscum contagiosum (MC), milia, keratosis pilaris (KP), verruca plana (VP), seborrheic keratosis (SK), and juvenile xanthogranuloma (JXG) are common papule dermatoses...
BACKGROUND
Molluscum contagiosum (MC), milia, keratosis pilaris (KP), verruca plana (VP), seborrheic keratosis (SK), and juvenile xanthogranuloma (JXG) are common papule dermatoses on the face of children that have a similar appearance. In vivo evaluation of facial papule dermatoses with reflectance confocal microscopy (RCM) is helpful in the diagnosis of these ambiguous lesions in children. The purpose of this study was to clarify the RCM characteristics of MC, milia, KP, VP, SK, and JXG and explore the clinical application value of RCM for these common facial papule dermatoses.
METHODS
We recruited 113 patients referred for unequivocal facial papule dermatosis, including 21 patients with MC, 17 patients with milia, 19 patients with KP, 36 patients with VP, 8 patients with SK, and 12 patients with JXG. We evaluated the characteristics and distinguishing features of the six kinds of facial papule dermatoses using RCM.
RESULTS
The main RCM features of the six dermatoses included a well-demarcated border of the lesion area. MC, milia and KP all manifested cyst-like structures, and their distinguishing features were the location of the cystic structures and the refractive index of the contents. Although VP, SK, and JXG did not have obvious cystoid structures, VP was typically characterized by uniformly distributed petal-like structures with a medium-to-high refractive index in the epidermis. With regard to SK, the characteristic features were an obviously thickened epidermis and cobblestone-like structures. JXG was mainly characterized by multiple large round and ovoid cells with a foamy cytoplasm, and discoid-shaped multinucleated large cells were diffusely distributed in the dermis.
CONCLUSION
RCM allows the real-time visualization of major key diagnostic and distinguishing features of common facial papule dermatoses in children, including MC, milia, KP, VP, SK, and JXG.
Topics: Child; Dermoscopy; Diagnosis, Differential; Facial Dermatoses; Humans; Keratosis, Seborrheic; Microscopy, Confocal; Skin Neoplasms; Warts
PubMed: 35726961
DOI: 10.1111/srt.13170 -
Indian Journal of Dermatology 2015A 3-month-old female patient with a giant ulcerated nodule over the back since birth was diagnosed as congenital giant juvenile xanthogranuloma (JXG) based on clinical...
A 3-month-old female patient with a giant ulcerated nodule over the back since birth was diagnosed as congenital giant juvenile xanthogranuloma (JXG) based on clinical and histopathological examination. Congenital giant JXG with ulceration at birth is a rare presentation of JXG and commonly misdiagnosed. This case emphasizes the importance of being aware of the myriad presentations of JXG in order to make a correct diagnosis and avoid unnecessary investigations or treatment.
PubMed: 26288431
DOI: 10.4103/0019-5154.160515 -
JAAD Case Reports Oct 2020
PubMed: 32995422
DOI: 10.1016/j.jdcr.2020.07.034 -
EFORT Open Reviews Jun 2018Non-Langerhans cell histiocytosis (N-LCH) summarizes a group of rare diseases with different clinical presentations, pathogenesis and morphology. These include primary...
Non-Langerhans cell histiocytosis (N-LCH) summarizes a group of rare diseases with different clinical presentations, pathogenesis and morphology. These include primary cutaneous N-LCH, cutaneous N-LCH with systemic involvement, and primary extracutaneous systemic forms with occasional cutaneous involvement.The juvenile (JXG) and non-juvenile xanthogranuloma (N-JXG) family of histiocytoses are N-LCH: the JXG family consisting of the JXG (cutaneous), xanthoma disseminatum (cutaneous and systemic) and Erdheim-Chester disease (ECD; systemic); and the N-JXG family consisting of the solitary reticulohistiocytoma (cutaneous), multicentric reticulohistiocytosis (cutaneous and systemic) and Rosai-Dorfman disease (RDD; systemic).ECD is a clonal disorder from the JXG family of N-LCH; RDD is a reactive proliferative entity from the non-juvenile xanthogranuloma family of N-LCH.ECD and RDD N-LCH are rare disorders, which are difficult to diagnose, with multi-organ involvement including bone and systemic symptoms, and which respond to therapy in an unpredictable way.The key to successful therapy is accurate identification at tissue level and appropriate staging. Patients should be observed and monitored in a long-term pattern. Prognosis depends on disease extent and the organs involved; it is generally good for RDD disease and variable for ECD. Cite this article: 2018;3:381-390. DOI: 10.1302/2058-5241.3.170047.
PubMed: 30034819
DOI: 10.1302/2058-5241.3.170047 -
Cureus Aug 2020Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis that typically presents as a solitary lesion in infancy. Multiple lesions, especially in patients...
Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis that typically presents as a solitary lesion in infancy. Multiple lesions, especially in patients over one year of age, are rarely described in the literature. The authors report a case of a 17-year-old female who presented with multiple asymptomatic nodules and plaques. The diagnosis of xanthogranuloma was confirmed with histopathologic examination of foamy histiocytes and the characteristic Touton giant cells. The expected course of multiple JXG in older patients may differ from those presenting with a solitary lesion earlier in life.
PubMed: 32884872
DOI: 10.7759/cureus.9516