scholarly journals Juvenile Xanthogranuloma

2020 ◽  
Vol 2 (1) ◽  
pp. 52-57
Author(s):  
Irma Primawati

Juvenile xanthogranuloma is the most common form of non-Langerhans-cells histiocytosis or non-X histiocytosis. Most often affecting infants and young children.A case of  juvenile xanthogranuloma in a 3-month-old male infant has been reported. There were multiple, discrete, firm, red-brownish, orange nodules, round and oval in shape, well-defined, 0.5–2 cm in diameter, disseminated on his scalp, face, trunk, upper and lower extremities, also genitalia and some of them with ulceration on its surface. No mucous membrane and systemic involvement. Pathology result revealed Touton giant cells that characteristic to juvenile xanthogranuloma although the other diseases might contain these cells such as Hashimoto-Pritzker disease and dermatofibroma. Within few years, the lesions could regression spontaneously and leaving a flat, atrophic scar or an area of altered pigmentation.

Rare Tumors ◽  
2021 ◽  
Vol 13 ◽  
pp. 203636132199733
Author(s):  
Yasser Elborai ◽  
Nawaf Alkhayat ◽  
Ghaleb Elyamany ◽  
Mohammad Alshahrani ◽  
Walid Ibrahim ◽  
...  

Rare pediatric tumors are heterogeneous group containing a variety of histopathological diseases, they represent approximately 10% of all childhood cancers. These rare tumors had a diversity of histology and clinical behaviors that pose different challenges to the investigators. Exploring different pediatric rare tumors. The data were reviewed, retrospectively, through the medical records of seven rare pediatric diseases between 2012 and 2019. Giant cell fibroblastoma (GCF) presented as painless swelling in the trunk, positive for CD34 with PTEN gene mutation. Neuroglial heterotopic tissue presented in 7 days old girl with facial asymmetry and bulging in the oral cavity, maximal de-bulking was done, histopathology was positive for GFAP and S100p. Left side neck mass, surgically excised revealed non-metastatic salivary grand mucoepidermoid carcinoma. Follow up without any chemotherapy or radiotherapy for 5 years with complete remission. Mesenchymal chondrosarcoma (MCS) presented in maxillofacial bones by persistent nasal bleeding, HEY1-NCOA2 fusion gene confirmed the diagnosis. Extra-osseous Ewing sarcoma (EES) presented as rubbery painless swelling in the scalp with fusion transcript involving EWSR1-FL11. Juvenile xanthogranuloma (JXG) presented by butter fly like skin patch in the face with foamy histiocytes in upper dermis with few Touton giant cells, extensive systemic involvement of lung and bone marrow. Metastatic ovarian choriocarcinoma with choriocarcinoma syndrome received induction two different lines of chemotherapy and consolidated with autologous stem cell transplant. Seven pediatric rare tumors, with different aspects of challenges in diagnosis and management, despite the absence of formal protocols and rarity of other center experiences.


2020 ◽  
Vol 24 (6) ◽  
pp. 340-347
Author(s):  
Samet Aktaş ◽  
Oktay Çakmakçı

Background and Study Aim. In this study, it is aimed to evaluate the power and force parameters of lower and upper extremities of the individual and team athletes who practice different branches. Material and Methods. 32 elite athletes, most of whom are university students, with an average age of 21.16 ± 2.08, who are active in sports, participated in the study. The groups were formed from Boxing and Handball (BG and HG) branches in which the upper extremities are used predominantly and from Taekwondo and Football (TG and FG) branches in which the lower extremities are used predominantly. The anaerobic power values of each individual’s upper and lower extremities were measured by a Monark 894E. The isokinetic force values of dominant arms and legs were measured by a Cybex humac norm device. Results. After examination of the data it was found that the upper extremity power values of BG and HG were significantly higher than TG when the top values of peak powers (PP) were evaluated.  In the evaluations with respect to leg 60°/s and 180°/s extension and flexion; HG and FG was significantly higher than TG in 60°/s extension at PP; HG and FG were significantly indifferent but they were significantly higher than BG and TG in 60°/s flexion at PP. In the comparisons of the groups’ 60°/s and 180°/s extension and flexion values of lower extremities anaerobic power and isokinetic force and lower extremities’ wingate values, a number of positive relations were found between all of the groups. All of the groups were positively related to each other in terms of upper extremities wingate and 60°/s, 180°/s internal and external isokinetic forces.  Conclusions. Both the isokinetic arm force values and arm wingate levels were higher in the branches in which the upper extremities are used predominantly than the branches in which the lower extremities are used predominantly. On the other hand, the difference seen in the upper extremities was not seen in the lower extremities.


2020 ◽  
Vol 26 (4) ◽  
pp. 449-453
Author(s):  
Jacob A. Kahn ◽  
Jeffrey T. Waltz ◽  
Ramin M. Eskandari ◽  
Cynthia T. Welsh ◽  
Michael U. Antonucci

The authors report an unusual presentation of juvenile xanthogranuloma (JXG), a non–Langerhans cell histiocytosis of infancy and early childhood. This entity typically presents as a cutaneous head or neck nodule but can manifest with more systemic involvement including in the central nervous system. However, currently there is limited information regarding specific imaging features differentiating JXG from other neuropathological entities, with diagnosis typically made only after tissue sampling. The authors reviewed the initial images of a young patient with shunt-treated hydrocephalus and enlarging, chronic, extraaxial processes presumed to reflect subdural collections from overshunting, and they examine the operative discovery of a mass lesion that was pathologically proven to be JXG. Their results incorporate the important associated histological and advanced imaging features, including previously unreported metabolic activity on FDG PET. Ultimately, the case underscores the need to consider JXG in differential diagnoses of pediatric intracranial masses and highlights the potential role of PET in the initial diagnosis and response to treatment.


2021 ◽  
pp. 1-8
Author(s):  
Dekui Cheng ◽  
Fengyu Yang ◽  
Ziji Li ◽  
Fan Qv ◽  
Wei Liu

<b><i>Introduction:</i></b> Xanthogranuloma of the sellar region is a rare benign lesion, and there are few cases reported in children. Its histogenesis is controversial, and it is difficult to strictly differentiate it from craniopharyngioma (CP), Rathke’s cleft cyst, or pituitary adenoma. <b><i>Case Presentation:</i></b> A 16-year-old boy presented with a rare xanthogranuloma of the sellar region after complaining of retardation of growth 5 years previously. The ophthalmologic evaluation revealed no visual field disturbance. Endocrinological examination revealed hypopituitarism. Magnetic resonance imaging showed an intrasellar mass extending into the suprasellar region and compressing the optic chiasma, which appeared mixed signals on T1-weighted images. Endonasal transsphenoidal resection of the tumor was performed. Histological analysis of the tumor sections demonstrated granulomatous tissue with cholesterol clefts, hemosiderin deposits, fibrous tissues, multinucleated giant cells, and lymphocyte. Thus, the tumor was pathologically diagnosed as xanthogranuloma of the sellar region, which is different from adamantinomatous CP. There was no epithelial tissue in any part of the tumor including tumor capsule but have focal necrosis and calcification. His endocrinological dysfunction did not recover, so a hormonal replacement was continuously required. <b><i>Conclusion:</i></b> Xanthogranuloma of the sellar region is a rare entity but must be considered in the differential diagnosis of lesions of the sellar region, even in pediatric population. We should think about this disease when dealing with children with stunted growth accompanied by a long medical history. Our case demonstrates the natural progression of the disease, suggesting that xanthogranuloma of the sellar region without epithelial components may be an independent disease.


2016 ◽  
Vol 71 (3) ◽  
pp. 372-376 ◽  
Author(s):  
L Otten ◽  
A Bosy-Westphal ◽  
J Ordemann ◽  
E Rothkegel ◽  
N Stobäus ◽  
...  

Author(s):  
Cody R. Perskin ◽  
Abhijit Seetharam ◽  
Brian H. Mullis ◽  
Andrew J. Marcantonio ◽  
John Garfi ◽  
...  

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