Juvenile xanthogranuloma (XGJ) is the most common form of non-Langerhansian histiocytosis. We report a pediatric case of multiple XGJ without visceral involvement in a 6-month-old female infant who was hospitalized wi...Juvenile xanthogranuloma (XGJ) is the most common form of non-Langerhansian histiocytosis. We report a pediatric case of multiple XGJ without visceral involvement in a 6-month-old female infant who was hospitalized with disseminated congenital skin nodules, of firm consistency, of variable diameter ranging from a few millimetres to 1 cm, involving the face, scalp, trunk and limbs. There was no mucosal localization. The remainder of the somatic examination was unremarkable. The diagnosis of disseminated XGJ was confirmed by skin biopsy without visceral or systemic involvement. Therapeutic abstention was recommended. This observation underlines the fact that XGJ is a rare clinical entity that should not be overlooked when faced with congenital skin nodules.展开更多
Juvenile xanthogranuloma （JXG） is an uncommon benign disease, which usually occurs on the skin of head and neck region, as a single or multiple nodular lesions. Tracheal involvement of JXG is extremely rare, and onl...Juvenile xanthogranuloma （JXG） is an uncommon benign disease, which usually occurs on the skin of head and neck region, as a single or multiple nodular lesions. Tracheal involvement of JXG is extremely rare, and only few cases have been reported in literature. Herein we presented an additional new case and discussed the optimal management of this uncommon disease.展开更多
Xanthogranulomas(XG) are benign proliferative disorder of histiocytes, a non-Langerhans cell histiocytosis. Whose etiology is unknown. The nature of these lesions is controversial and could be either reactive or neopl...Xanthogranulomas(XG) are benign proliferative disorder of histiocytes, a non-Langerhans cell histiocytosis. Whose etiology is unknown. The nature of these lesions is controversial and could be either reactive or neoplastic;the presence of monoclonal cells does, however, favor the second hypothesis. Xanthogranuloma is frequently found in young adults and children(under 20 years old), mainly in the skin. In about 5%-10% of all Juvenile XG(JXG) cases xanthogranuloma are extracutaneous. Within this group, the site most frequently involved is the eye. Other involved organs are heart, liver, adrenals, oropharynx, lung, spleen, central nervous system and subcutaneous tissue, although involvement of the spine is uncommon. Isolated lesions involving the sacral region are extremely rare. To date, this is the first reported case of a giant JXG arising from S1 with extension into the pelvic region in an adult spine.展开更多
Background:Juvenile xanthogranuloma(JXG)is a rare disorder that belongs to the broad group of non-Langerhans cell histiocytosis.It is characterized by one or more nodules with predilection sites on the head,neck,and t...Background:Juvenile xanthogranuloma(JXG)is a rare disorder that belongs to the broad group of non-Langerhans cell histiocytosis.It is characterized by one or more nodules with predilection sites on the head,neck,and trunk,and lesions that may be several millimeters in diameter.These are reddish or yellowish benign papules or nodules that usually resolve spontaneously.The involvement of organs other than the skin is termed systemic juvenile xanthogranuloma(SJXG).The eye is the most frequent extracutaneous location of the JXG.Case presentation:We report a case of SJXG in a male child,with onset in the second month of life.He presented with several nodules,approximately5 mm in diameter and tan-orange in color,located on the head,face,and trunk.The nodules enlarged to 10 mm in diameter,and new lesions were found in the right eye,which resulted in spontaneous hyphema and secondary glaucoma without treatment.The pathological findings suggested that the nodule was of histiocytic origin,and immunohistochemical analysis resulted in the diagnosis of JXG.Chemotherapy based on the Langerhans cell histiocytosis(LCH)regimen resulted in a good prognosis.Conclusion:SJXG has low morbidity,but is unpredictable,and rare and self-limited.Treatment is required for patients with extracutaneous involvement,who may have increased morbidity.The LCH-Ⅲprotocol of the International Histiocyte Society is the most commonly used and effective chemotherapy regimen.展开更多
Introduction:Histiocytoses are localized or systemic diseases that can broadly be classified into Langerhans cell histiocytosis and non-Langerhans cell histiocytosis.Juvenile Xanthogranuloma(JXG)belongs to the latter ...Introduction:Histiocytoses are localized or systemic diseases that can broadly be classified into Langerhans cell histiocytosis and non-Langerhans cell histiocytosis.Juvenile Xanthogranuloma(JXG)belongs to the latter group,which occurs in around 0.5%population younger than 5 years,and presents as solitary or multiple lesions.Here,we present a case of disseminated JXG in an infant with the lesions regressing spontaneously.Case presentation:A 7-month-old boy presented with multiple erythematous nodular lesions over the body excluding limbs for 3 months.Initial laboratory work up was normal.Skin histopathology showed a dermal nodule with sheets of histiocytes exhibiting grooved vesicular nuclei and pale eosinophilic cytoplasm along with touton giant cells.Immunohistochemistry markers further confirmed the diagnosis.Lesions healed completely with symptomatic relief in 1.5 years and no recurrence occurred.Discussion:Disseminated JXG is a benign childhood disorder that can sometimes be associated with internal organs involvement mainly bones,eyes,and brain.Serious complications may follow in case of extracutaneous spread.Most frequently,JXG follows the occurrence of another neoplastic disorder but can sometimes appear de novo.Skin biopsy is required for the diagnosis and better education of the family.Conclusion:JXG in infancy is a rarity in South Asia.Therefore,along with the clinical judgment,clinicians must also get histological confirmation in order to manage this disorder successfully.Close surveillance for multiorgan involvement is also required to avoid any irreversible sequalae.展开更多
Introduction Xanthogranuloma is a kind of rare,non-Langerhans cell histiocytosis that most commonly occurs in infancy or early childhood.Adult xanthogranuloma was first described by Gartmann and Tritsch in 1963,and th...Introduction Xanthogranuloma is a kind of rare,non-Langerhans cell histiocytosis that most commonly occurs in infancy or early childhood.Adult xanthogranuloma was first described by Gartmann and Tritsch in 1963,and there have been only approximately one hundred cases reported until now[1].Diagnosis of this disease might be complicated due to its unusual forms,and a group of dermatoses need to be differentiated.We here described a case of multiple adult xanthogranuloma (MAXG) in a young man.展开更多
文摘Juvenile xanthogranuloma (XGJ) is the most common form of non-Langerhansian histiocytosis. We report a pediatric case of multiple XGJ without visceral involvement in a 6-month-old female infant who was hospitalized with disseminated congenital skin nodules, of firm consistency, of variable diameter ranging from a few millimetres to 1 cm, involving the face, scalp, trunk and limbs. There was no mucosal localization. The remainder of the somatic examination was unremarkable. The diagnosis of disseminated XGJ was confirmed by skin biopsy without visceral or systemic involvement. Therapeutic abstention was recommended. This observation underlines the fact that XGJ is a rare clinical entity that should not be overlooked when faced with congenital skin nodules.
文摘Juvenile xanthogranuloma （JXG） is an uncommon benign disease, which usually occurs on the skin of head and neck region, as a single or multiple nodular lesions. Tracheal involvement of JXG is extremely rare, and only few cases have been reported in literature. Herein we presented an additional new case and discussed the optimal management of this uncommon disease.
文摘Xanthogranulomas(XG) are benign proliferative disorder of histiocytes, a non-Langerhans cell histiocytosis. Whose etiology is unknown. The nature of these lesions is controversial and could be either reactive or neoplastic;the presence of monoclonal cells does, however, favor the second hypothesis. Xanthogranuloma is frequently found in young adults and children(under 20 years old), mainly in the skin. In about 5%-10% of all Juvenile XG(JXG) cases xanthogranuloma are extracutaneous. Within this group, the site most frequently involved is the eye. Other involved organs are heart, liver, adrenals, oropharynx, lung, spleen, central nervous system and subcutaneous tissue, although involvement of the spine is uncommon. Isolated lesions involving the sacral region are extremely rare. To date, this is the first reported case of a giant JXG arising from S1 with extension into the pelvic region in an adult spine.
基金supported by the National Key R&D Program of China(Nos.2024YFC2309803 and 2023YFC2706100)the Capital Clinical Characteristic Diagnosis and Treatment Technology Research and Transformation(No.Z221100007422054)+1 种基金the National Natural Science Foundation of China(Nos.82200203 and 82370189)the Beijing Natural Science Foundation(No.7234367)
文摘Background:Juvenile xanthogranuloma(JXG)is a rare disorder that belongs to the broad group of non-Langerhans cell histiocytosis.It is characterized by one or more nodules with predilection sites on the head,neck,and trunk,and lesions that may be several millimeters in diameter.These are reddish or yellowish benign papules or nodules that usually resolve spontaneously.The involvement of organs other than the skin is termed systemic juvenile xanthogranuloma(SJXG).The eye is the most frequent extracutaneous location of the JXG.Case presentation:We report a case of SJXG in a male child,with onset in the second month of life.He presented with several nodules,approximately5 mm in diameter and tan-orange in color,located on the head,face,and trunk.The nodules enlarged to 10 mm in diameter,and new lesions were found in the right eye,which resulted in spontaneous hyphema and secondary glaucoma without treatment.The pathological findings suggested that the nodule was of histiocytic origin,and immunohistochemical analysis resulted in the diagnosis of JXG.Chemotherapy based on the Langerhans cell histiocytosis(LCH)regimen resulted in a good prognosis.Conclusion:SJXG has low morbidity,but is unpredictable,and rare and self-limited.Treatment is required for patients with extracutaneous involvement,who may have increased morbidity.The LCH-Ⅲprotocol of the International Histiocyte Society is the most commonly used and effective chemotherapy regimen.
文摘Introduction:Histiocytoses are localized or systemic diseases that can broadly be classified into Langerhans cell histiocytosis and non-Langerhans cell histiocytosis.Juvenile Xanthogranuloma(JXG)belongs to the latter group,which occurs in around 0.5%population younger than 5 years,and presents as solitary or multiple lesions.Here,we present a case of disseminated JXG in an infant with the lesions regressing spontaneously.Case presentation:A 7-month-old boy presented with multiple erythematous nodular lesions over the body excluding limbs for 3 months.Initial laboratory work up was normal.Skin histopathology showed a dermal nodule with sheets of histiocytes exhibiting grooved vesicular nuclei and pale eosinophilic cytoplasm along with touton giant cells.Immunohistochemistry markers further confirmed the diagnosis.Lesions healed completely with symptomatic relief in 1.5 years and no recurrence occurred.Discussion:Disseminated JXG is a benign childhood disorder that can sometimes be associated with internal organs involvement mainly bones,eyes,and brain.Serious complications may follow in case of extracutaneous spread.Most frequently,JXG follows the occurrence of another neoplastic disorder but can sometimes appear de novo.Skin biopsy is required for the diagnosis and better education of the family.Conclusion:JXG in infancy is a rarity in South Asia.Therefore,along with the clinical judgment,clinicians must also get histological confirmation in order to manage this disorder successfully.Close surveillance for multiorgan involvement is also required to avoid any irreversible sequalae.
文摘Introduction Xanthogranuloma is a kind of rare,non-Langerhans cell histiocytosis that most commonly occurs in infancy or early childhood.Adult xanthogranuloma was first described by Gartmann and Tritsch in 1963,and there have been only approximately one hundred cases reported until now[1].Diagnosis of this disease might be complicated due to its unusual forms,and a group of dermatoses need to be differentiated.We here described a case of multiple adult xanthogranuloma (MAXG) in a young man.
