Bilateral multifocal uveal juvenile xanthogranuloma in a young boy with systemic disease

Labalette, P.; Guilbert, F; Jourdel, D.; Nelken, Brigitte; Cuvellier, J.; Maurage, Claude‐Alain

doi:10.1007/s00417-002-0465-z

Cited by 16 publications

(8 citation statements)

References 13 publications

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“…Учитывая тесную связь оболочек, патологический процесс затрагивает несколько структур глаза. Гемофтальм и субретинальные инфильтраты (диффузные или узловые) являются наиболее частыми из описанных симптомов заболевания [28][29][30]31]. Вовлечение ДЗН является исключительно редким, и, как правило, оно односторон-нее и ассоциировано с высоким риском потери зрения.…”

Section: рис 5 юкг радужки при первичном обращении (а) и через 1 меunclassified

“…Манифестация заболевания может начаться со стушеванности границ ДЗН, его побледнения или образования гранулем [28,31,32]. Билатеральная мультифокальная увеальная ЮКГ может быть признаком системного заболевания, например синдром Леттерера-Сиве -болезнь, характеризующаяся образованием очагов разрастания атипических гистиоцитов в коже, костях, внутренних органах [30]. Учитывая редкость заболевания, диагноз нередко удается поставить лишь после проведения витрэктомии и иммуногистохимического исследования [28].…”

Section: рис 5 юкг радужки при первичном обращении (а) и через 1 меunclassified

“…Учитывая редкость заболевания, диагноз нередко удается поставить лишь после проведения витрэктомии и иммуногистохимического исследования [28]. Лечение должно проводиться длительно и подразумевает системное применение ГКС [29,30]. Если внутривенная и пероральная гормональная терапия полностью не останавливает патологический процесс, в качестве альтернативного лечения возможно проведение ДЛТ [5].…”

Section: рис 5 юкг радужки при первичном обращении (а) и через 1 меunclassified

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Juvenile xanthogranuloma of the eye

et al. 2018

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Juvenile xanthogranuloma (JX) is a benign growth of fibrohistiocytoma range that mainly affects children. The most frequent localization of the tumor is skin integument. Non-cutaneous forms of JX are found in 5-10% of cases; they are manifested as deep lesions of soft tissues, involvement of various organs (oropharynx, lungs, liver, spleen, pericardium, gastrointestinal tract, central nervous system, bone marrow) including the organ of vision. JX can develop in any part of the eye and its appendages. Taking into consideration how rare this pathology is, its diagnosis and treatment are complicated. The article reviews 2 clinical cases of JX observed by the authors.

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Section: рис 5 юкг радужки при первичном обращении (а) и через 1 меunclassified

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Juvenile xanthogranuloma of the eye

et al. 2018

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“…Touton giant cells can be identified in some cases. The immunoprofile includes diffuse CD68 positivity and S100 and CD1a negativity . In testicular cases, surgical resection is curative.…”

Section: Miscellaneous Tumours Of the Testismentioning

confidence: 99%

The World Health Organization 2016 classification of testicular non‐germ cell tumours: a review and update from the International Society of Urological Pathology Testis Consultation Panel

et al. 2016

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The World Health Organization (WHO) released a new tumour classification for the genitourinary system in early 2016 after consensus by pathologists with expertise in these organs. It utilized the framework of the 2004 classification, and incorporated the most up-to-date information concerning these tumours. In testicular tumours, the majority of the changes occurred in the nomenclature and classification of germ cell tumours; however, several modifications were also made for non-germ cell tumours. Among sex cord-stromal tumours, sclerosing Sertoli cell tumour (SCT) is no longer recognized as a separate entity but as a morphological variant of SCT not otherwise specified (NOS), as CTNNB1 gene mutations have been noted in both neoplasms but not in the other forms of SCT. Similarly, the lipid cell variant is not separately classified, but is considered to be a morphological variant of SCT NOS. Large-cell calcifying SCT is recognized as a distinct entity that occurs either sporadically or in association with Carney complex, with the latter patients having a distinct germline PRKAR1A gene mutation. Intratubular large-cell hyalinizing Sertoli cell neoplasia is also accepted as a separate entity linked with Peutz-Jeghers syndrome. The subcategories of 'mixed' and 'incompletely differentiated' forms of sex cord/gonadal stromal tumours have been replaced by 'mixed and unclassified sex cord-stromal tumours'. New entities introduced in the latest WHO revision include: myoid gonadal stromal tumour and 'undifferentiated gonadal tissue', a putative precursor lesion of gonadoblastoma, whereas juvenile xanthogranuloma and haemangioma are included in the miscellaneous category of tumours.

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“…Exceptionally, cardiac, retroperitoneal, gonadal, adrenal gland, and skeletal examples have been reported 3 . While vastly outnumbered by cutaneous examples, occasional JXGs have been reported involving all levels of the neuroaxis, including the central and peripheral nervous system (PNS) and their enveloping meninges 2,4–37 . Clinically, JXGs are infrequently included in the differential diagnosis of lesions of the nervous system, particularly in the absence of pathologically confirmed cutaneous lesions.…”

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confidence: 99%

Juvenile xanthogranulomas of the nervous system: A report of two cases and review of the literature

Deisch

Patel²,

Koral³

et al. 2012

Neuropathology

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Juvenile xanthogranulomas (JXG) are uncommon non-Langerhans cell histiocytic proliferations which arise most often in children. While most cases present as solitary cutaneous lesions, occasional cases involve extracutaneous sites. Rare examples of JXGs have been reported involving all levels of the neuroaxis. We present two cases of JXGs involving the nervous system, and review the literature. The first patient was a 14-year-old female with headaches and a mass involving the left trigeminal nerve; pathologic examination showed a JXG. At 11 months follow-up, after administration of systemic chemotherapy, the patient remained stable with residual tumor. The second patient was a 15-year-old female with leg weakness and numbness, who underwent complete surgical resection of a dural JXG. At eight months follow-up, she showed no evidence of tumor, and was able to walk without difficulty. Review of the literature revealed 38 previously published reports of JXGs involving the nervous system. The CNS was involved in the majority (75%) of cases. The clinical characteristics of JXGs arising in the CNS varied significantly from cases in the peripheral nervous system (PNS); CNS tumors occurred in younger patients, more often males, and were more likely to be associated with concurrent cutaneous and extra-nervous systemic lesions. The clinical outcomes were similar for CNS and PNS lesions, with the caveat that all three lethal JXGs occurred in the CNS. The clinical and radiologic presentation of JXGs is nonspecific, thus necessitating biopsy and pathologic examination to arrive at the diagnosis. The pathologic differential diagnosis includes a heterogeneous group of histiocytic proliferations; immunostaining for histiocytic markers CD68, factor XIIIa, and Fascin, and the absence of Birbeck granules and CD1a immunoexpression suggests the diagnosis of JXG. In many cases, total surgical resection is curative. However, some cases will require additional chemotherapy and/or radiotherapy.

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Bilateral multifocal uveal juvenile xanthogranuloma in a young boy with systemic disease

Abstract: In this atypical systemic variant of juvenile xanthogranuloma with bilateral uveal involvement, the immunohistochemical and ultrastructural findings were crucial in distinguishing juvenile xanthogranuloma from Langerhans cell histiocytosis.

Cited by 16 publications

References 13 publications

Juvenile xanthogranuloma of the eye

Juvenile xanthogranuloma of the eye

The World Health Organization 2016 classification of testicular non‐germ cell tumours: a review and update from the International Society of Urological Pathology Testis Consultation Panel

Juvenile xanthogranulomas of the nervous system: A report of two cases and review of the literature

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