Juvenile Xanthogranuloma
Pathologie
Simon Haefliger
Juvenile Xanthogranuloma
• Classification
• Clinical aspects and epidemiology
• Dermatologic description
• Histological aspects
First description
1871: “cutaneous xanthomas” in a child
Rudolf Virschow
JXG
Non Langerhans cell histiocytosis: • Rosai-
Dorfman • JXG • Erdheim-
Chester • ….
Med Pediatr Oncolo. 1997 Sep;29(3):157-66. Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic Tumor
Langerhans cell histiocytosis
Classification
CUTANEOUS
Cutaneous JXG
Definition: Benign non Langerhans cell histiocytosis
2005
Classification
CUTANEOUS
DISSEMINATED
«Disseminated» xanthogranuloma
2008
• > more than 1
extracutaneous sites involved
• Can involve almost every organ (central nervous system, liver…)
«Disseminated» xanthogranuloma
«…dermal JXG is vastly more common than others forms and does not progresss to more disseminated forms…»
2008
Epidemiology
19%
Am J Surg Pathol2005 Jan;29(1):21-8. Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the kiel pediatric tumor registry
Classification
CUTANEOUS
DISSEMINATED
ISOLATED FORMS
Isolated forms
Cranial xanthogranuloma
2016
« Juvenile xanthogranuloma arising in the brain either without or with cutaneous lesions »
Classification
CUTANEOUS
DISSEMINATED
ISOLATED FORMS
XGJ
• Classification
• Clinical aspects and epidemiology
• Dermatologic description
• Histological aspects
Epidemiology
Cutaneous
Extra-cutaneous81%
Am J Surg Pathol2005 Jan;29(1):21-8.
Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients
from the kiel pediatric tumor registry.
19%
Incidence ? Prevalence ?
Epidemiology
• Dehner LP. Juvenile xanthogranulomas in the first two decades of life. Am J Surg Pathol 2003; 27: 579-93.
19%
Am J Surg Pathol2005 Jan;29(1):21-8. Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the kiel pediatric tumor registry
Dehner LP. Juvenile xanthogranulomas in the first two decades of life. Am J Surg Pathol 2003; 27: 579-93.
Epidemiology
19%
Am J Surg Pathol2005 Jan;29(1):21-8. Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the kiel pediatric tumor registry
Clinical aspects
CUTANEOUS
DISSEMINATED
ISOLATED FORMS
Clinical aspects
• Classification
• Clinical aspects and epidemiology
• Dermatologic description
• Histological aspects
Dermatologic description
• The papular form is the most frequent
• and is characterized by numerous (up to
• 100), firm hemispheric lesions, 2-5 mm in
• diameter, that are red-brown at first and
• then quickly turn yellowish. These lesions
• are associated in perhaps 20% of
• patients with café-au-lait spots of neurofibromatosis
• {1140} and may be related to
• juvenile chronic myeloid leukaemia
• {538,1650}.
• The nodular form is less frequent, and is
• marked by one or a few lesions. The nodules
• are round to oval, 1-2 cm in diameter,
• high-domed, shiny, translucent, yellowish
• or red brown and sometimes show
• telangectasias on their surface. The term
• giant JXG has been used to indicate
• lesions larger than 2 cm. Unusual clinical
• variants {378,383} are the mixed form
• (simultaneous presence of both papules
• and nodules) and the form en plaque, a
• group of JXG lesions with a tendency to
• coalesce into a plaque as the only
• expression of the disease.
XGJ
• Classification
• Clinical aspects and epidemiology
• Dermatologic description
• Histological aspects
3 Forms
• 3 different forms are described:
Early XGJ
Classic XGJ
Late XGJ
Janssen,D. and Harms,D. (2005d). Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the kiel pediatric tumor registry. Am. J Surg. Pathol
Early XGJ
• Mononuclear histiocytes displayed a compact sheet-like infiltrate
• Some foamy histiocyte
• Some Touton like
giant cell DD: Langerhans cell histiocytosis
Classical XGJ
• Histiocytes with vacuolated cytoplasm
• Touton like giant cell
• Giant cell of Langhans type
Late XGJ
• Predominance of
spindle cells
• Some Touton like giant cell
• ++++ in the extracutaneous localisation
IHC
Cell of origin: dermal dendritic cell
CD 68, XIIIa, CD14 +
Misery L, Boncheron S, Clandy AL. Factor XIIIa expression in juvenile xanthogranuloma. Acta Derm Venereol 1994
S100, CD1a, Langerin -
Touton giant cells
Dermatologist Karl Touton
Touton giant cells
Differential diagnosis
• Langerhans cell histiocytosis
• NO Touton like giant cell
• Eosinophilic infiltrate +++
• Other IHC
• Other clinical presentation
IHC
Dendritic cell Langerhans cell
S100, CD1a, Langerin -
CD 68, XIIIa, CD14 + CD 68, XIIIa, CD14 -
S100, CD1a, Langerin +
Differential diagnosis
• Langerhans cell histiocytosis
• Dermatofibrom
• Xanthoma
Pathogenesis
References
• Dehner LP. Juvenile xanthogranulomas in the first two decades of life. Am J Surg Pathol 2003; 27: 579-93. • Freyer DR, Kennedy R, Bostrom BC, et al. Juvenile xanthogranuloma: form
of systemic disease. J Pediatr 1996; 129: 227-37.
• World Heath Organization. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: International Agency for Research on Cancer; 2008.
• Janssen D, Harms D. Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study. Am J Surg Pathol. 2005;29(1):21-28.
• Meshkini A, Shahzadi S, Zali A, et al. Systemic juvenile xanthogranuloma with multiple central nervous system lesions. J Cancer Res Ther. 2012;8(2):311-313.
LHC
Differential diagnosis