juvenile xanthogranuloma -...

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