Giant Juvenile Xanthogranuloma: Case Report, Literature Review, and Algorithm for Classification

Abstract

Juvenile xanthogranuloma (JXG) is a member of the non-Langerhans cell group of proliferative disorders of mononuclear phagocytes. JXG is a benign tumour of histiocytic cells. Classic JXG is divided into 2 main clinical subtypes: dome-shaped papules (<0.5 cm) and single/multiple nodules (<2.0 cm). A rare variant is referred to as giant; this term encompasses JXG lesions larger than 2.0 cm. In this article, we report a case of a congenital cutaneous giant JXG. In addition, we reviewed and analyzed all cases (n = 51) of giant JXG reported in the English literature. We propose an algorithm for classifying giant JXG based on the following factors: onset of lesions (congenital and acquired), number of lesions (solitary ± satellites and multiple), morphology of cutaneous/mucosal lesions (plaque, nodular, ulcerated-nodular, macular, and other), and extracutaneous manifestations.

A large congenital cutaneous tumor

“…Immunohistochemistry generally shows an expression of CD68, HAM56, and factor XIIIa markers. However, S100 and Langerin markers are negative, and thus Langerhans cell histiocytosis is excluded [1,2].…”

Ein großer kongenitaler kutaner Tumor

“…Am häufigsten findet man es am Kopf, am Hals oder im oberen Rumpfbereich. Die Dermatoskopie zeigt einen orange-gelben Zentralbereich, der wie bei unserer Patientin, als Zeichen der untergehenden Sonne bezeichnet wird; gelegentlich finden sich an der Peripherie verzweigte lineare Gefäße [1,2].…”

“…Immunhistochemisch findet man üblicherweise die Expression von CD68, HAM56 und Faktor XIIIa. S100-und Langerin sind jedoch negativ, so dass eine Langerhans-Zell-Histiozytose ausgeschlossen ist [1,2].…”

Juvenile xanthogranuloma: retrospective analysis of 44 pediatric cases (single tertiary care center experience)

“…Juvenile xanthogranuloma (JXG), initially termed xanthoma multiplex , juvenile xanthoma, and/or xanthoma tuberosum, is the most common type of non‐Langerhans cell histiocytosis 1 . JXG most often occurs during infancy or soon after.…”

“…On histopathology, these papules and nodules are characterized by the accumulation of the histiocytes with a progressively increasing degree of lipidation within the dermis. The most common locations of these lesions are the head and neck, followed by the upper and lower extremities 1‐3 . Extracutaneous involvement is rare but may occur in the eyes, bones, lungs, and liver 3,4 .…”