Juvenile xanthogranuloma: a case report and review of the literature

Abstract

BackgroundJuvenile xanthogranuloma is a rare disorder which may be present at birth, or appears in infancy. It can also occur in adults of all ages; appears with lesions that may be solitary or multiple nodules several millimeters in diameter. The predilection sites are head and neck, but it may occur on the extremities and trunk also. There can also be involved internal organs such as lung, kidney, gastrointestinal tract, etc. The most frequent extracutaneous location is the eye.Case presentationWe report a case of juvenile xanthogranuloma in a male child with onset in the fourth month of life. He presented with a nodule 8 millimeters in diameter, tan-orange in color, ulcerated in the centre, located on the left corner of the left eye. A biopsy without total excision was performed. After the biopsy, the nodule enlarged to 1.5 cm in diameter and became haemorrhagic. The histologic evaluation and immunohistochemistry analysis resulted in the diagnosis of juvenile xanthogranuloma. For aesthetic reasons the nodule was removed by surgical resection.ConclusionJuvenile xanthogranuloma is on a spectrum of histiocytic disorders, which is necessary to differentiate from maligniances in childhood by biopsy.

Multiple Juvenile Xanthogranuloma of the Eyelids

“…In our case, multiple nodules on both eyelids developed after or at the same time as cutaneous lesions when our patient was 10 years old. Within ocular involvements, eyelid involvements were found in 6-25% of patients [15,32]. Most reports of JXG were a solitary lesion in the eyelid [19,20,21,22,28].…”

“…The pathogenesis of JXG remains unclear. The findings of macrophages and giant cells suggest that JXG is an inflammation-related and/or nonspecific injury disorder [32]. A histopathological examination is necessary to make an accurate diagnosis of JXG.…”

Juvenile xanthogranuloma of the eye

“…ЮКГ кожи век и периорбитальной области. Клиническая картина ЮКГ кожи век подобна патологическому процессу на других участках кожного покрова -на поверхности кожи развивается гранулема желтого цвета, хорошо отграничена от окружающих тканей; с увеличением размеров опухоль становится желто-коричневой, приобретает резиноподобную или хрящевидную консистенцию [33]. Подкожные формы образования редки, они склонны к инвазивному росту в орбиту, вызывая симптом экзофтальма [4,34].…”

Xanthogranuloma in the heavily irradiated low neck in a patient with head and neck cancer

“…Xanthogranuloma is a histopathological diagnosis, referring to a lesion comprised of abundant histiocytes, often displaying foamy cytoplasm and associated foreign body giant cells [ 3 ]. Juvenile xanthogranulomatosis (JXG) is a clinical diagnosis first described in the 1950s, referring to children with one or more of these lesions [ 4 ]. JXG is thought to be a benign disorder, although ocular involvement may impact 0.5 % of patients with skin involvement [ 4 ].…”