Isolated Intraspinal Juvenile Xanthogranuloma in an Infant Presenting as Acute Paraplegia

Abstract

Juvenile xanthogranuloma is a histiocytic proliferative disease that predominantly affects the skin. Extracutaneous involvement is rare. We present the case of a 6-month-old infant with acute paraplegia. Magnetic resonance imaging showed an intraspinal extradural mass at midthoracic level with marked compression of the spinal cord. Complete tumor removal was achieved by emergency surgery and was followed by complete neurologic recovery. Histologic examination led to the diagnosis of a juvenile xanthogranuloma. To the best of our knowledge, an isolated intraspinal juvenile xanthogranuloma in the first 12 months of life has not been described before. Juvenile xanthogranuloma is a histiocytic proliferative disease which predominantly affects the skin. Extracutaneous involvement is rare. We present a 6 month old infant with an acute paraplegia. MR-imaging showed an intraspinal extradural mass at midthoracic level with marked compression of the myelon. Complete tumor removal was achieved by emergency surgery, followed by complete neurological recovery.Histological examination led to the diagnosis of a juvenile xanthogranuloma. To our best knowledge, an isolated intraspinal juvenile xanthogranuloma in the first 12 months of life has not been described before.

Solitary juvenile xanthogranuloma in the spine pretreated with neoadjuvant denosumab therapy followed by surgical resection in a 5-year-old child: case report and literature review

“…Systemic JXG may involve any number and combination of extracutaneous sites [30]. The most frequent extracutaneous site involved with JXG is the eye, followed by subcutaneous soft tissue, the central nervous system, lung, liver, spleen, and kidneys [30,33]. Systemic JXG may also exhibit skeletal manifestations, but solitary osseous JXG is rare [31].…”

“…In 1993, Shimosawa et al [34] reported a case of intradural extramedullary JXG. Since then, only a few case studies have reported spinal JXGs in the cervical spine [3,8,35,36], the thoracic spine [33,37], or the cauda equina [38], and one study reported JXG with spinal S1 nerve root involvement [39]. Only one study reported an extracutaneous manifestation of JXG in the bony spine, which occurred in a 14-year-old girl with a vertebral lesion in L3 [31].…”

Solitary intraspinal juvenile xanthogranuloma in an infant

“…JXG was first described in 1905 by Adamson as a “congenital xanthoma multiplex.”[ 1 ] Histiocytic disorders are further subtyped as (1) dendritic-cell related, (2) macrophage-related, or (3) malignant histiocytic disorders. JXG is an example of a dendritic cell disorder, another being Langerhans cell histiocytosis (LCH).…”

“…An extensive literature search has revealed only two cases reported in this location in an infant. [ 1 2 ]…”