Juvenile Xanthogranuloma JXG
Juvenile xanthogranuloma, also known as JXG, is a rare, non-Langerhans-Cell Histiocytosis that is usually benign and self-limiting. It occurs most often in the skin of the head, neck, and trunk but can also occur in the arms, legs, feet, and buttocks. JXG can affect the eye, most commonly in young children with multiple skin lesions. Less commonly JXG may involve locations such as the lung, liver, adrenal gland, appendix, bones, bone marrow, pituitary gland, central nervous system, kidney, heart, small and large intestines, and spleen.

JXG involves the over-production of a kind of histiocyte called a dendritic cell (not a macrophage). These cells then accumulate and lead to various symptoms, depending on location. The cause of this disease is not known.

This disease may have been first reported by Rudolf Virchow in 1871 and again in 1905 by H.G. Adamson. In 1954, it was named juvenile xanthogranuloma to reflect the appearance of the cells under a microscope.

JXG mainly affects infants and small children with an average age of 2 years, although it can also occur in adults of all ages. Usually it presents as a single skin lesion, which varies in size, but children less than 6 months of age are more likely to have multiple lesions. It occurs at birth in about 10% of patients and more males are affected than females. When JXG occurs in adults, it tends to be more complicated and is not known to spontaneously improve. The total number of patients with JXG is not known, but it may be higher than reported since this disease is sometimes misdiagnosed or may spontaneously improve in children.

Skin lesions are self-limited and rarely require treatment in most patients. Those with large abdominal masses, liver, bone marrow, or central nervous system involvement may do well with treatment such as chemotherapy similar to that used for Langerhans Cell Histiocytosis. Because this disease is so rare, no large studies have been performed, and there is no established, proven treatment for the more complicated cases.

Please be advised that all the information you read here is not a replacement for the advice you will get from your consultant and their team.

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Juvenile xanthogranuloma (JXG)
The questions below specifically relate to Juvenile xanthogranuloma (JXG).

1. What causes juvenile xanthogranuloma (JXG)?
JXG involves the over-production of a kind of histiocyte called a dendritic cell. What triggers these cells to accumulate is not known.

2. Is there a cure for JXG?
We usually do not use the term “cure” with this disease, although most patients with only skin or soft tissue JXG have spontaneous remission over time without treatment. Children with liver, bone marrow, CNS involvement and masses in the abdominal cavity usually survive with chemotherapy treatment. There is no established period of inactive disease before JXG is considered cured.

3. What are the different therapies/treatments commonly used to treat JXG?
Patients with a single lesion or just a few lesions, as well as children with skin-only JXG often require no therapy. Surgical removal may be required if the mass is causing organ dysfunction. A small percentage of patients with rapidly growing disease may require treatment with chemotherapy or low-dose radiation, however there is no agreed standard. Steroids have been used to treat eye lesions and in some cases low dose radiation has been effective in preventing visual loss.

4. Can an infant be tested at birth for JXG?
A biopsy of the affected tissue, rather than a blood test, is required for diagnosis and would therefore not be appropriate as a routine test unless this disease is suspected.

Please be advised that all the information you read here is not a replacement for the advice you will get from your consultant and their team.

Help ensure that we can continue to bring you this vital informational material, make a donation today

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pubmed: juvenile xanthogranu...

NCBI: db=pubmed; Term=juvenile xanthogranuloma

Related Articles

A novel fixation technique using anterior C1 screw in a pediatric solitary cervical spinal juvenile xanthogranuloma.

Eur Spine J. 2019 Feb 15;:

Authors: Rajasekhar R, Karthigeyan M, Salunke P, Gupta K

Abstract
PURPOSE: Juvenile xanthogranuloma (JXG) presenting as solitary vertebral body lesion is infrequently seen and usually limited to one or two levels. We report a case of an isolated JXG with extensive cervical spinal (bony and extradural) involvement in a 6-year-old child. There was a diagnostic dilemma as the radiologic and intraoperative picture resembled tuberculosis. The spinal reconstruction was also challenging due to involvement of multiple vertebral levels and necessitated an anterior C1 screw.
METHODS: The lytic lesion was multicompartmental, involving the craniovertebral junction and the subaxial spine (till C6 vertebral body) and extending into the retropharyngeal space. Noticeably, an associated thoracic syringomyelia was also present. Near-total excision of the lesion and 360° spinal fixation was performed using fibular strut graft. The graft was cranially anchored to the C1 anterior arch, thereby sharing the load with the posterior occipito-cervical instrumentation in order to avoid a construct failure due to cantilever effect.
RESULTS: At 12-month follow-up, the patient had good clinico-radiologic outcome with evidence of bony fusion and resolution of syrinx.
CONCLUSION: The report highlights the diagnostic dilemma of JXG lesion on both the radiology and surgery and discusses the challenges in the management and the relevant literature. The described technique can be a viable option in pediatric tumors with extensive C2 vertebral body involvement. Occasionally, extradural compression can have associated syrinx formation and the primary treatment per se could tackle the underlying syringomyelia.

PMID: 30771048 [PubMed - as supplied by publisher]

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