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

Juvenile xanthogranuloma Newsfeed

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Related Articles

Intradural Juvenile Xanthogranuloma with Involvement of Multiple Nerve Roots. A Case Report and Review of the Literature.

World Neurosurg. 2018 Aug 07;:

Authors: Wolfe C, El Ahmadieh TY, Aoun SG, Vance AZ, Hatanpaa KJ, Wohlfeld B

Abstract
BACKGROUND: Juvenile Xanthogranuloma is a rare, non-Langerhan's cell histiocytic disorder that primarily presents as multiple cutaneous lesions in young males. Solitary lesions in the spinal column are a particularly rare presentation of this disease, and CNS involvement can portend a poor prognosis. This report highlights an unusual case of an adult woman with an unresectable JXG of the lumbar spine. A review of the published cases of thoracolumbar JXG and the current state of the literature in regard to diagnosis and treatment is presented.
CASE DESCRIPTION: A 28-year-old woman presented with back pain and worsening lower extremity pain, numbness and weakness. MRI demonstrated an enhancing lumbar mass, but at the time of surgery, no discrete mass was identified. Multiple roots were grossly enlarged, and electrical stimulation identified the L4 root as the most abnormal. Despite an attempt to debulk, the majority of the mass could not be safely removed. The patient had incomplete improvement of symptoms post-operatively but elected to forgo chemotherapy. Three-month follow-up imaging showed active lumbar spinal disease, and imaging and follow up at 27-months has revealed no changes. Her symptoms have been satisfactorily controlled with conservative therapy.
CONCLUSIONS: Juvenile Xanthogranuloma of the spine is a rare disease who's nonspecific clinical and radiographic findings can make difficult to diagnose and dictates the use of immunohistochemical staining. If possible, total surgical resection offers the greatest outcomes, but other modalities such as chemotherapy may be viable alternatives or adjuvants.

PMID: 30096507 [PubMed - as supplied by publisher]

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