What is a Xanthogranuloma?
What is a Xanthogranuloma?
Xanthogranuloma is a rare benign tumour, part of the non-Langerhans cell histiocytosis group, uncommon in adults and even less common in the paranasal sinuses. Despite its benign nature, it mimics neoplasm due to its local effects which can have serious functional consequences depending on the anatomical location.
What does JXG stand for?
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.
What causes Xanthogranuloma?
JXG is a skin lump caused by an excess of cells known as histiocytes. It is rare and occurs mainly in infants and young children, although about 10% occur in adults. It is not known what causes this condition. It is not a type of cancer.
How is juvenile Xanthogranuloma treated?
Pharmacotherapy: Topical, subconjunctival, intralesional, and systemic corticosteroids are used for intraocular and orbital juvenile xanthogranuloma (JXG). Orbital lesions may respond to intralesional steroid injections. Iris lesions are treated with topical prednisolone or subconjunctival steroids.
What is Xanthogranulomatous inflammation?
Xanthogranulomatous inflammation (XGI) is a rare benign inflammatory disease characterized by aggregation of lipid-laden foamy macrophages. This disease entity has been described in various organs but most commonly in the kidney and gallbladder.
Is Xanthogranuloma malignant?
Juvenile xanthogranuloma (JXG) – UpToDate. Juvenile xanthogranuloma (JXG) is a benign, proliferative disorder of histiocytic cells of the dermal dendrocyte phenotype. JXG belongs to the broad group of non-Langerhans cell histiocytoses and is typically a disorder of early childhood.
What is Necrobiotic Xanthogranuloma?
Necrobiotic xanthogranuloma (NXG) is a form of non-Langerhans histiocytosis characterized by the development of red-brown, violaceous, or yellowish cutaneous papules and nodules that evolve to form infiltrated plaques. The periorbital skin is the most common site for NXG.
Does juvenile Xanthogranuloma go away?
Treatment. JXGs on the skin do not usually cause other problems. These usually go away by themselves over several years without any treatment. After the bumps are gone, your child may have a scar or skin color change where the JXG was.
What is Xanthomatous inflammation?
Xanthomatous inflammation is a rare form of chronic inflammation, manifested by the presence of lipid-laden macrophages admixed with lymphocytes, plasma cells, neutrophils, and often multinucleated giant cells with or without cholesterol clefts [1]. It was initially described in the kidney by Osterlind in 1944 [2].
What is Xanthogranulomatous reaction?
Xanthogranulomatous inflammation (XGI) is a relatively rare chronic inflammatory process that is characterized by prominent histiocytic component with clusters of xanthoma-type cells. 1 XGI can involve any organ, mainly occurring in the kidneys and gallbladder.
Is Xanthogranuloma benign?
Xanthogranuloma is a benign, asymptomatic, and self-healing disorder of non-Langerhans cell histiocytosis, affecting mostly infants, children, and rarely adults. Diagnosis is easy in typical cases but become more complex in unusual forms.
What does Necrobiotic mean?
Necrobiosis is the physiological death of a cell, and can be caused by conditions such as basophilia, erythema, or a tumor. It is identified both with and without necrosis. It is associated with necrobiosis lipoidica and granuloma annulare.
What does xanthogranuloma stand for in medical category?
juvenile xanthogranuloma a benign, self-limited disorder of infants and children, with single or multiple yellow, pink, orange, or reddish brown papules or nodules on the scalp, face, proximal limb, or trunk, sometimes with involvement of mucous membranes, viscera, eye, and other organs. xanthogranuloma. [zan′thəgran′yəlō′mə] pl.
Which is the most aggressive form of xanthogranuloma?
Adult xanthogranuloma may present in isolated forms in the eyelid or anterior orbit (necrobiotic xanthogranuloma, adult-onset xanthogranuloma) or in an aggressive, potentially lethal systemic form (Erdheim-Chester disease). Xanthogranuloma presents with fibrosclerosis of the orbital tissues.
Is there such a thing as a choroid plexus xanthogranuloma?
Choroid plexus xanthogranuloma. Choroid plexus xanthogranulomas are common, incidental and almost invariably asymptomatic lesions. It is unclear in much of the literature whether they represent a distinct entity from adult choroid plexus cysts, but they share imaging characteristics and are only likely to be distinguishable on autopsy.
How is xanthogranuloma treated in erdheim-chester disease?
Xanthogranuloma presents with fibrosclerosis of the orbital tissues. In Erdheim-Chester disease, the mediastinum, pericardium, pleura, bone, and retroperitoneum may be involved, and orbital disease is more diffuse with potential for vision loss. Local lesions are treated with systemic corticosteroids and other immunosuppressants.
