Is juvenile Xanthogranuloma cancerous?

Is juvenile Xanthogranuloma cancerous?

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 the meaning of Xanthogranuloma?

Xanthogranulomas are nodules in the skin of neonates, infants, and adults that are characterized histologically by large foamy macrophages (xanthoma cells).

What causes juvenile xanthogranuloma?

A juvenile xanthogranuloma (zan tho grahn you LOH mah), or JXG, is a benign skin lump or bump caused by a collection of cells called histiocytes. These may be red, orange or tan at first, but over time may become more yellow in color. These bumps usually appear on the head, neck and trunk.

What causes Xanthogranuloma?

The cause of JXG is not clearly understood but it is believed to result from an overproduction of a type of histiocyte cell used in the body’s immune system in response to nonspecific tissue injury. Histiocytes work in the immune system to fight bacteria and dispose of tissue waste products.

Does JXG 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.

How is 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.08-Feb-2019

How long does it take for JXG to go away?

JXG can sometimes be seen at birth. Other times the bumps can form after birth for up to 1 to 2 years. The bumps normally go away on their own in children over 3 to 6 years.

Is Xanthogranuloma a cancer?

Abstract. Juvenile xanthogranuloma (JXG) is generally a benign, self-limited histiocytic disorder of the skin. We report two cases of multisystem JXG presenting with clinical features more commonly seen in Langerhans cell histiocytosis (LCH), including diabetes insipidus and lytic bony lesions.Abstract. Juvenile xanthogranuloma (JXG) is generally a benign, self-limited histiocytic disorder of the skin. We report two cases of multisystem JXG presenting with clinical features more commonly seen in Langerhans cell histiocytosisLangerhans cell histiocytosisLangerhans cell histiocytosis (LCH) is a disorder that primarily affects children, but is also found in adults of all ages. People with LCH produce too many Langerhans cells or histiocytes, a form of white blood cell found in healthy people that is supposed to protect the body from infection.https://rarediseases.info.nih.gov › langerhans-cell-histiocytosisLangerhans cell histiocytosis – Genetic and Rare Diseases Information (LCH), including diabetes insipidus and lytic bony lesions.

Is Xanthogranuloma benign?

Xanthogranulomas are benign, usually asymptomatic, self-healing, red, yellow, or brown papules, nodules composed of histiocytic cells that predominantly occur in infancy and childhood.

Treatment of necrobiotic xanthogranuloma – a systematic review

Necrobiotic xanthogranuloma (NXG) is an uncommon non-Langerhans cell histiocytosis involving skin and extracutaneous tissues. The lesions are usually asymptomatic and commonly appear in the periorbital area. Paraproteinemia is closely associated with NXG and its pathogenesis remains unclear. NXG pro …

Treatment of juvenile xanthogranuloma – PubMed

Juvenile xanthogranuloma (JXG) is generally a benign, self-limited histiocytic disorder of the skin. We report two cases of multisystem JXG presenting with clinical features more commonly seen in Langerhans cell histiocytosis (LCH), including diabetes insipidus and lytic bony lesions. Histologically …

Xanthogranuloma – an overview | ScienceDirect Topics

Eduardo Calonje MD, DipRCPath, in McKee’s Pathology of the Skin, 2020. Xanthoma disseminatum and scalloped cell xanthogranuloma. Xanthoma disseminatum (XD) usually develops in young adults (<25 years), with rare cases in children and in the elderly. 61-68 There is a male predilection. 61,62 Early lesions are red-yellow papules, nodules, and plaques, with predilection for the flexures (Fig

Xanthogranuloma — Ozark Dermatology

Treatment of juvenile xanthogranuloma is typically not required, as lesions are harmless unless they affect the eyes. This make removal generally unnecessary, and lesions will typically resolve over a period of 2-3 years without scarring. If required, surgical removal is possible, but will result in scarring of the affected area.

Juvenile Xanthogranuloma – StatPearls – NCBI Bookshelf

Juvenile xanthogranuloma (JXG) is a relatively common entity and is the most common form of non-Langerhans cell histiocytic disorder of childhood.[1], [2] It is estimated that in 75% of cases, lesions appear during the first year of life, with >15-20% of patients having lesions at birth. JXG is rare in adults, with a peak incidence in the late twenties to thirties.

Xanthogranuloma – Histiocytosis Association

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.

What Is Xanthogranuloma? Causes, Symptoms and Treatment

Treatment of xanthogranuloma depends on the severity of the condition and the organ involved. The skin lesions are generally self healing and they just need to be observed from time to time. Surgery may be indicated for many reasons such as: for biopsy purpose, or when the function of the affected organ is hampered or due to cosmetic reasons

Pyelonephritis Xanthogranulomatous – StatPearls – NCBI

Xanthogranulomatous pyelonephritis (XGP) is a rare and aggressive variant of chronic pyelonephritis resulting in a non-functioning kidney. It is most often associated with chronic obstruction and stones with ongoing infection.[1] It is also referred to as a pseudotumor due to an enlarged kidney resembling a tumor and the ability of local invasion and destruction.

Juvenile xanthogranuloma – DermNet NZ

Juvenile xanthogranuloma is rarely a clinical diagnosis, although dermoscopy is characteristic. Confirmation may require biopsy [see Juvenile xanthogranuloma pathology ]. Patients with more than two skin lesions, early onset lesions, and/or a unilateral red eye or ocular symptoms should be referred for an eye examination.

Xanthogranuloma in Adults | JAMA Dermatology | JAMA Network

Abstract. † Nine cases of cutaneous xanthogranuloma in adults are reported. These lesions were histologically indistinguishable from the xanthogranulomas in infants and children (juvenile xanthogranuloma), and, like those in the young, they were not associated with abnormalities of serum lipids. Unlike juvenile xanthogranuloma, however, in

The Rare Case of an Adult-Onset Xanthogranuloma of the

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. We present the rare case of a young lady who presented insidiously with a

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Juvenile Xanthogranuloma Treatment & Management: Approach

Juvenile xanthogranuloma (JXG) primarily is a self-limited dermatologic disorder that is associated rarely with systemic manifestations. Infants and small children are mainly affected.

Necrobiotic xanthogranuloma | Genetic and Rare Diseases

Necrobiotic xanthogranuloma (NXG) is a rare, chronic form of non-Langerhans histiocytosis usually found in older adults. Xanthogranulomas are lesions made of immune cells known as a histiocytes. The term necrobiotic refers to the buildup of broken down collagen fibers that can be seen under a microscope. The typical lesion is a yellow, thickened, or raised lesion (plaque) located around the eyes.

Xanthogranuloma – Eyelid Diseases – Mobile Health Knowledge

TREATMENT While xanthogranulomas are benign they can be associated with significant morbidity and mortality when there is systemic involvement. Therefore all patients should have systemic evaluation to rule out Erdheim-Chester disease and necrobiotic xanthogranuloma. Surgical excision of the lesion will provide cosmetic improvement.

Juvenile Xanthogranuloma – EyeWiki

Juvenile xanthogranuloma (JXG) is a rare benign histiocytic proliferation that develops in infants and young children. JXG is the most common form of non-Langerhans’ cell histiocytoses. It is characterized by the presence of Touton giant cells.

Xanthogranuloma symptoms, treatments & forums | PatientsLikeMe

Xanthogranuloma is characterized as a skin condition, it appears as a firm raised papule/nodule. It may appear as a single lesion or multiple lesions, with pink to brown discoloration. Common symptoms reported by people with xanthogranuloma. Common symptoms. How bad it is. What people are taking for it.

(PDF) Treatment of Juvenile xanthogranuloma

Objective: To investigate the clinical characteristics, treatment, and prognosis of children with systemic juvenile xanthogranuloma (JXG). Methods: Clinical data of children with JXG who were

Juvenile Xanthogranuloma (JXG) – Nationwide Children's

A juvenile xanthogranuloma (zan tho grahn you LOH mah), or JXG, is a benign skin lump or bump caused by a collection of cells called histiocytes. These may be red, orange or tan at first, but over time may become more yellow in color. These bumps usually appear on the head, neck and trunk.

Necrobiotic Xanthogranuloma Images, Symptoms, Causes

The treatment strategies of the Necrobiotic Xanthogranuloma are based on whether Necrobiotic Xanthogranuloma is being treated alone or with multiple myeloma or systemic involvement. In the case of Necrobiotic Xanthogranuloma with myeloma is often treated with Alkylating agents such as Melphalan, Chlorambucil and Cyclophosphamide.

Necrobiotic Xanthogranuloma – EyeWiki

Necrobiotic Xanthogranuloma is a non-Langerhans cell histiocytosis with multisystem involvement that commonly affects the skin and periorbital structures. The diagnostic criteria and best treatment course are both evolving, but the strong association with hematologic disorders warrants close hematology surveillance when the disease is recognized.

Choroid plexus xanthogranuloma | Radiology Reference

Choroid plexus xanthogranulomata 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.As the clinical history is the same, the distinction, at least from a radiologist’s and

Dermatologic Manifestations of Juvenile Xanthogranuloma

Juvenile xanthogranulomas (JXGs) are benign; usually asymptomatic; self-healing; red, yellow, or brown papules and nodules composed of histiocytic cells that predominantly occur in infancy and childhood. Papules or nodules occur in the skin, eyes, and viscera.

Periorbital necrobiotic xanthogranuloma successfully

Background: Necrobiotic xanthogranuloma (NXG) is a rare non-Langerhans histiocytosis with cutaneous manifestations, most commonly of the periorbital skin, and is often associated with hematologic disorders such as monoclonal gammopathy. Treatment of NXG is notoriously difficult, and fraught with recurrence and progression.

Efficacy of Topical Use Crisaborole 2% Ointment for

Necrobiotic xanthogranuloma (NXG) was first defined by Kossard et al in 1980 and is characterized as a rare chronic disease presenting as firm yellow to reddish-brown nodules and plaques, frequently involving the periorbital area.1 Microscopically, NXG is distinguished by an infiltrate composed of macrophages, foam cells, and other inflammatory cells.2 The extensive necrobiotic regions of

UpToDate

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. Extracutaneous involvement of ocular, respiratory, cardiac, or other

Juvenile Xanthogranuloma: Case Report and Literature

Introduction. Juvenile Xanthogranuloma (JXG) is a relatively uncommon non-Langerhans cell histiocytosis disorder. 1 The lesions usually present as asymptomatic yellow-brown papules and nodules that are mostly self-limited. JXG can present anywhere in the body but primarily occurs as a solitary lesion on the head, the neck, or upper trunk. 1 Biopsy of the lesions can reveal the definitive

Xanthogranuloma | definition of xanthogranuloma by Medical

xanthogranuloma: [ zan″tho-gran″u-lo´mah ] a tumor having histologic characteristics of both granuloma and xanthoma . juvenile xanthogranuloma a benign, self-limited disorder of infants and children, usually present at birth, manifested by the development of single or multiple papules or nodules, which may be yellow, pink, orange, or reddish

Treatment of necrobiotic xanthogranuloma – a systematic

The aim of this systematic review is to summarize all reported treatments of necrobiotic xanthogranuloma of the skin, with or without underlying malignant condition and based on articles from the PubMed database using the query ‘necrobiotic xanthogranuloma treatment’, both in English and German, about ‘human’ subjects and published

Treatment of necrobiotic xanthogranuloma – a systematic

The aim of this systematic review is to summarize all reported treatments of necrobiotic xanthogranuloma of the skin, with or without underlying malignant condition and based on articles from the PubMed database using the query ‘necrobiotic xanthogranuloma treatment’, both in English and German, about ‘human’ subjects and published between 1980

Necrobiotic Xanthogranuloma – Dermatology Advisor

“Generalized necrobiotic xanthogranuloma successfully treated with lenalidomide”. J Drugs Dermatol. vol. 9. 2010. pp. 273 (This brief report discusses the limited treatment options for NXG and provides an example of successful treatment with resolution of paraproteinemia and NXG.) Spicknall, KE, Mehregan, DA. “Necrobiotic xanthogranuloma”.

Multiple intracranial juvenile xanthogranulomas. Case report

The mass in the left middle fossa was resected and diagnosed as juvenile xanthogranuloma (JXG). Thirty months later, the patient again became symptomatic, exhibiting behavioral abnormalities and a decrease in mental powers. Although JXGs are biologically benign lesions, the treatment of patients with multifocal and/or progressive

Multiple adult xanthogranuloma: case report of successful

Multiple adult xanthogranuloma is a rare and late variant of Juvenile xanthogranuloma, a non-Langerhans cell histiocytosis. It usually corresponds to a single lesion in adults, and the manifestation of multiples lesions is uncommon. We report a case of multiple adult xanthogranuloma, with Isotretinoin therapy failure and optimal response to CO2 Laser treatment in the surgical mode.

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Successful treatment of juvenile xanthogranuloma using

Juvenile xanthogranuloma (JXG) is an uncommon non-Langerhans cell histiocytic disorder that occurs predominantly in infants. Traditional treatment of ocular JXG involves the administration of topical or local corticosteroids.

Periocular Necrobiotic Xanthogranuloma Successfully

Finelli LG, Ratz JL. Plasmapheresis, a treatment modality for necrobiotic xanthogranuloma. J Am Acad Dermatol 1987; 17: 351-354. View article Google Scholar; Hallermann C, Tittelbach J, Norgauer J, Ziemer M. Successful treatment of necrobiotic xanthogranuloma with intravenous immunoglobulin. Arch Dermatol 2010; 146: 957-960.

Necrobiotic Xanthogranuloma – American Academy of

Necrobiotic xanthogranuloma (NXG) is 1 of 4 adult orbital xanthogranulomatous diseases, along with adult-onset asthma with periocular xanthogranuloma, Erdheim-Chester disease, and adult-onset xan­thogranuloma. This group of diseases is characterized histologically by infiltra­tion of foamy histiocytes, Touton-type giant cells, lymphocytes

A case report of recurrent adult-onset xanthogranuloma: is

Adult-onset xanthogranuloma (AOX) is one of the four uncommon syndromes called adult xanthogranulomatous disease (AXD), which is diagnosed by characteristic histopathology. AXD is rare and heterogeneous group of entities that can affect multiple organ systems. Orbital involvement is included in the xanthogranulomatous disease although less prevalent. This work focuses on the use of external

Xanthogranuloma and Anterior interosseous syndrome – eHealthMe

We study 158 people who have Xanthogranuloma or Anterior interosseous syndrome. No report of Anterior interosseous syndrome is found for people with Xanthogranuloma. The study is created by eHealthMe and uses data from the Food and Drug Administration (FDA). It is updated regularly.

PDF Early treatment xanthogranuloma iris with subconjunctival

Juvenile xanthogranuloma has a tendency to affect the iris, but other ocular manifestations such as eyelid,5 and epibulbar lesions, corneal involvement,67 and orbital involvement have Recently, successful treatment with one supplemental subconjunctival injection of steroids has been reported in a case with resis-

Juvenile xanthogranuloma: a case report and review of the

Juvenile xanthogranuloma (JXG) is a rare disorder, which belongs to the broad group of non – Langerhans cell histiocytosis [].JXG may be present at birth, but it mostly arises during the first year of life [].It is characterized by one or more nodules with the predilection sites on the head and neck, although the appearance on the trunk, extremities and extracutaneous locations has been

Treatment of necrobiotic xanthogranuloma – a systematic

Treatment of necrobiotic xanthogranuloma – a systematic review. J Eur Acad Dermatol Venereol. 2017; 31(2):221-235 (ISSN: 1468-3083) Miguel D; Lukacs J; Illing T; Elsner P. Necrobiotic xanthogranuloma (NXG) is an uncommon non-Langerhans cell histiocytosis involving skin and extracutaneous tissues.

Juvenile Xanthogranuloma – DoveMed

Juvenile Xanthogranuloma is an uncommon condition that is mostly observed in infants and young children. Three-fourths of the cases (75%) are seen within the first 12 months, while 15-30% arise congenitally (present at birth) Around 1 in 10 cases are described in adults. Both males and females may be affected.

Multiple generalized xanthogranuloma in adult: Case report

Commonly, no treatment is necessary for cutaneous xanthogranuloma, but severe cutaneous involvement of adult form like our case may require some treatment to hasten disease improvement. The growth of a number of tumor cell lines seems to be inhibited by retinoids, but response may be variable.

Multiple generalized xanthogranuloma in adult: case report

Commonly, no treatment is necessary for cutaneous xanthogranuloma, but severe cutaneous involvement of adult form like our case may require some treatment to hasten disease improvement. The growth of a number of tumor cell lines seems to be inhibited by retinoids, but response may be variable.

Azathioprine and prednisone combination treatment for

xanthogranuloma in two tertiary referral orbital centres from 1984 to 2008. Patient records were reviewed and data collected on orbital localization, immune dysfunction, applied treatment and outcome.

Necrobiotic Xanthogranuloma – Eyelid Diseases

INTRODUCTION Necrobiotic xanthogranuloma is associated with paraproteimia and with histocytic proliferative disorders. It may be associated with the presence or subsequent development of multiple myeloma or leukemia. Laboratory findings show dysproteinemia due to an IgG paraprotein, leukopenia, greatly elevated ESR, hyperlipidemia, cryoglobulinemia, and depressed complement levels.

Frontiers | Disseminated Juvenile Xanthogranuloma and

The association between acute lymphoblastic leukemia (ALL), non-Langerhans cell histiocytosis (non-LCH), and hemophagocytic lymphohistiocytosis (HLH), to the best of our knowledge, has not been published to date. Juvenile xanthogranuloma (JXG), as a type of non-LCH, is usually a benign disease limited to the skin. Systemic involvement is rarely reported. The present case report describes a 15

PDF Juvenile Xanthogranuloma Disease

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; it can also occur in the arms, legs, feet, and buttocks.

Periorbital Necrobiotic Xanthogranuloma Treated

Ghani and associates 3 reported the successful use of the immunomodulatory drugs thalidomide and lenalidomide (Revlimid, Celgene), in the treatment of a patient with periorbital necrobiotic xanthogranuloma (NXG) who was noted to have IgG κ monoclonal gammopathy. NXG is a rare and progressive granulomatous disorder that is most common in the

Pathology Outlines – Juvenile xanthogranuloma

Juvenile xanthogranuloma (JXG) is a rare, benign proliferative non-Langerhans cell histiocytic proliferation. Occurs predominantly in young children. Clinically, it presents as a solitary red-brown, yellowish papule or nodule, most often on the head and neck area. Systemic JXG is rare and may involve any organ system.

PDF Local Corticosteroid Treatment of Eyelid and Orbital

LOCAL CORTICOSTEROID TREATMENT OF EYELID AND ORBITAL XANTHOGRANULOMA BY Victor M. Elner MD PhD,* Roni Mintz MD, Hakan Demirci MD, AND Adam S. Hassan MD ABSTRACT Purpose: To demonstrate the efficacy of local corticosteroid therapy for the treatment of eyelid and orbital xanthogranuloma in adults. Methods: We performed a retrospective chart review of six patients receiving local triamcinolone

Management of orbital xanthogranuloma with methotrexate

Background/aims: To evaluate the efficacy of methotrexate (MTX) treatment for the periorbital findings in adult-onset xanthogranuloma (AOX). Methods: The medical records of three patients with AOX, with and without asthma, who were treated with MTX at Oregon Health & Science University, Portland, Oregon, USA were examined. Diagnosis of AOX was made by biopsy in all patients.

Clinical Features and Treatment Outcomes of Patients With

the clinical features of 35 patients with necrobiotic xanthogranuloma and monoclonal gammopathy and their subsequent disease course and response to treatment. Introduction: Necrobiotic xanthogranuloma (NXG) is a rare chronic granulomatous disorder of the skin associated with a monoclonal gammopathy.

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Necrobiotic Xanthogranuloma (NXG) Associated with

BACKGROUND: Necrobiotic Xanthogranuloma (NXG) is a rare chronic granulomatous disorder of the skin associated with monoclonal gammopathies (MG). Due to its rarity, data on response to various therapies are largely limited to single case reports or small case series. Hence, we described the clinical features in patients with NXG and MG as well as their subsequent disease course and response to

Necrobiotic xanthogranuloma pathology | DermNet NZ

Necrobiotic xanthogranuloma is a rare multisystem histiocytic disease usually affecting older adults. The typical clinical lesion is an asymptomatic yellowish indurated plaque in a periorbital location. Approximately 80% of cases are associated with paraproteinaemia.Additional extracutaneous features include ophthalmic involvement, cryoglobulinaemia and lymphoproliferative disorders.

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. JXG typically presents in the first two years of life as a solitary, reddish or yellowish skin papule or

Treatment of necrobiotic xanthogranuloma – a systematic

Necrobiotic xanthogranuloma (NXG) is an uncommon non‐Langerhans cell histiocytosis involving skin and extracutaneous tissues. The lesions are usually asymptomatic and commonly appear in the periorbital area. Paraproteinemia is closely associated with NXG and its pathogenesis remains unclear. NXG prognosis is poor with several treatments showing variable results. Treatment of monoclonal

Xanthogranuloma in the heavily irradiated low neck in a

The xanthogranuloma arose in a location where the skin had ruptured over a large nodal mass during the course of treatment. This represents a late radiation toxicity that has not yet been reported in the English literature in a patient with head and neck cancer.

Successful treatment of necrobiotic xanthogranuloma with

Background: Necrobiotic xanthogranuloma (NXG) is a rare chronic disorder characterized by firm yellow to red-orange nodules and plaques affecting the face, abdomen, and extremities with the potential for systemic involvement.

Necrobiotic Xanthogranuloma – Hematology & Oncology

Necrobiotic xanthogranuloma (NXG) is a rare systemic disease primarily associated with cutaneous plaques; these plaques can manifest anywhere, but most commonly affect the face and periorbital regions. It has a distinct histopathology with extensive necrobiosis and infiltrates of inflammatory cells.

Choroid plexus xanthogranuloma | Radiology Case

Case Discussion. Xanthogranulomas of the choroid plexus are common benign lesions which only rarely cause symptoms. These consist of desquamated epithelium into the lumen of choroid cysts within the choroid plexus. Therefore the MRI signal characteristics depend on the mixture of lipid, fluid and blood products .They appear like cystic lesions.

Treatment of juvenile xanthogranuloma, Pediatric Blood

Treatment of juvenile xanthogranuloma Treatment of juvenile xanthogranuloma Stover, Daniel G.; Alapati, Srilatha; Regueira, Osvaldo; Turner, Curtis; Whitlock, James A. 2008-07-01 00:00:00 INTRODUCTION Disseminated histiocytoses are a rare, heterogeneous group of disorders characterized by tissue infiltration by cells of the monocyte/macrophage or dendritic cell lineages.

British Association of Dermatologists – Patient

Juvenile 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.

A 7-Year History of Necrobiotic Xanthogranuloma following

Necrobiotic xanthogranuloma (NXG) is a rare destructive xanthomatous granuloma with chronic, indolent, and progressive course. The morbidity and mortality are the results from wound complications and associated disorders. Because of its strong association with monoclonal gammopathy and multiple myeloma, early recognition of disease is mandatory to monitor and prevent systemic involvements of

Uncommon histiocytic disorders: Rosai-Dorfman, juvenile

Treatment is restricted to patients with life-threatening, multiple-relapsing, or autoimmune-associated disease. JXG is a pediatric histiocytosis characterized by xanthomatous skin lesions that usually resolve spontaneously. In a minority of cases, systemic disease can occur and can be life threatening. Treatment of juvenile xanthogranuloma

Juvenile Xanthogranuloma: Symptoms, Diagnosis and

We report a rare case of juvenile xanthogranuloma in a 16-year-old girl who presented with a neck mass and left-sided ptosis 2.5 months previously. [ncbi.nlm.nih.gov] Awareness of these features should prompt radiologists to include juvenile xanthogranuloma in the differential diagnosis of pediatric head and neck masses.. Juvenile xanthogranuloma presenting as a large neck mass and ocular

Periorbital and Ocular Necrobiotic Xanthogranuloma Leading

The pathogenesis remains unknown. The differential diagnosis for NXG includes necrobiosis lipoidica, xanthogranuloma, Erdheim-Chester syndrome, erythema induratum of Bazin, foreign body granuloma, and sarcoidosis. 3 The disease and its treatment were thoroughly reviewed by Spicknall and Mehregan. 4

BRAF V600E mutation in Juvenile Xanthogranuloma family

The family of juvenile xanthogranuloma family neoplasms (JXG) with ERK-pathway mutations are now classified within the “L” (Langerhans) group, which includes Langerhans cell histiocytosis (LCH) and Erdheim Chester disease (ECD). Although the BRAF V600E mutation constitutes the majority of molecular alterations in ECD and LCH, only three reported JXG neoplasms, all in male pediatric

Necrobiotic xanthogranuloma: a 30-year single-center

Treatment decisions should be based on coexisting conditions and pattern of disease involvement. AB – To characterize the clinical features, associated disorders, and treatment of necrobiotic xanthogranuloma (NXG), a rare non-Langerhans cell histiocytosis, we conducted a retrospective review of pathologically confirmed NXG at Mayo Clinic

Picture of Juvenile Xanthogranuloma – WebMD

Juvenile xanthogranuloma. This is a common and completely benign cutaneous nodule. Typically, a juvenile xanthogranuloma is firm and dome-shaped. At first, the lesion is reddish, but develops a

Xanthogranuloma oftheChoroid Plexus

Xanthogranuloma oftheChoroid Plexus BertLincoln Pear1 Uncommon tumefactions knownasxanthogranulomas can occurthroughout thebody.Athistologic examination, they consist oflipid-laden cells,generally histiocytes, associated with amixed population ofgiantcells,fibroblasts, chronic inflammatory cells,andoftenhemosiderin. Manyarepostin-

Xanthogranuloma of the choroid plexus (Conference) | OSTI.GOV

@article{osti_6867131, title = {Xanthogranuloma of the choroid plexus}, author = {Pear, B L}, abstractNote = {Uncommon tumefactions known as xanthogranulomas can occur throughout the body. Some appear to develop as benign or malignant neoplasms. An even less familiar xanthogranuloma occurs within the choroid plexus.

Intralesional glucocorticoid treatment of an isolated

Juvenile xanthogranuloma is a type of non-Langerhans cell histiocytic process that appears primarily in children and is described as a benign lesion. Although they typically present as a cutaneous lesion, it can also present in other areas including within the central nervous system. We report a 6-month-old infant who presented with seizure-like activity who was found to have a single

Case for diagnosis. Adult-type xanthogranuloma.

S100 immunohistochemistry was negative for CD1a and positive for CD4 and CD68. Based on clinical and histopathological findings associated with immunohistochemistry, we concluded that it was a case of adult-type xanthogranuloma. Because it was a solitary lesion without other clinical signs and symptoms, the medical conduct adopted was patient

Juvenile xanthogranuloma – Wikipedia

Juvenile xanthogranuloma is a form of histiocytosis, classified as “non-Langerhans cell histiocytosis”, or more s