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Childs Nerv Syst 2019 May 28;35(5):871-874. Epub 2019 Feb 28.

Department of Radiology, West China Hospital of Sichuan University, #37 GuoXue Xiang, Chengdu, 610041, Sichuan, China.

Juvenile xanthogranuloma (JXG) is a benign, self-limiting histiocytic disorder of infancy and early childhood, usually presented as a single or multiple cutaneous lesions. The central nervous system is rarely affected by JXG. There were only a few reports of intracranial JXG cases which described its features on MR spectroscopy (MRS) and diffusion-weighted imaging (DWI), but its features on susceptibility-weighted imaging (SWI) and perfusion-weighted imaging (PWI) have not been reported yet. Read More

Eur J Ophthalmol 2019 Feb 27:1120672119832187. Epub 2019 Feb 27.

1 Cornea, Cataract and Refractive Surgery Services, Division of Cornea, Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Purpose:: To describe a clinical case of corneoscleral xanthogranuloma, a rare manifestation of juvenile xanthogranuloma, and xanthoma disseminatum, which responded well to chemotherapy.

Methods:: Interventional case report and literature search.

Results:: A 9-year-old female patient with a disseminated disease showed complete regression of her corneoscleral xanthogranuloma with methotrexate and azathioprine therapy. Read More

Rev Paul Pediatr 2019 Feb 25. Epub 2019 Feb 25.

Pedro Hispano Hospital, Local Health Unit of Matosinhos, Matosinhos, Portugal.

Objective: To report a rate case of Juvenile xanthogranuloma in a newborn infant.

Case Description: We present the case of a 31-week preterm newborn with multiple skin lesions whose clinical, histological and immunohistochemical findings allowed the diagnosis of juvenile xanthogranuloma. Currently, the patient has nine months-old, and there is no aggravation of the skin lesions or evidence of extra-cutaneous involvement, particularly ophthalmic. Read More

Eur Spine J 2019 Feb 15. Epub 2019 Feb 15.

Department of Histopathology, PGIMER, Chandigarh, India.

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. Read More

Indian Pediatr 2018 Nov;55(11):1013

Department of Pediatrics, Postgraduate Institute of Medical Sciences, Rohtak, Haryana, India.

Mod Pathol 2018 Dec 20. Epub 2018 Dec 20.

Department of Anatomical Pathology, Royal Children's Hospital, Melbourne, VIC, Australia.

In 2008, we presented three cases of ALK-positive histiocytosis as a novel systemic histiocytic proliferation of early infancy with hepatosplenomegaly and dramatic hematological disturbances. This series of 10 cases (including the original three cases) describes an expanded clinicopathological spectrum and the molecular findings of this histiocytic proliferation. Six patients had disseminated disease: five presented in early infancy with eventual disease resolution, and the sixth presented at 2 years of age and died of intestinal, bone marrow, and brain involvement. Read More

J Dermatol 2019 Feb 7;46(2):161-165. Epub 2018 Dec 7.

Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan.

Histiocytoses, including Langerhans cell histiocytosis (LCH), juvenile or adult xanthogranuloma (AXG) and Rosai-Dorfman disease (RDD), are rare disorders characterized by the proliferation of cells derived from monocyte/macrophage lineages. A few cases of LCH coexisting with xanthogranuloma or RDD have been reported. The etiology of these diseases remains unclear. Read More

Ann Thorac Surg 2019 Apr 14;107(4):e285. Epub 2018 Nov 14.

Department of Surgery, Banner University Medical Center-Tucson, University of Arizona, Tucson, Arizona.

Br J Dermatol 2019 Apr 27;180(4):933-934. Epub 2018 Dec 27.

Section of Dermatology and Venereology, Department of Medicine, University of Verona, Piazzale A. Stefani 1, 37126, Verona, Italy.

Medicine (Baltimore) 2018 Oct;97(42):e12869

Department of Cardiology, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders.

Rationale: Familial hypercholesterolemia (FH) is a common inherited cause of coronary heart disease (CHD) and premature death in an early age. Nevertheless, an ischemic heart failure (IHF) associated with FH seems to be rare, and an early diagnosis and therapy could influence the prognosis.

Patient Concerns: In this 13-year-old girl, multiple xanthomas began to develop from the first day of birth. Read More

Ocul Oncol Pathol 2018 Sep 12;4(2):73-78. Epub 2017 Aug 12.

Department of Ophthalmology, Kansai Medical University, Osaka, Japan.

Juvenile xanthogranuloma (JXG) is a rare and benign tumor in infants. A solitary lesion on the eyelid has been reported in patients with JXG. We report a 15-year-old boy with multiple involvement of JXG on both eyelids. Read More

G Ital Dermatol Venereol 2018 Oct;153(5):733-734

Division of Dermatology, Department of Surgery and Translational Medicine, University of Florence Medical School, Florence, Italy.

Clin Exp Dermatol 2019 Jan 21;44(1):91-92. Epub 2018 Sep 21.

The Rotterdam Eye Hospital, Schiedamse Vest 180, 3011 BH, Rotterdam, The Netherlands.

J Pediatr Endocrinol Metab 2018 Oct;31(10):1161-1164

Department of Pediatrics, Faculty of Medicine, University of Yamanashi, Shimokato, Chuo, Yamanashi, Japan.

Background Xanthogranuloma of the sellar region is a rare entity. Its pathology is controversial and it is difficult to strictly differentiate it from craniopharyngioma or Rathke's cyst. Case presentation We report a case of xanthogranuloma accompanied by growth hormone deficiency in an 11-year-old girl. Read More

Dermatol Clin 2018 Oct 16;36(4):487-501. Epub 2018 Aug 16.

Dermatology Clinic, University of Catania, Via Santa Sofia 78, Catania 95123, Italy.

Dermatoscopy and in vivo reflectance confocal microscopy are noninvasive techniques that provide a horizontal approach, with an en face view of the skin structures. Both techniques assist in the clinical diagnosis of a variety of inflammatory and infectious cutaneous disorders. In many cases, they have shown concordance. Read More

Dermatol Clin 2018 Oct 16;36(4):463-472. Epub 2018 Aug 16.

Dermatology Clinic, University of Catania, Via S. Sofia 78, Catania 95123, Italy.

The use of dermatoscopy to assist in the diagnosis of a variety of proliferative, pigmentary, inflammatory, infectious, congenital, and genetic cutaneous and skin appendage disorders is constantly increasing, as it is effective, affordable, noninvasive, and quick to perform. Read More

Dermatol Online J 2018 Jul 26;24(5). Epub 2018 Jul 26.

MedStar Georgetown University Hospital, Washington, District of Columbia Children's National Health System, Washington, District of Columbia.

Fibrous hamartoma of infancy (FHI) is a benign mesenchymal tumor of young children. It has a broad clinical differential diagnosis and is often clinically confused for vascular and malignant soft tissue neoplasms. Recognition of the unique histologic features of FHI, a triphasic population of mature adipose tissue, mature fibrous tissue, and immature mesenchymal tissue, will ensure the correct diagnosis. Read More

Dermatol Online J 2018 May 15;24(5). Epub 2018 May 15.

Department of Dermatology, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.

Subcutaneous juvenile xanthogranuloma (JXG) of the inguinal fold, an unusual location, was diagnosed in an infant. Subcutaneous JXG should be included in the differential diagnosis of subcutaneous nodules of the lower body, despite the absence of the characteristic yellowish hue usually associated with JXG. Read More

Middle East Afr J Ophthalmol 2018 Apr-Jun;25(2):115-117

Department of Pathology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA.

An 8-year-old girl was referred for an amelanotic iris tumor believed to have enlarged slightly over 3 months, suspicious for juvenile xanthogranuloma (JXG). The affected right eye had a lightly pigmented, vascular iris mass measuring 6 mm in basal dimension and 2 mm in thickness. There were no feeder vessels, seeding, inflammatory cells, or cutaneous abnormalities. Read More

J Cutan Pathol 2018 Dec 9;45(12):914-917. Epub 2018 Oct 9.

Department of Internal Medicine, The University of Texas, McGovern School of Medicine, Houston, Texas.

A 21-year-old man with B-cell acute lymphoblastic leukemia developed an eruption of multiple flesh-colored nodules and persistent fevers. A lesional biopsy showed diffuse dermal infiltrates of histiocytes, foam cells, and Touton giant cells consistent with juvenile xanthogranulomatosis. Upon further investigation, the patient's constellation of findings fit criteria for Erdheim-Chester disease. Read More

Eur J Dermatol 2018 Jun;28(3):429-431

Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, Würzburg, Germany.

World Neurosurg 2018 Nov 7;119:189-196. Epub 2018 Aug 7.

Department of Neurological Surgery, The University of Texas Southwestern Medical Center, Dallas, Texas, USA.

Background: Juvenile xanthogranuloma (JXG) is a rare, non-Langerhans cell histiocytic disorder that primarily presents as multiple cutaneous lesions in young males. Solitary lesions in the spinal column are an especially rare presentation of this disease, and central nervous system involvement can portend a poor prognosis. We report an unusual case of an adult woman with an unresectable JXG of the lumbar spine. Read More

Ann Hematol 2018 Nov 6;97(11):2117-2128. Epub 2018 Aug 6.

Department of Pathology 824, Radboud University Medical Center, POB 9101, 6500 HB, Nijmegen, The Netherlands.

The bone marrow is a preferential site for both reactive and neoplastic histiocytic proliferations. The differential diagnosis ranges from reactive histiocyte hyperplasia in systemic infections, vaccinations, storage diseases, post myeloablative therapy, due to increased cell turnover, and in hemophagocytic lymphohistiocytosis, through extranodal Rosai-Dorfman disease to neoplasms derived from histiocytes, including histiocytic sarcomas (HS), Langerhans cell histiocytoses (LCH), Erdheim-Chester disease (ECD), and disseminated juvenile xanthogranuloma (JXG). One of the most important recent developments in understanding the biology of histiocytic neoplasms and in contributing to diagnosis was the detection of recurrent mutations of genes of the Ras/Raf/MEK/ERK signaling pathway, in particular the BRAF mutation, in LCH and ECD. Read More

Ann Pathol 2018 Aug 4;38(4):223-229. Epub 2018 Jul 4.

Hôpital Necker-Enfants Malade, AP-HP, 149, rue de Sèvres, 75015 Paris, France. Electronic address:

EFORT Open Rev 2018 Jun 23;3(6):381-390. Epub 2018 Jun 23.

Second Department of Internal Medicine, Hematology Unit, National and Kapodistrian University of Athens, School of Medicine, Greece.

Non-Langerhans cell histiocytosis (N-LCH) summarizes a group of rare diseases with different clinical presentations, pathogenesis and morphology. These include primary cutaneous N-LCH, cutaneous N-LCH with systemic involvement, and primary extracutaneous systemic forms with occasional cutaneous involvement.The juvenile (JXG) and non-juvenile xanthogranuloma (N-JXG) family of histiocytoses are N-LCH: the JXG family consisting of the JXG (cutaneous), xanthoma disseminatum (cutaneous and systemic) and Erdheim-Chester disease (ECD; systemic); and the N-JXG family consisting of the solitary reticulohistiocytoma (cutaneous), multicentric reticulohistiocytosis (cutaneous and systemic) and Rosai-Dorfman disease (RDD; systemic). Read More

Skin Res Technol 2019 Jan 18;25(1):106-107. Epub 2018 Jul 18.

Department of Dermatology, Hospital de Santa Maria, Lisboa, Portugal.

Juvenile xanthogranuloma is the most common type of non-Langerhans cell histiocytosis. It appears primarily in infancy, where its differential diagnosis includes Spitz nevus, dermal nevus and molluscum contagiosum. We report the case of a 2-year-old boy with juvenile xanthogranuloma, as diagnosed using real-time, in vivo reflectance confocal microscopy. Read More

Cureus 2018 May 8;10(5):e2595. Epub 2018 May 8.

Dermatology, University of Texas Mcgovern Medical School at Houston, Houston, USA.

Spindle cell xanthogranuloma is a rare variant of juvenile xanthogranuloma that most commonly presents in adults as papulonodules. We describe a vascular-appearing case of spindle cell xanthogranuloma on the nose of a 10-year-old boy. The lesion was a dark red, well-demarcated, dome-shaped papule. Read More

Pediatr Dermatol 2018 Sep 12;35(5):582-587. Epub 2018 Jul 12.

Department of Dermatology, University of California, San Francisco, CA, USA.

Background/objectives: Congenital juvenile xanthogranulomas are infrequently described in the medical literature. We report three previously unpublished cases and systematically review the literature to better characterize this variant.

Methods: We surveyed English-language articles indexed in MEDLINE (1951-March 2017) and EMBASE (1974-March 2017) for cases of congenital-onset juvenile xanthogranulomas confirmed on histopathology. Read More

Eur Spine J 2018 Jul 6;27(Suppl 3):555-560. Epub 2018 Jun 6.

Department of Orthopedics and Traumatology, Unit of Musculoskeletal Surgery, Tampere University Hospital, Tampere, Finland.

Purpose: We present a case report that describes neoadjuvant denosumab therapy initiated in a child with a solitary giant cell-rich juvenile xanthogranuloma tumor involving the spine, and review the current literature.

Methods: A giant cell-rich histiocytic lesion involving the 11th thoracic vertebral body was identified in a healthy 5-year-old girl with persistent back and pelvic pain for several months. Imaging examinations and an open biopsy were performed to obtain a definite pathologic diagnosis. Read More

Childs Nerv Syst 2018 Nov 5;34(11):2321-2324. Epub 2018 Jun 5.

Department of Neurosurgery, Rigshospitalet University Hospital, Blegdamsvej 9, 2100, Copenhagen, Denmark.

Histiocytosis is a heterogeneous group of disease entities, comprised by two main categories, namely Langerhans and non-Langerhans cell histiocytoses. Central nervous system involvement in histiocytosis is considered very rare and is often secondary to affection of anatomically related bone structures and/or multi-organ disease. We present a never-before described case of rare childhood histiocytosis with hybrid features of Langerhans cell histiocytosis and juvenile xanthogranuloma confined to the central nervous system in a 2- and a half-year-old boy with distinct treatment response to clofarabine. Read More

J Cutan Pathol 2018 May 24. Epub 2018 May 24.

Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire.

Cutaneous Rosai-Dorfman disease (RDD) can be difficult to distinguish from other non-Langerhans cell histiocytoses, particularly xanthogranuloma (XG). Pathologists use S100 immunoreactivity, abundant plasma cells, and the presence of emperipolesis to distinguish RDD from XG. However, S100 expression has been reported in XG and, in practice, we have occasionally observed emperipolesis in cases that were otherwise clinically and pathologically consistent with XG. Read More

Childs Nerv Syst 2018 Aug 25;34(8):1445-1446. Epub 2018 May 25.

Department of Neurosurgery, Hospital Universitario Donostia, San Sebastián, Guipúzcoa, Spain.

J Cutan Med Surg 2018 Sep/Oct;22(5):488-494. Epub 2018 May 21.

1 Division of Dermatology, Department of Medicine, University of Calgary, Calgary, AB, Canada.

Juvenile xanthogranuloma (JXG) is a member of the non-Langerhans cell group of proliferative disorders of mononuclear phagocytes. JXG is a benign tumour of histiocytic cells. Classic JXG is divided into 2 main clinical subtypes: dome-shaped papules (<0. Read More

Ocul Oncol Pathol 2018 Apr 4;4(3):141-144. Epub 2017 Oct 4.

Department of Ophthalmology, Baylor College of Medicine, Houston, TX, USA.

Purpose: To report a case of juvenile xanthogranuloma that simulated a chalazion and to discuss the association between juvenile xanthogranuloma and Langerhans cell histiocytosis.

Method: Case report and review of literature.

Results: A 13-year-old boy with a prior history of Langerhans cell histiocytosis was referred to our clinic for a possible chalazion. Read More

Mod Pathol 2018 09 9;31(9):1479-1486. Epub 2018 May 9.

Department of Pathology, Stanford University School of Medicine, Stanford, CA, 94305, USA.

Distinguishing classical dendritic cells from other myeloid cell types is complicated by the shared expression of cell surface markers. ZBTB46 is a zinc finger and BTB domain-containing transcription factor, which is expressed by dendritic cells and committed dendritic cell precursors, but not by plasmacytoid dendritic cells, monocytes, macrophages, or other immune cell populations. In this study, we demonstrate that expression of ZBTB46 identifies human dendritic cell neoplasms. Read More

Med Clin (Barc) 2018 Nov;151(10):e59-e60

Anatomía Patológica, Hospital Infanta Margarita, Cabra, Córdoba, España.

An Bras Dermatol 2018 Jan-Feb;93(1):138-140

Dermatology Department at Hospital Santa Casa de Curitiba - Curitiba (PR), Brazil.

Juvenile xanthogranuloma is the most common form of non-Langerhans cell histiocytosis. It manifests clinically as a red-yellow papule, usually showing spontaneous remission. The diagnosis is based on clinical and histological findings. Read More

J Cutan Pathol 2018 Jul 9;45(7):515-521. Epub 2018 May 9.

Oral Pathology Section, Universidad Autónoma de Guadalajara (UAG), Zapopan, Mexico.

Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis (non-LCH) affecting normolipemic infants and children most frequently in the first year of life, often showing spontaneous regression within 3 to 6 years. Classic JXG is characterized by a yellowish asymptomatic papule or nodule, often located in the skin of the head, neck and upper trunk. Oral JXG has been reported, but is rare. Read More

Pediatr Dev Pathol 2018 Mar-Apr;21(2):208-251

2 Lauren V. Ackerman Laboratory of Surgical Pathology, St. Louis Children's Hospital and Dermatopathology, Washington University Medical Center, St. Louis, Missouri.

This article focuses on cutaneous hematopoietic neoplasms that are more likely to be encountered in the pediatric age-group and includes both lymphoproliferative and histiocytic disorders. The cutaneous hematologic disorders in children have a different epidemiologic profile to what is seen during adulthood. Although mycosis fungoides is the most frequent form of cutaneous lymphoma in adults, it is very rare in children. Read More

Vestn Oftalmol 2018;134(1):89-96

Kemerovo Regional Clinical Ophthalmological Hospital, Oktyabrsky pr., 22А, Kemerovo, Russian Federation, 650066.

Juvenile xanthogranuloma (JX) is a benign growth of fibrohistiocytoma range that mainly affects children. The most frequent localization of the tumor is skin integument. Non-cutaneous forms of JX are found in 5-10% of cases; they are manifested as deep lesions of soft tissues, involvement of various organs (oropharynx, lungs, liver, spleen, pericardium, gastrointestinal tract, central nervous system, bone marrow) including the organ of vision. Read More

World Neurosurg 2018 Jun 7;114:22-26. Epub 2018 Mar 7.

Department of Neurosurgery, Nara Medical University, Nara, Japan.

Background: Xanthomatous pituitary diseases rarely occur in childhood. We report a rare pediatric case of a xanthogranuloma that developed in the sellar region, resulting in a visual disturbance that was treated successfully with endoscopic endonasal surgery.

Case Description: A 13-year-old boy came to us with a headache and visual disturbance that occurred 1 month prior. Read More

BMJ Case Rep 2018 Mar 7;2018. Epub 2018 Mar 7.

Oncology, Johns Hopkins School of Medicine, Baltimore, Maryland, USA.

Asian J Neurosurg 2018 Jan-Mar;13(1):172-175

Department of Pathology, Topiwala National Medical College and B. Y. L. Nair Hospital, Mumbai, Maharashtra, India.

Juvenile xanthogranuloma (JXG) is a benign, non-Langerhans cell histiocytic proliferative disorder. We report a case of solitary JXG in an infant presenting as an intraspinal mass. Awareness of this mode of presentation is very important as subsequent prognosis differs from other tumors at the same location. Read More

Pediatr Dermatol 2018 May 28;35(3):329-335. Epub 2018 Feb 28.

Division of Dermatology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.

Background/objectives: Cutaneous juvenile xanthogranuloma is an uncommon, usually benign disease affecting infants and young children. Ocular and other systemic involvement have been reported, but their incidence is unclear, and the utility of routine screening is not well established. Our aim was to characterize the risk of ocular and systemic complications in children with cutaneous juvenile xanthogranuloma. Read More

Dermatol Online J 2017 Jul 15;23(7). Epub 2017 Jul 15.

Children's Mercy Hospital, Kansas City, Missouri.

Congenital juvenile xanthogranuloma (JXG) is an uncommon diagnosis and even more rarely presents with ulceration. We report such a case in a two-week-old girl. Biopsy was performed to rule out any concerning entities. Read More

Am J Ophthalmol Case Rep 2018 Mar 23;9:10-13. Epub 2017 Sep 23.

Department of Pathology, University Basel, Schönbeinstrasse 40, 4003 Basel, Switzerland.

Purpose: To report a case of juvenile xanthogranuloma involving the iris and skin that clincally was diagnosed with an obvious cutaneous lesion.

Observations: A four month-old girl with hyphema and increased intraocular pressure of the left eye persisting for 2 weeks. A suspicious yellow-brown mass with nodular surface and traversed by irregular vascularization was noted on the inferior iris surface. Read More

Rom J Ophthalmol 2017 Jul-Sep;61(3):229-236

Opthalmology Clinic, "Sf. Spiridon" University Hospital, Iași, Romania.

Juvenile xanthogranuloma (JXG) is a benign histiocytic skin disorder mainly encountered during infancy and childhood. Although with multiple potential localizations, less than 1% of the cases exhibit ocular manifestations. Some of these might lead to serious complications, specifically, secondary glaucoma that can result in severe and blinding eye disease. Read More

Clin Exp Dermatol 2018 Jul 10;43(5):642-644. Epub 2018 Feb 10.

University of Texas Health Science Center at San Antonio, San Antonio, TX, USA.

JAAD Case Rep 2018 Mar 16;4(2):155-158. Epub 2018 Jan 16.

Department of Pediatrics and Medicine, Division of Dermatology, Dell Medical School at the University of Texas at Austin, Austin, Texas.