Juvenile Xanthogranuloma Workup

  • Author: Theodore Curtis, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Jan 26, 2010
 

Laboratory Studies

  • Juvenile xanthogranuloma (JXG) is mainly a clinical diagnosis, which can be confirmed histologically. Histology of excised lesions and cytology of aqueous fluid may be used.
  • Routine screening for metabolic or hematologic abnormalities is not recommended.
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Imaging Studies

  • High-frequency ultrasound of the anterior segment can aid in identifying lesions, which typically appear as solid homogeneous masses, especially when hyphema is present. Ocular ultrasound may also be used to help confirm the location of the intraocular or orbital lesions.
  • CT scan and MRI are rarely indicated.
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Other Tests

  • Biomicroscopy is the main technique used in ocular diagnosis.
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Procedures

  • Anterior chamber paracentesis to obtain cytologic material has been described and can be useful in cases where the diagnosis is uncertain.
  • Gonioscopy is helpful to identify peripheral lesions and to look for causes of secondary glaucoma.
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Histologic Findings

Lesions contain dense polyhedral histiocytes with large amounts of cytoplasm that often contain vacuoles. Touton giant cells are present in 85% of cases.[1] They have a wreath of nuclei surrounding a homogenous eosinophilic cytoplasmic center.

Immunohistochemistry shows the lesions to be positive for factor XIIIa, CD68, CD163, fascin, and CD14 but negative for S100 and CD1. This can be used to differentiate these lesions from Langerhans cell histiocytoses.

A prominent vascular network is often present. Evidence of tissue inflammation is seen in the swelling and degeneration of epithelial cells and redundant capillary basement membranes with perivascular edema.

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Contributor Information and Disclosures
Author

Theodore Curtis, MD  Assistant Professor, Department of Ophthalmology, University of Colorado; Consulting Staff, Rocky Mountain Lions Eye Institute

Theodore Curtis, MD is a member of the following medical societies: American Academy of Ophthalmology and American Association for Pediatric Ophthalmology and Strabismus

Disclosure: Nothing to disclose.

Coauthor(s)

David T Wheeler, MD  Associate Professor, Departments of Ophthalmology and Pediatrics, Oregon Health & Science University

David T Wheeler, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

Gerhard W Cibis, MD  Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas, Kansas City

Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

J James Rowsey, MD  Former Director of Corneal Services, St Luke's Cataract and Laser Institute, Florida

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

  1. Cypel TK, Zuker RM. Juvenile xanthogranuloma: Case report and review of the literature. Can J Plast Surg. Fall 2008;16(3):175-7. [Medline]. [Full Text].

  2. Liang S, Liu YH, Fang K. Juvenile xanthogranuloma with ocular involvement. Pediatr Dermatol. Mar-Apr 2009;26(2):232-4. [Medline].

  3. Karcioglu ZA, Mullaney PB. Diagnosis and management of iris juvenile xanthogranuloma. J Pediatr Ophthalmol Strabismus. Jan-Feb 1997;34(1):44-51. [Medline].

  4. Vendal Z, Walton D, Chen T. Glaucoma in juvenile xanthogranuloma. Semin Ophthalmol. Jul-Sep 2006;21(3):191-4. [Medline].

  5. Cambiaghi S, Restano L, Caputo R. Juvenile xanthogranuloma associated wiht neurofibromatosis 1: patients without evidence of hematologic malignancies. Pediatr Dermatol. 2004;21(2):97-101. [Medline].

  6. Cadera W, Silver MM, Burt L. Juvenile xanthogranuloma. Can J Ophthalmol. Jun 1983;18(4):169-74. [Medline].

  7. Chang MW. Update on juvenile xanthogranuloma: unusual cutaneous and systemic variants. Semin Cutan Med Surg. Sep 1999;18(3):195-205. [Medline].

  8. DeBarge LR, Chan CC, Greenberg SC, et al. Chorioretinal, iris, and ciliary body infiltration by juvenile xanthogranuloma masquerading as uveitis. Surv Ophthalmol. Jul-Aug 1994;39(1):65-71. [Medline].

  9. Harley RD, Romayananda N, Chan GH. Juvenile xanthogranuloma. J Pediatr Ophthalmol Strabismus. Jan-Feb 1982;19(1):33-9. [Medline].

  10. Hernandez-Martin A, Baselga E, Drolet BA, Esterly NB. Juvenile xanthogranuloma. J Am Acad Dermatol. Mar 1997;36(3 Pt 1):355-67; quiz 368-9. [Medline].

  11. Kaur H, Cameron JD, Mohney BG. Severe astigmatic amblyopia secondary to subcutaneous juvenile xanthogranuloma of the eyelid. J AAPOS. Jun 2006;10(3):277-8. [Medline].

  12. Miszkiel KA, Sohaib SA, Rose GE, et al. Radiological and clinicopathological features of orbital xanthogranuloma. Br J Ophthalmol. Mar 2000;84(3):251-8. [Medline].

  13. Shields JA, Shields CL. Clinical spectrum of histiocytic tumors of the orbit. Trans Pa Acad Ophthalmol Otolaryngol. 1990;42:931-7. [Medline].

  14. Tanz WS, Schwartz RA, Janniger CK. Juvenile xanthogranuloma. Cutis. Oct 1994;54(4):241-5. [Medline].

  15. Weitzman S, Jaffe R. Uncommon histiocytic disorders: The non-Langerhans cell histiocytoses. Pediatr Blood Cancer. 2005;44:1-9. [Medline].

  16. Zvulunov A, Barak Y, Metzker A. Juvenile xanthogranuloma, neurofibromatosis, and juvenile chronic myelogenous leukemia. World statistical analysis. Arch Dermatol. Aug 1995;131(8):904-8. [Medline].

 
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