PEDIATRIC SURGERY UPDATE ©

VOLUME 53 NO 02 AUGUST 2019

Xanthogranulomas

Xanthogranulomas are rare benign nodules that usually arise in the subcutaneous tissue. When they appear in early infancy or childhood they are called juvenile xanthogranulomas (JXG). Approximately 20% are present at birth and most arise in the first year of life. The anatomic predilection of appearance of juvenile xanthogranuloma in children occurs in the head and neck region, though appearance on the trunk, extremities and extracutaneous locations has also been reported. Skin lesions are self-limited and can vary in size; they can be solitary or multiple. In the eye, particularly the uveal tract, is the most frequent site of extracutaneous involvement. JXG are composed of collections of histiocytes, foamy cells and Touton giant cells. The diagnosis of JXG requires histologic analysis of a biopsy specimen or the nodule itself.  JXG is a benign cutaneous fibrohistiocytic lesion with a type of granulomatous process. Clinically the lesion is papulonodular with a tan to orange color and several millimeters in size. Children with multiple skin lesions or younger than two years of age are at a greater risk for ocular involvement. Systemic JXG which involves the visceral organs is rare occurring in less than 5% of cases. Systemic JXG can appear in the lung, heart, gastrointestinal tract, CNS, adrenal gland, pituitary gland, bones or kidney. There is a sex male predilection for JXG. Spontaneous regression of the cutaneous lesion is frequent. Differential diagnosis includes spitz nevi, mastocytomas, and dermatofibromas. Should the cutaneous nodule keep growing or cause functional impairment it should be removed surgically. Removal must be complete to avoid recurrence of the lesion. JXG generally carries a good prognosis.  Congenital giant JXG is a rare subtype most commonly affecting newborn females.

References:
1- Pajaziti L, Hapaiu SR, Pajaziti A: Juvenile xanthogranuloma: a case report and review of the literature. BMC Res Notes. 7:174, 2014
2- Logue ME(1), Elwood H, Smidt A: Congenital juvenile xanthogranuloma with ulceration: a pediatric case report. Dermatol Online J. 23(7), 2017
3-  Paxton CN, O'Malley DP, Bellizzi AM, et al: Genetic evaluation of juvenile xanthogranuloma: genomic abnormalities are uncommon in solitary lesions, advanced cases may show more complexity. Mod Pathol. 30(9):1234-1240, 2017
4- Mitra S, Gupta S, Menon P, Rao KL, Bal A: Juvenile Xanthogranuloma: Presenting as an Isolated Renal Involvement. Fetal Pediatr Pathol. 35(6):420-424, 2016
5- Zahir ST, Sharahjin NS, Vahedian H, Akhavan A: Juvenile xanthogranuloma presenting as a large neck mass and ocular complications: a diagnostic and therapeutic dilemma. BMJ Case Rep. 2014 Apr 15;2014
6- Berti S, Coronella G, Galeone M, Balestri R, Patrizi A, Neri I: Giant congenital juvenile xanthogranuloma. Arch Dis Child. 98(4):317, 2013

   ERAS

*Edited by: Humberto Lugo-Vicente, MD, FACS, FAAP
P.O. Box 10426, Caparra Heights Station, San Juan, Puerto Rico 00922-0426
Tel (787)-999-9450 Fax (787)-720-6103 E-mail: TITOLUGO@COQUI.NET
Internet Address:HTTP://HOME.COQUI.NET/TITOLUGO
® Copyright Office of the Library of Congress 1997


Home
Table
Index
Past
Review
Submit
Techniques
Editor
Handbook
Articles
Download
UPH
Journal Club
WWW
Meetings
Videos
Pediatric Surgery Update ISSN 1089-7739
Last updated: September 2019