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Juvenile Xanthogranulomas in the First Two Decades of Life a Clinicopathologic Study of 174 Cases with Cutaneous and Extracutaneous Manifestations

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Juvenile Xanthogranulomas in the First Two Decades of Life a Clinicopathologic Study of 174 Cases with Cutaneous and Extracutaneous Manifestations The American Journal of Surgical Pathology 27(5): 579–593, 2003 © 2003 Lippincott Williams & Wilkins, Inc., Philadelphia Juvenile Xanthogranulomas in the First Two Decades of Life A Clinicopathologic Study of 174 Cases With Cutaneous and Extracutaneous Manifestations Louis P. Dehner, M.D. Juvenile xanthogranulomas (JXG) is a histiocytic disorder, pri- Langerhans cell histiocytosis together as “dendritic cell- marily but not exclusively seen throughout the first two de- related” histiocytoses. cades of life and principally as a solitary cutaneous lesion. This Key Words: Juvenile xanthogranuloma—Nevoxanthoendo- study is a retrospective clinical and pathologic review of 174 thelioma—Histiocytosis—Histiocytic disorders—Langerhans cases documenting the cutaneous and extracutaneous manifes- cell histiocytosis—Touton giant cell. tations in patients presenting from the neonatal period to 20 years of age (mean 3.3 years; median 1 year). There was a male Am J Surg Pathol 27(5): 579–593, 2003. predominance (99 male:75 female) in all categories of clinical presentation, but especially notable in the group with multiple cutaneous lesions (12 male:1 female). A solitary cutaneous Juvenile xanthogranuloma (JXG) or nevoxanthoendo- lesion accounted for 67% of all cases, followed by a solitary thelioma, as it was known in the earlier literature, is subcutaneous or deep soft tissue mass (28 cases, 16%), multiple the “other” principal histiocytic disorder of childhood, cutaneous lesions (13 cases, 7%), a solitary extracutaneous, which is often compared with and differentiated from nonsoft tissue lesion (9 cases, 5%), and multiple cutaneous and 4,29,30,40,51 visceral–systemic lesions (8 cases, 5%). The recorded deaths Langerhans cell histiocytosis (LCH). In differ- due to disease included two neonates with systemic JXG who ent classifications and discussions, JXG has been re- developed hepatic failure and thrombocytopenia and at autopsy garded as a non-LCH together with several other histio- had giant cell-neonatal hepatitis in addition to JXG in the liver cytic entities including papular xanthoma, benign cephalic and other visceral sites. A third death in a 3-month-old boy with histiocytosis, sinus histiocytosis with massive lymphade- a retroperitoneal–pelvic JXG occurred after failure to control severe hypercalcemia. The characteristic Touton giant cell in nopathy (Rosai-Dorfman disease), and hemophagocytic 4,58 variable numbers was a consistent feature of the cutaneous histiocytosis. lesions; however, these cells were either absent or present in As a clinical and pathologic entity, Adamson1 and reduced numbers in the various extracutaneous lesions when McDonagh34 in 1905 and 1912, respectively, are credited compared with JXG in the skin. Spindle cells intermingled with the first reports of JXG in the literature, although among the mononuclear cells or forming short fascicles were 19 seen in both cutaneous and extracutaneous lesions. Immuno- Helwig and Hackney have made reference to Rudolf histochemistry was performed on all extracutaneous lesions, Virchow as describing a case in a child with “cutaneous and the constituent cells, regardless of their individual morpho- xanthomas” in 1871. Adamson’s patient was a 2.5-year- logic features, were uniformly positive for vimentin, CD68, and old boy with multiple cutaneous nodules in the head and factor XIIIa and negative for S-100 protein and CD1a. It is neck region and trunk; the initial lesion was detected widely held that JXG is a proliferative disorder of dendrocytes, possibly dermal dendrocytes; thus, its clinical and pathologic within the first 2 weeks of life, and additional lesions had 1 similarities to Langerhans cell histiocytosis are not entirely developed at the time of the case presentation. A biopsy unexpected in light of the most recently proposed international had not been performed in this case when it was pre- classification of histiocytic disorders, which includes JXG and sented before the Dermatological Society of London. Several years later in 1912, McDonagh reported five cases in children of so-called nevoxanthoendothelioma 34 From the Lauren V. Ackerman Laboratory of Surgical Pathology, and biopsies had been performed in four of the five. Barnes-Jewish and St. Louis Children’s Hospitals, Washington These five children all had multiple cutaneous and/or University Medical Center, St. Louis, Missouri, U.S.A. subcutaneous lesions that were detected at birth in two Address correspondence and reprint requests to Louis P. Dehner, MD, Campus Box 8118, 660 S. Euclid Avenue, St. Louis, MO 63110, cases, in the first 3 weeks of life in two others, and at 10 U.S.A.; e-mail: [email protected] months of age in the fifth child. A dense dermal infiltrate 579 580 L. P. DEHNER of round cells was the microscopic characterization of mercial stainer using the manufacturer’s protocol with the biopsies in three cases and the fourth biopsy also had the application of the following antibodies: vimentin a spindle cell component. Xanthomatous cells were also (1:200 dilution, Biogenex), CD68 (1:2000, Dako), factor described in these biopsies. Only one case had a specific XIIIa (1:400, Calbiochem), CD1a (1:40, Immunotech), notation about the presence of giant cells and a comment and S-100 protein (1:6000, Dako). about their absence was made in two others. McDonagh concluded that these lesions had taken “their origin from endothelium of the capillaries.”34 He also noted that RESULTS these lesions had a resemblance to a “soft fibroma or connective-tissue tumors” during their evolution. In the The study group consisted of 174 cases, representing final clinical stage, McDonagh pointed that “these cells 99 (57%) males and 75 (43%) females who ranged in age disappear” with the implication that the lesion(s) even- from newborn to 20 years of age with a mean age of 3.3 tually resolve spontaneously. It could be argued that years and a median age of 1 year at the time of diagnosis. McDonagh had made most of the fundamental clinical Although 79 (45%) patients were diagnosed before 1 and morphologic observations about JXG in his now year of age, 33 (19%) patients with solitary cutaneous 90-year-old publication.34 lesions were diagnosed between the ages of 6 and 20 Over the last decade or so, we have been provided the years, which accounted for the difference between the opportunity to examine a number of cases of JXG pre- mean and median ages (Fig. 1). senting in the first two decades of life. This series has A solitary cutaneous lesion was the most common grown to 174 cases and has afforded us a perspective on clinical presentation among the 174 cases (Table 1). A the diversity of clinical presentations and histopathologic solitary mass in the subcutis and/or deeper soft tissues features that have challenged us and our colleagues. was next in frequency accounting for 16% of cases. Mul- tiple skin and soft tissue lesions with or without deep organ involvement accounted for a total of 21(12%) MATERIALS AND METHODS cases. A solitary, extracutaneous lesion comprised a total Case Selection of nine cases (5%), seven of which presented in the head and neck region. Eight children (4%) had systemic JXG, A systematic search of the computerized database and which included the involvement of two or more visceral the earlier “organ-lesion file” of the Lauren V. Ackerman organs in addition to multiple cutaneous and subcutane- Laboratory of Surgical Pathology, Barnes-Jewish, and ous lesions. St. Louis Children’s Hospitals at the Washington University Medical Center (St. Louis, MO, USA) was conducted for all cases with the pathologic diagnosis of Solitary Skin JXG, non-LCH, histiocytosis of unclassified type, xan- thoma, and xanthogranulomas in patients whose ages at Approximately two thirds of JXGs presented as a soli- diagnosis ranged from newborn to 20 years. Cases with tary cutaneous nodule with a predilection for the head the pathologic diagnoses of LCH, Rosai-Dorfman dis- and neck region (Table 2). There were 62 males and 54 ease, or sinus histiocytosis with massive lymphadenop- females who ranged in age from 3 months to 20 years at athy, hemophagocytic histiocytosis and “malignant his- diagnosis (mean 4.4 years; median 2 years). This presen- tiocytosis” or anaplastic large cell lymphoma, regardless tation of JXG was the only one seen in all age groups of age, were not included in this analysis. (Fig. 1). Microscopic slides and pathology reports were re- A firm nodule was infrequently in excess of 1 cm in trieved and the hematoxylin and eosin-stained sections greatest dimension, but the tinctorial character varied were examined to confirm the diagnosis of JXG using from flesh colored to erythematous or yellowish in ap- 33,52,55 established pathologic criteria. If the diagnosis pearance. Rather than a nodule, three children had could not be confirmed by additional histologic and/or slightly raised plaque-like lesions with a reddish to yel- immunohistochemical studies, the case was eliminated lowish coloration. Excisional biopsy was the most com- from further consideration. mon form of management, and local recurrence was not recorded in any case, although one patient developed Immunohistochemical Staining additional cutaneous lesions during the follow-up period. This patient was assigned to the category of multiple Immunohistochemical studies were performed on all cutaneous lesions, illustrating the point that a solitary extracutaneous lesions, which for the most part had been lesion at presentation may herald the appearance of ad- submitted in consultation from other institutions. The ditional lesions over the next several weeks of months in immunoperoxidase studies were performed on a com- a small minority of cases. Am J Surg Pathol, Vol. 27, No. 5, 2003 JUVENILE XANTHOGRANULOMAS 581 FIG. 1. Bar graph showing the number of cases (ordinate) and age groupings (abscissa) of the five clinical groups of JXG. Soft Tissue and multiple nodules on the peritoneum and in the omen- tum. No skin or peripheral soft tissue lesions were noted.
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