Journal of Cytology
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Year : 2011  |  Volume : 28  |  Issue : 4  |  Page : 217-218
Juvenile xanthogranuloma-diagnostic challenge on fine-needle aspiration cytology

Department of Pathology, North Bengal Medical College and Hospital, Darjeeling, India

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Date of Web Publication20-Oct-2011


Juvenile xanthogranuloma (JXG) is a rare cutaneous lesion with paucity of literature on its cytological features. We report one such case which on fine-needle aspiration cytology yielded a mixed population of foamy histiocytes, multinucleated giant cells and variable admixture of lymphocytes and eosinophils causing diagnostic dilemma with other differentials, especially Langerhans cell histiocytosis. However, clinical correlation followed by histopathology confirmed the diagnosis. Hence, JXG has characteristic and diagnostic cytologic features.

Keywords: FNAC; Juvenile xanthogranuloma; xanthoma

How to cite this article:
Bandyopadhyay A, Gangopadhyay M, Chakraborty S. Juvenile xanthogranuloma-diagnostic challenge on fine-needle aspiration cytology. J Cytol 2011;28:217-8

How to cite this URL:
Bandyopadhyay A, Gangopadhyay M, Chakraborty S. Juvenile xanthogranuloma-diagnostic challenge on fine-needle aspiration cytology. J Cytol [serial online] 2011 [cited 2020 May 26];28:217-8. Available from:

   Introduction Top

Juvenile xanthogranuloma (JXG) is a benign non-Langerhans cell histiocytic proliferation of skin and soft tissue. [1] It is a relatively rare cutaneous lesion [2] with limited literature available on its cytological features. Grenko et al. [3] first reported the cytologic features of JXG in 1996. They opine that JXG has characteristic cytologic features.

We report a case of JXG in a 12-year-old boy who presented with a chest wall nodule. Cytologic features of that lesion created diagnostic dilemma with other histiocytic lesions. Ultimately, accurate cytologic diagnosis could be done and it was confirmed by subsequent histopathological examination.

   Case Report Top

A 12-year-old boy presented with a gradually increasing skin nodule in the anterior chest wall ([Figure 1], inset). The lesion was present for more than 6 months and it was 2.5 cm in diameter, yellowish in color, soft in consistency and not fixed to the underlying structure. The lesion was non-tender. All routine investigations, including chest radiograph and serum lipid profile, were within normal limits. The patient was subjected to fine-needle aspiration cytology (FNAC), which revealed dispersed as well as clusters of foamy histiocytes admixed with Touton giant cell having wreath-like nuclei surrounded by foamy cytoplasm and multinucleated foreign body-type giant cells in a background of lymphocytes, eosinophils and few polymorphs [Figure 1]. Wet preparation of the aspirate was also done to exclude any crystals if present. The cytologic differentials were JXG, cutaneous Langerhans cell disease (xanthomatous variety) and xanthoma. Location of the lesion and normal lipid profile along with typical cytological features ruled out possibility of xanthoma. Meticulous examination of the histiocytes was performed and none of the cells showed folded, lobulated or kidney-shaped nucleus typically found in Langerhans cell disease. So, a provisional diagnosis of JXG was made.
Figure 1: FNAC smears showing clusters of foamy histiocytes admixed with Touton giant cells in a background of inflammatory cells (H and E, ×400); Inset: Shows skin nodule in the anterior chest wall

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The lesion was excised; on gross examination, it showed a 2.5 × 2 cm grayish yellow soft mass. Subsequent histopathological examination confirmed our diagnosis, which revealed thinned-out epidermis and a well-circumscribed lesion in the dermis composed of foamy histiocytes, Touton giant cells, foreign body giant cells, lymphocyte, eosinophils and neutrophils.

   Discussion Top

JXG is a normolipemic, non-Langerhans cell histiocytosis characterized by yellowish cutaneous nodules that usually appear in early infancy and childhood, but may present even in adulthood. [4] Most lesions are asymptomatic. Our case was a 12-year-old boy who presented with a non-tender nodule over chest wall. The most common site is the head and neck region with solitary lesions in 60 to 82% of the patients that usually regress spontaneously within 3 to 6 years, leaving a small atrophic scar. [4]

The clinical differential diagnosis includes spitz nevi, mastocytomas and dermatofibromas, [1] which present more academic interest than practical diagnostic difficulty. [5]

JXG has characteristic cytologic features which include presence of numerous finely vacuolated histiocytes, eosinophils, multinucleated giant cells and scattered Touton giant cells. These histiocytes are CD 68 positive and S100 and CD 1a negative, unlike those of Langerhans cell disease. [6]

According to Li et al., [6] JXG should be considered in the differential diagnosis of any histiocytic/fibrohistiocytic soft tissue lesion of childhood and this entity can be diagnosed by its FNAC and immunocytochemistry findings. JXG can be differentiated from xanthoma in FNAC by the distribution of lesion and absence of lipid abnormalities. The major difficulty in clinical diagnosis is with nodular from of Langerhans cell disease. [7] Although stain for CD 68, CD1a and S100 may be necessary for differentiating two conditions, [1] meticulous examination of cells, particularly nuclear groove, may help in proper diagnosis of the condition. As the typical histologic picture of JXG is unmistakable, subsequent histopathological examination confirms the diagnosis. [1] Immunocytochemistry was not done in our case due to lack of infrastructure.

We conclude that JXG has characteristic diagnostic cytologic features and this case has been presented to highlight the importance of FNAC in the preoperative diagnosis of this rare cutaneous lesion.

   References Top

1.Burgdorf WHC, Zelger B. The histiocytoses.In: Elder DE, Elenitsas R, Johnson BL, Murphy GF, Xu X, editors. Lever's histopathology of the skin. 10 th ed. Philadelphia: Lippincott William and Wilkins; 2009. p. 667-8.  Back to cited text no. 1
2.Iwuagwu FC, Rigby HS, Payne F, Reid CD. Juvenile xanthogranuloma variant: a clinicopathological case report and review of literature. Br J Plast Surg 1999;52:591-3.  Back to cited text no. 2
3.Grenko RT, Sickel JZ, Abendroth CS, Cilley RE. Cytologic features of deep juvenile xanthogranuloma. Diagn Cytopathol 1996;15:329-33.  Back to cited text no. 3
4.Hernandez-Martin A, Baselga E, Drolet BA, Esterly NB. Juvenile xanthogranuloma. J Am Acad Dermatol 1997;36:355-67.  Back to cited text no. 4
5.Miguelez A, Rodriguez- Peralto JL, Santos-Briz A, Vargas J, Lopez-Rios F. Mitotically active juvenile xanthogranuloma. Arch Pathol Lab Med 2002;126:1219-21.  Back to cited text no. 5
6.Li HC, Roux JJ, Pranikoff T, Kilpatrick SE, Cappellari JO. Deep juvenile xanthogranuloma presenting as a chest wall mass: a case report. Acta Cytol 2008;52:607-11.  Back to cited text no. 6
7.Chu AC. Histiocytoses.In: Burns T, Breathnach S, Cox N, Griffith C, editors. Rook's textbook of dermatology. 7 th ed. Massachusetts: Blackwell Sciences; 2004.p. 52.16.  Back to cited text no. 7

Correspondence Address:
Arghya Bandyopadhyay
Department of Pathology, North Bengal Medical College and Hospital, Darjeeling
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-9371.86356

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