Systemic juvenile xanthogranulomatosis imitating a malignant abdominal wall tumor with lung metastases

Juvenile xanthogranulomatosis (JXG) is a rare benign condition, which usually presents with characteristic skin lesions and can be diagnosed clinically. However, systemic JXG may involve a wide range of extracutaneous sites and may pose a diagnostic dilemma for the clinician, radiologist, and pathologist. In particular it may simulate malignancy. Here, we report a case of systemic JXG within the abdominal wall musculature and lungs, which imitated a sarcoma with pulmonary metastases on computerized tomography. To the best of our knowledge, this is the first such reported case. In particular, systemic disease presenting without skin lesions is unusual.