Juvenile Xanthogranuloma of the Head and Neck: Imaging Findings in 11 Cases

Author:

Chalard François1,Nguyen Toan1,Morel Baptiste2,Leiber Louis-Marie3,Roux Charles-Joris4,Petit Philippe5,Soto Ares Gustavo6,Donadieu Jean7,Ducou le Pointe Hubert1

Affiliation:

1. Radiologie pédiatrique, hôpital Armand Trousseau, Paris

2. Radiologie pédiatrique, hôpital Gatien de Clocheville, Tours

3. Radiologie, hôpital universitaire, Angers

4. Radiologie pédiatrique, hôpital Necker Enfants Malades, Paris

5. Radiologie pédiatrique, hôpital de la Timone Enfants, Marseille

6. Neuroradiologie, hôpital Roger Salengro, Lille

7. Onco-hématologie pédiatrique, hôpital Armand Trousseau, Paris, France

Abstract

Background: Juvenile Xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis, occurring mainly in infancy. With an extracutaneous lesion, its diagnosis is difficult, because of a wide clinical spectrum. Here we demonstrate and characterize imaging features of 11 patients with JXG of the head and neck in various locations. Material and Methods: We recorded clinical data and reviewed all imaging studies of 11 patients with JXG of the head and neck. Ultrasonography (US) alone was performed in 1 patient; MRI alone in 6 patients; US and MRI in 1 patient; and US, CT, and MRI in 3 patients. We evaluated the following characteristics in all studies: location and number of lesions, echogenicity and vascularization on US, density on CT, signal intensity on T1- and T2-weighted images, ADC and enhancement on MRI, and tumor boundaries and bone involvement. Results: Lesions were well-defined in 9 cases, and bone erosion was present in 2. On US, lesions were hypoechoic or hyperechoic and with or without vascularization. On CT, lesions were hyper-dense, with no calcification. On MRI, lesions were mildly hyper-intense or iso-intense on T1-weighted images in 8 of 9 patients, hypo-intense on T2-weighted images in 7 of 10, low ADC in 7 of 9, and enhancement in 7 of 7. Conclusions: The diagnosis of extra cutaneous JXG may be proposed, with the following suggestive criteria: age < 1 year, well-defined lesion, mild hyper-intensity on T1-weighted images, hypo-intensity on T2-weighted images, low ADC, enhancement, and possible adjacent bone involvement.

Publisher

Ovid Technologies (Wolters Kluwer Health)

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