Multisystem FDG-Avid Lesions Mimicking Malignancy in a Teenager with Hyper-IgE Syndrome: A Case Study with Literature Review

Articles in Press

Document Type : Case report

Authors

1 Tehran University of Medical Sciences, Nuclear medicine

2 Tehran University of Medical Sciences Department of Nuclear Medicine

3 Tehran University of Medical Sciences Nuclear medicine

4 ehran University of Medical Sciences, Nuclear medicine

5 Department of Nuclear Medicine, Vali-Asr Hospital, Tehran University of Medical Sciences

10.22038/aojnmb.2026.91014.1671

Abstract

Hyper-IgE Syndrome (HIES) is a rare immunodeficiency predisposing patients to infections, inflammation, and occasional neoplasia. We report a 17-year-old boy with HIES who presented with severe right thigh pain. FDG-PET/CT demonstrated a right adrenal mass (SUVmax 5.4), multiple pulmonary nodules, and a lytic-sclerotic femoral lesion (SUVmax 4.6), initially suggesting disseminated malignancy. Biopsy confirmed acute osteomyelitis and a benign spindle cell tumor of the adrenal gland. Two months later, persistent fever and elevated ESR prompted re-evaluation, revealing intense FDG uptake in the thoracic aorta (SUVmax 20.5) consistent with large vessel vasculitis, later confirmed by angiography. The combination of infection, benign tumor, and vasculitis illustrates the broad FDG uptake spectrum in HIES and the risk of misdiagnosis as malignancy. Integration of clinical, imaging, and histopathologic data was critical for accurate diagnosis. This case highlights FDG-PET/CT’s value in detecting inflammatory vascular disease and monitoring therapy, while reinforcing the importance of biopsy for definitive diagnosis. In immunodeficient patients, a multimodal diagnostic approach is essential to distinguish between malignant and benign FDG-avid lesions.

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Asia Oceania Journal of Nuclear Medicine and Biology

Articles in Press, Accepted Manuscript
Available Online from 21 February 2026

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