Erdheim-Chester disease (ECD) is a rare form of non–Langerhans’ cell histiocytosis with unknown aetiology. The typical ECD diagnostic triad is composed by bone pain, diabetes insipidus and painless bilateral exophthalmos. About half of the patients have extraskeletal manifestations and the prognosis is worse if central nervous system and cardiovascular system are involved. We report a case of a 24 years old man came at our attention suspect for central diabetes insipidus (CDI). He also presented arthromyalgia especially in the knees. He performed a Brain Magnetic Resonance (MR) which showed presence of multiple hyperintense lesions, one of these in neurohypophysis. He also performed a chest and abdomen Contrast Enhancement Computed Tomography that didn’t show abnormalities. Lately a whole-body 18-Fluorine-labelled 2-deoxy-2-fluoro-D-glucose positron emission tomography/computed tomography (18F-FDG PET/CT) was performed and it confirmed the presence of brain lesion seen in MR, and the absence of visceral lesions, but also showed the presence of an atypical bone uptake of 18F-FDG. These findings, however, associated with clinical data have raised the suspicion of an ECD, corroborated by 99mTc-methyl-diphosphonate skeletal scintigraphy findings and confirmed by histological findings. While conventional imaging modalities have mostly been confined to the study of a single region, 18F-FDG PET/CT scanning is usually applied as a whole-body examination, and thus, it appears to be very advantageous in this regard. Although the diagnosis of ECD is histological, molecular imaging as 18F-FDG PET/CT and 99mTc-methyl-diphosphonate skeletal scintigraphy, are useful o adress to a correct diagnosis.

18F-FDG positron emission tomography/computed tomography and 99mTc-MDP skeletal scintigraphy in a case of Erdheim-Chester disease / Niccoli Asabella A; Cimino A; Altini C; Notaristefano A; Rubini G. - In: HELLENIC JOURNAL OF NUCLEAR MEDICINE. - ISSN 1790-5427. - 14:3(2011), pp. 311-312.

18F-FDG positron emission tomography/computed tomography and 99mTc-MDP skeletal scintigraphy in a case of Erdheim-Chester disease

NICCOLI ASABELLA, ARTOR;RUBINI, Giuseppe
2011

Abstract

Erdheim-Chester disease (ECD) is a rare form of non–Langerhans’ cell histiocytosis with unknown aetiology. The typical ECD diagnostic triad is composed by bone pain, diabetes insipidus and painless bilateral exophthalmos. About half of the patients have extraskeletal manifestations and the prognosis is worse if central nervous system and cardiovascular system are involved. We report a case of a 24 years old man came at our attention suspect for central diabetes insipidus (CDI). He also presented arthromyalgia especially in the knees. He performed a Brain Magnetic Resonance (MR) which showed presence of multiple hyperintense lesions, one of these in neurohypophysis. He also performed a chest and abdomen Contrast Enhancement Computed Tomography that didn’t show abnormalities. Lately a whole-body 18-Fluorine-labelled 2-deoxy-2-fluoro-D-glucose positron emission tomography/computed tomography (18F-FDG PET/CT) was performed and it confirmed the presence of brain lesion seen in MR, and the absence of visceral lesions, but also showed the presence of an atypical bone uptake of 18F-FDG. These findings, however, associated with clinical data have raised the suspicion of an ECD, corroborated by 99mTc-methyl-diphosphonate skeletal scintigraphy findings and confirmed by histological findings. While conventional imaging modalities have mostly been confined to the study of a single region, 18F-FDG PET/CT scanning is usually applied as a whole-body examination, and thus, it appears to be very advantageous in this regard. Although the diagnosis of ECD is histological, molecular imaging as 18F-FDG PET/CT and 99mTc-methyl-diphosphonate skeletal scintigraphy, are useful o adress to a correct diagnosis.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11586/132035
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