title: The role of 18FDG, 18FDOPA PET/CT and 99mTc bone scintigraphy imaging in Erdheim-Chester disease.
abstract: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocitosis, characterized by multisystemic xanthogranulomatous infiltration by foamy histiocytes that stain positively for CD68 marker but not express CD1a and S100 proteins. Etiology and pathogenesis are still unknown and only about 500 cases are related in the literature. Multisystemic involvement leads to a wide variety of clinical manifestations that results in a poor prognosis although recent advances in treatment. We present the clinical, nuclear medicine findings and therapeutic aspects of a serie of 6 patients with histopathological diagnosis of ECD, who have undergone both bone scintigraphy (BS) and 18F-fluorodeoxyglucose (18FDG)-PET/CT scans in our institution. A complementary 18F-fluorodopa (18FDOPA)-PET/CT was performed in one case. Three different presentations of the disease were observed in our casuistic: most indolent form was a cutaneous confined disease, presented in only one patient. Multifocal involvement with central nervous system (CNS) preservation was observed in two patients. Most aggressive form consisted in a systemic involvement with CNS infiltration, presented in three patients. In our experience neurological involvement, among one case with isolate pituitary infiltration, was associated with mortality in all cases. 18FDG-PET/CT and BS were particularly useful in despite systemic involvement; locate the site for biopsy and the treatment response evaluation. By our knowledge, 18FDOPA-PET/CT not seems useful in the initial staging of ECD. A baseline 18FDG-PET/CT and BS may help in monitoring the disease and could be considered when patients were incidentally diagnosed and periodically 18FDG-PET/CT must be performed in the follow up to evaluate treatment response.
authors.affiliation: Department of Nuclear Medicine, Virgen del Rocío Universitary Hospital, Seville, Spain. Electronic address: javier191185@gmail.com.
authors.forename: F J
authors.initials: FJ
authors.lastname: García-Gómez
authors.affiliation: Department of Nuclear Medicine, Virgen del Rocío Universitary Hospital, Seville, Spain.
authors.forename: I
authors.initials: I
authors.lastname: Acevedo-Báñez
authors.affiliation: Department of Nuclear Medicine, Virgen del Rocío Universitary Hospital, Seville, Spain.
authors.forename: R
authors.initials: R
authors.lastname: Martínez-Castillo
authors.affiliation: Department of Nuclear Medicine, Virgen del Rocío Universitary Hospital, Seville, Spain.
authors.forename: J L
authors.initials: JL
authors.lastname: Tirado-Hospital
authors.affiliation: Department of Nuclear Medicine, Virgen del Rocío Universitary Hospital, Seville, Spain.
authors.forename: J I
authors.initials: JI
authors.lastname: Cuenca-Cuenca
authors.affiliation: Department of Nuclear Medicine, Virgen del Rocío Universitary Hospital, Seville, Spain.
authors.forename: V M
authors.initials: VM
authors.lastname: Pachón-Garrudo
authors.affiliation: Department of Nuclear Medicine, Virgen del Rocío Universitary Hospital, Seville, Spain.
authors.forename: R M
authors.initials: RM
authors.lastname: Álvarez-Pérez
authors.affiliation: Department of Nuclear Medicine, Virgen del Rocío Universitary Hospital, Seville, Spain.
authors.forename: R
authors.initials: R
authors.lastname: García-Jiménez
authors.affiliation: Department of Pathology, Virgen del Rocío Universitary Hospital, Seville, Spain.
authors.forename: E
authors.initials: E
authors.lastname: Rivas-Infante
authors.affiliation: Department of Internal Medicine, Virgen del Rocío Universitary Hospital, Seville, Spain.
authors.forename: J S
authors.initials: JS
authors.lastname: García-Morillo
authors.affiliation: Department of Nuclear Medicine, Virgen del Rocío Universitary Hospital, Seville, Spain.
authors.forename: I
authors.initials: I
authors.lastname: Borrego-Dorado
mesh_terms: D000328:Adult; D000368:Aged; D001842:Bone and Bones / Q000000981:diagnostic imaging; D004295:Dihydroxyphenylalanine / Q000031:analogs & derivatives*; D031249:Erdheim-Chester Disease / Q000175:diagnosis*; D005260:Female; D019788:Fluorodeoxyglucose F18; D006801:Humans; D008297:Male; D008875:Middle Aged; D064847:Multimodal Imaging; D011877:Radionuclide Imaging; D019275:Radiopharmaceuticals; D012189:Retrospective Studies; D013667:Technetium; D014057:Tomography, X-Ray Computed / Q000009:adverse effects
chemical_list: D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18; C043437:fluorodopa F 18; D004295:Dihydroxyphenylalanine; D013667:Technetium
keywords: 18FDG; 18FDOPA; Bone scintigraphy; Erdheim–Chester disease; PET/CT