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Erdheim-Chester disease and its findings on bone scintigraphy imaging

Czech version

Authors: Z. Řehák 1;  V. Ptáčník 3;  G. Horňák 5;  Z. Adam 13;  J. Karban 4;  K. Popelková 6;  M. Skácelová 6;  E. Krčálová 8;  Z. Šimková 9;  V. Kratochvíl 10;  S. Soboličová 1;  J. Foukal 12;  Z. Balážová 1,14;  M. Hulová 2;  V. Látal 7;  M. Sýkora 11;  M. Doležílek 15;  L. Pour 13
Authors‘ workplace: Oddělení nukleární medicíny 1;  Oddělení urologické onkologie, Masarykův onkologický ústav, Brno 2;  Ústav nukleární medicíny 3;  1. Interní klinika 1. LF UK a VFN v Praze 4;  Klinika nukleární medicíny 5;  III. interní klinika 6;  Hemato-onkologická klinika LF UP v Olomouci a FN Olomouc 7;  Oddělení nukleární medicíny, LF UK v Hradci Králové a FN Hradec Králové 8;  Oddělení nukleární medicíny, Nemocnice Jihlava 9;  Oddělení nukleární medicíny 10;  Oddělení klinické hematologie, Nemocnice České Budějovice 11;  Klinika radiologie a nukleární medicíny 12;  Interní hematoonkologická a onkologická klinika, LF MU Brno a FN Brno 13;  Oddělení nukleární medicíny, Nemocnice Kyjov 14;  Klinika nukleární medicíny, LF OU a FN Ostrava, ČR 15
Published in: NuklMed 2026;15:18-24
Category: Original Article

Overview

Introduction: Erdheim-Chester disease is a very rare disorder belonging to the group of malignant histiocytosis. Bone involvement is typical for this disease and can be verified by bone scintigraphy. The disease also tends to have extraskeletal involvement, which includes involvement of the CNS, orbits, heart, lungs, periaortic and perirenal tissues and other tissues in the body.

Materials and methods: Retrieval and retrospective analysis of scintigraphic images of this disease in 11 patients examined in PET centers in the Czech Republic.

Results: All patients (11/11) had some pathological finding on skeletal scintigraphy, all patients (11/11) had high radiopharmaceutical accumulation in the distal femurs, 10/11 also in the proximal tibias, 9/11 in the distal tibias and 8/9 also in the forearms or elbows (8/9). Other less frequent findings included accumulations in the proximal humeri, fibulae, metatarsals and tarsals, in the clavicles, craniofacial skeleton, pelvis.

Conclusion: The skeletal scintigraphy findings are relatively typical for this disease –⁠ symmetrically increased accumulation of osteotropic radiopharmaceutical in the long bones of the limbs (often up to the periphery or including the clavicles) with several possible additional deposits in the craniofacial skeleton or pelvis, with the typical sign of “hot knees” or accumulation in the bones of the forearms. The publication aims to increase awareness of the disease in the nuclear medicine community, given that bone scintigraphy can be and still is indicated for lower limb pain.

Keywords:

Erdheim-Chester disease – bone scintgraphy – histiocytoses – hot knees – diabetes insipidus

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