
==== Front
Eur J Nucl Med Mol Imaging
Eur J Nucl Med Mol Imaging
European Journal of Nuclear Medicine and Molecular Imaging
1619-7070
1619-7089
Springer Berlin Heidelberg Berlin/Heidelberg

38758369
6751
10.1007/s00259-024-06751-5
Image of the Month
Distinct [18F]FDG-PET imaging features of a newly recognized and yet uncharacterized RDD-ECD overlap disease entity
http://orcid.org/0000-0002-4849-3292
Huellner Martin W. martin.huellner@usz.ch

1
http://orcid.org/0000-0003-0850-3326
Bühler Marco M. 2
http://orcid.org/0000-0001-9206-4885
Kölzer Viktor H. 2
http://orcid.org/0000-0003-4158-4666
Limani Perparim 3
http://orcid.org/0000-0003-0495-1674
Rösler Wiebke 4
1 https://ror.org/02crff812 grid.7400.3 0000 0004 1937 0650 Department of Nuclear Medicine, University Hospital Zurich, University of Zurich, Raemistrasse 100, Zurich, CH-8091 Switzerland
2 https://ror.org/02crff812 grid.7400.3 0000 0004 1937 0650 Department of Pathology and Molecular Pathology, University Hospital Zurich, University of Zurich, Zurich, Switzerland
3 https://ror.org/02crff812 grid.7400.3 0000 0004 1937 0650 Department of Surgery and Transplantation, University Hospital Zurich, University of Zurich, Zurich, Switzerland
4 https://ror.org/02crff812 grid.7400.3 0000 0004 1937 0650 Department of Hematology and Oncology, University Hospital Zurich, University of Zurich, Zurich, Switzerland
17 5 2024
17 5 2024
2024
51 11 34653466
27 3 2024
1 5 2024
© The Author(s) 2024
2024
https://creativecommons.org/licenses/by/4.0/ Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

University of ZurichOpen access funding provided by University of Zurich

issue-copyright-statement© Springer-Verlag GmbH Germany, part of Springer Nature 2024
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pmcA newly recognized histiocytosis entity, encompassing clinical and histopathologic features of Rosai-Dorfman disease (RDD) and Erdheim-Chester disease (ECD), is driven by MAP2K1 mutations [1, 2]. [18F]fluorodeoxyglucose ([18F]FDG) positron emission tomography (PET) features have not yet been reported.

This 46 year-old man presented with a two-year history of clinical hallmarks resembling RDD rather than ECD, including lymphadenopathy and painless testicle enlargement [3], being also visible on [18F]FDG-PET (A). Testicular RDD-ECD involvement was also reported in 6/13 patients by Razanamahery et al. [2]. Diffuse omental proliferations, manifesting as faintly [18F]FDG-avid omental thickening resembling a fishing net (SUVmax 5.5; A, B, C), and symmetric large-joint synovitis were reported as specific features of RDD-ECD [1, 2], Notably, none of these features are characteristic of RDD or hitherto known ECD subtypes. Other RDD and/or ECD features were absent [4–7].

Open biopsy targeted peritoneal lesions (D) localized on the diaphragm (d), peritoneum (p) and greater omentum (go). Histopathology revealed nodular fibrosis, foamy cell infiltrates, pigment deposits and chronic perivascular inflammatory infiltrates (E). Molecular genetic analyses confirmed presence of a characteristic MAP2K1 mutation (p.Q56P).

Diamond et al. effectively treated a patient harboring the identical mutation with MEK inhibitors [8]. FAPI-PET focusing on fibrosis aspects of histiocytosis might help determining disease extent and assessing treatment response [9, 10].

In summary, the newly recognized RDD-ECD overlap histiocytosis demonstrates distinct [18F]FDG-PET features setting it apart from RDD and ECD. The concurrent presence of omental proliferations, symmetric large-joint synovitis, and high testicular uptake should raise suspicion for this yet uncharacterized disease.

Author contributions

All authors contributed to the conception and design. Image analysis / image compilation and design of the final image were performed by M.H. The first draft of the manuscript was written by M.H: and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Funding

Open access funding provided by University of Zurich. The authors declare that no funds, grants, or other support was received during the preparation of this manuscript.

Open access funding provided by University of Zurich

Declarations

Competing Interests

The authors have no relevant financial or non-financial interests to disclose.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
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