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FDG-PET/CT findings in systemic mastocytosis: a French multicentre study

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European Journal of Nuclear Medicine and Molecular Imaging Aims and scope Submit manuscript

Abstract

Introduction

Mastocytosis is a clonal haematological disease characterized by uncontrolled proliferation and the activation of mast cells. The value of FDG-PET/CT (FDG-PET) in mastocytosis has yet to be determined.

Methods

We retrospectively identified patients with an established diagnosis of systemic mastocytosis (SM), according to the WHO criteria, who underwent PET using the French Reference Centre for Mastocytosis database. Semi-quantitative and visual analysis of FDG-PET was performed and compared to the clinico-biological data.

Results

Our cohort included 19 adult patients, median age 65 years [range 58–74], including three with smouldering SM (SSM), three with aggressive SM (ASM), 10 with an associated clonal haematological non-mast-cell lineage disease (SM-AHNMD), and three with mast cell sarcoma (MCS). FDG-PET was performed at the time of the SM diagnosis (15/19), to evaluate lymph node (LN) activity (3/19) or the efficacy of therapy (1/19). FDG uptake was observed in the bone marrow (BM) (9/19, 47 %), LN (6/19, 32 %), spleen (12/19, 63 %), or liver (1/19, 5 %). No significant FDG uptake was observed in the SSM and ASM patients. A pathological FDG uptake was observed in the BM of 6/10 patients with SM-AHNMD, appearing as diffuse and homogeneous, and in the LN of 5/10 patients. All 3 MCS patients showed intense and multifocal BM pathological uptake, mimicking metastasis. No correlation was found between the FDG-PET findings and serum tryptase levels, BM mast cell infiltration percentage, and CD30 and CD2 expression by mast cells.

Conclusions

FDG uptake does not appear to be a sensitive marker of mast cell activation or proliferation because no significant FDG uptake was observed in most common forms of mastocytosis (notably purely aggressive SM). However, pathological FDG uptake was observed in the SM-AHNMD and in MCS cases, suggesting a role of FDG-PET in their early identification and as a tool of therapeutic assessment in this subgroup of patients.

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Acknowledgments

We would like to thank Isabelle Hirsch and Laure Cabaret from CEREMAST for their work as clinical trial associates.

Compliance with ethical standards

ᅟ

Conflict of interest

The authors declare that they have no conflict of interest.

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For this type of study formal consent is not required.

Informed consent was obtained from all individual participants included in the study

Sources of support

No financial support was received.

Author information

Author notes

    Authors and Affiliations

    1. Department of Nuclear Medicine, Avicenne Hospital, Assistance Publique - Hôpitaux de Paris (APHP), Bobigny, France

      S. Djelbani-Ahmed, G. Pop, V. Eder & M. Soussan

    2. Sorbonne Paris Cité, Paris 13 University, Bobigny, France

      S. Djelbani-Ahmed & M. Soussan

    3. French Reference center for Mastocytosis (Centre de Référence des Mastocytoses, CEREMAST), Necker Children’s Hospital, APHP, Paris, France

      M. O. Chandesris, D. Canioni, C. Brouzes, K. Hanssens, L. Frenzel, G. Damaj, O. Lortholary & O. Hermine

    4. Department of Haematology, Necker Children’s Hospital, APHP, Paris, France

      M. O. Chandesris, L. Frenzel & O. Hermine

    5. Sorbonne Paris Cité, Imagine Institute, Paris Descartes University, Paris, France

      M. O. Chandesris, D. Canioni, C. Brouzes, L. Frenzel, O. Lortholary & O. Hermine

    6. Department of Internal Medicine and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), AP-HP, Saint Antoine Hospital, Paris, France

      A. Mekinian & O. Fain

    7. Department of Pathology, Necker Children’s Hospital, APHP, Paris, France

      D. Canioni

    8. Laboratory of Haematology, Necker Children’s Hospital, APHP, Paris, France

      C. Brouzes

    9. INSERM U1068, Centre de Recherche en Cancérologie de Marseille (Signaling, Hematopoiesis and Mechanism of Oncogenesis), Paoli Calmettes Institute, Aix-Marseille University, Marseille, France

      K. Hanssens

    10. Department of Internal and Vascular Medicine, Hospices Civils de Lyon, Groupe Hopitalier Sud, Université de Lyon, Pierre-Bénite, France

      I. Durieu & S. Durupt

    11. Department of Internal Medicine, Rennes University Hospital, Rennes, France

      B. Grosbois & S. Besnard

    12. Department of Internal Medicine, Clermont-Ferrand University Hospital, Clermont-Ferrand, France

      O. Tournilhac

    13. Department of Internal Medicine, Purpan University Hospital, Toulouse, France

      O. Beyne-Rauzy

    14. Department of Oncology and Haematology, Saint-Denis University Hospital, Saint-Denis de la Réunion, France

      P. Agapé

    15. Department of Haematology, Reims University Hospital, Reims, France

      A. Delmer

    16. Department of Haematology, Brabois University Hospital, Vandoeuvre les Nancy, France

      D. Ranta

    17. Department of Internal Medicine, Nice University Hospital, Nice, France

      P. Y. Jeandel

    18. Department of Internal Medicine, Tenon Hospital, Paris, France

      S. Georgin-Lavialle

    19. Department of Haematology, Caen University Hospital, Caen, France

      G. Damaj

    20. Department of Infectious Diseases and Tropical Medicine, Necker Children’s Hospital, APHP, Pasteur Institute, Paris, France

      O. Lortholary

    Authors
    1. S. Djelbani-Ahmed
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    2. M. O. Chandesris
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    3. A. Mekinian
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    7. G. Pop
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    8. I. Durieu
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    13. O. Beyne-Rauzy
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    14. P. Agapé
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    15. A. Delmer
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    18. S. Georgin-Lavialle
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    19. L. Frenzel
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    20. G. Damaj
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    21. V. Eder
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    22. O. Lortholary
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    23. O. Hermine
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    24. O. Fain
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    Corresponding author

    Correspondence to M. Soussan.

    Additional information

    S. Djelbani-Ahmed and M. O. Chandesris contributed equally to this work.

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    Djelbani-Ahmed, S., Chandesris, M.O., Mekinian, A. et al. FDG-PET/CT findings in systemic mastocytosis: a French multicentre study. Eur J Nucl Med Mol Imaging 42, 2013–2020 (2015). https://doi.org/10.1007/s00259-015-3117-3

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    • DOI: https://doi.org/10.1007/s00259-015-3117-3

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