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

  • S. Djelbani-Ahmed
  • M. O. Chandesris
  • A. Mekinian
  • D. Canioni
  • C. Brouzes
  • K. Hanssens
  • G. Pop
  • I. Durieu
  • S. Durupt
  • B. Grosbois
  • S. Besnard
  • O. Tournilhac
  • O. Beyne-Rauzy
  • P. Agapé
  • A. Delmer
  • D. Ranta
  • P. Y. Jeandel
  • S. Georgin-Lavialle
  • L. Frenzel
  • G. Damaj
  • V. Eder
  • O. Lortholary
  • O. Hermine
  • O. Fain
  • M. SoussanEmail author
Original Article

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.

Keywords

FDG PET/CT Systemic mastocytosis 

Notes

Acknowledgments

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

Compliance with ethical standards

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.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • S. Djelbani-Ahmed
    • 1
    • 2
  • M. O. Chandesris
    • 3
    • 4
    • 5
  • A. Mekinian
    • 6
  • D. Canioni
    • 3
    • 5
    • 7
  • C. Brouzes
    • 3
    • 5
    • 8
  • K. Hanssens
    • 3
    • 9
  • G. Pop
    • 1
  • I. Durieu
    • 10
  • S. Durupt
    • 10
  • B. Grosbois
    • 11
  • S. Besnard
    • 11
  • O. Tournilhac
    • 12
  • O. Beyne-Rauzy
    • 13
  • P. Agapé
    • 14
  • A. Delmer
    • 15
  • D. Ranta
    • 16
  • P. Y. Jeandel
    • 17
  • S. Georgin-Lavialle
    • 18
  • L. Frenzel
    • 3
    • 4
    • 5
  • G. Damaj
    • 3
    • 19
  • V. Eder
    • 1
  • O. Lortholary
    • 3
    • 5
    • 20
  • O. Hermine
    • 3
    • 4
    • 5
  • O. Fain
    • 6
  • M. Soussan
    • 1
    • 2
    Email author
  1. 1.Department of Nuclear Medicine, Avicenne HospitalAssistance Publique - Hôpitaux de Paris (APHP)BobignyFrance
  2. 2.Sorbonne Paris CitéParis 13 UniversityBobignyFrance
  3. 3.French Reference center for Mastocytosis (Centre de Référence des Mastocytoses, CEREMAST)Necker Children’s Hospital, APHPParisFrance
  4. 4.Department of HaematologyNecker Children’s Hospital, APHPParisFrance
  5. 5.Sorbonne Paris Cité, Imagine InstituteParis Descartes UniversityParisFrance
  6. 6.Department of Internal Medicine and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), AP-HPSaint Antoine HospitalParisFrance
  7. 7.Department of PathologyNecker Children’s Hospital, APHPParisFrance
  8. 8.Laboratory of HaematologyNecker Children’s Hospital, APHPParisFrance
  9. 9.INSERM U1068, Centre de Recherche en Cancérologie de Marseille (Signaling, Hematopoiesis and Mechanism of Oncogenesis), Paoli Calmettes InstituteAix-Marseille UniversityMarseilleFrance
  10. 10.Department of Internal and Vascular Medicine, Hospices Civils de Lyon, Groupe Hopitalier SudUniversité de LyonPierre-BéniteFrance
  11. 11.Department of Internal MedicineRennes University HospitalRennesFrance
  12. 12.Department of Internal MedicineClermont-Ferrand University HospitalClermont-FerrandFrance
  13. 13.Department of Internal MedicinePurpan University HospitalToulouseFrance
  14. 14.Department of Oncology and HaematologySaint-Denis University HospitalSaint-Denis de la RéunionFrance
  15. 15.Department of HaematologyReims University HospitalReimsFrance
  16. 16.Department of HaematologyBrabois University HospitalVandoeuvre les NancyFrance
  17. 17.Department of Internal MedicineNice University HospitalNiceFrance
  18. 18.Department of Internal MedicineTenon HospitalParisFrance
  19. 19.Department of HaematologyCaen University HospitalCaenFrance
  20. 20.Department of Infectious Diseases and Tropical Medicine, Necker Children’s Hospital, APHPPasteur InstituteParisFrance

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