Fatemeh Majidi1, Samuela Martino1, Mustafa Kondakci1, Christina Antke2, Matthias Haase3, Vasileios Chortis4,5, Wiebke Arlt4,5, Cristina L Ronchi5,6, Martin Fassnacht6,7, Claire Laurent8, Jean-Michel Petit8, Olivier Casasnovas9, Mouhammed Amir Habra10, Aleem Kanji11, Roberto Salvatori12, An Thi Nhat Ho13, Ariadni Spyroglou14, Felix Beuschlein14,15, Diego Villa16, Wasithep Limvorapitak16,17, Björn Engelbrekt Wahlin18, Oliver Gimm19, Martina Rudelius20, Matthias Schott3, Ulrich Germing1, Rainer Haas1, Norbert Gattermann1. 1. Department of Hematology, Oncology and Clinical Immunology, Heinrich Heine University Düsseldorf, Düsseldorf, Germany. 2. Klinik für Nuklearmedizin, Heinrich Heine Universität Düsseldorf, Düsseldorf, Germany. 3. Department of Endocrinology, Heinrich Heine University Düsseldorf, Düsseldorf, Germany. 4. Center for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK. 5. Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK. 6. Divison of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital, University of Würzburg, Würzburg, Germany. 7. Comprehensive Cancer Center Mainfranken, University of Würzburg, Würzburg, Germany. 8. University Hospital of Dijon, Dijon, France. 9. Department of Hematology, University Hospital of Djion, Dijon, France. 10. Department of Endocrine Neoplasia and Hormonal Disorders, MD Anderson Cancer Center, Houston, Texas, USA. 11. Baylor College of Medicine, Houston, Texas, USA. 12. Division of Endocrinology, Diabetes and Metabolism, Johns Hopkins University, Baltimore, Maryland, USA. 13. Department of Medicine, Medstar Harbor Hospital, Baltimore, Maryland, USA. 14. Klinik für Endokrinologie und Klinische Ernährung, Universitätsspital Zürich, Zürich, Switzerland. 15. Klinik und Poliklinik IV, Klinikum der Universität München, Ludweig-Maximilians-Universität München, Munich, Germany. 16. BC Cancer Centre for Lymphoid Cancer and University of British Columbia, Vancouver, Canada. 17. Division of Hematology, Department of Internal Medicine, Thammasat University, Pathumthani, Thailand. 18. Department of Medicine, Unit of Hematology, Karolinska Institute, Stockholm, Sweden. 19. Departments of Surgery, and Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden. 20. Institute of Pathology, Ludweig-Maximilians-Universität München, München, Germany.
Abstract
Purpose: We sought to refine the clinical picture of primary adrenal lymphoma (PAL), a rare lymphoid malignancy with predominant adrenal manifestation and risk of adrenal insufficiency. Methods: Ninety-seven patients from 14 centers in Europe, Canada and the United States were included in this retrospective analysis between 1994 and 2017. Results: Of the 81 patients with imaging data, 19 (23%) had isolated adrenal involvement (iPAL), while 62 (77%) had additional extra-adrenal involvement (PAL+). Among patients who had both CT and PET scans, 18FDG-PET revealed extra-adrenal involvement not detected by CT scan in 9/18 cases (50%). The most common clinical manifestations were B symptoms (55%), fatigue (45%), and abdominal pain (35%). Endocrinological assessment was often inadequate. With a median follow-up of 41.6 months, 3-year progression-free (PFS) and overall (OS) survival rates in the entire cohort were 35.5% and 39.4%, respectively. The hazard ratios of iPAL for PFS and OS were 40.1 (95% CI: 2.63-613.7, P = 0.008) and 2.69 (95% CI: 0.61-11.89, P = 0.191), respectively. PFS was much shorter in iPAL vs PAL+ (median 4 months vs not reached, P = 0.006), and OS also appeared to be shorter (median 16 months vs not reached), but the difference did not reach statistical significance (P = 0.16). Isolated PAL was more frequent in females (OR = 3.81; P = 0.01) and less frequently associated with B symptoms (OR = 0.159; P = 0.004). Conclusion: We found unexpected heterogeneity in the clinical spectrum of PAL. Further studies are needed to clarify whether clinical distinction between iPAL and PAL+ is corroborated by differences in molecular biology.
Purpose: We sought to refine the clinical picture of primary adrenal lymphoma (PAL), a rare lymphoid malignancy with predominant adrenal manifestation and risk of adrenal insufficiency. Methods: Ninety-seven patients from 14 centers in Europe, Canada and the United States were included in this retrospective analysis between 1994 and 2017. Results: Of the 81 patients with imaging data, 19 (23%) had isolated adrenal involvement (iPAL), while 62 (77%) had additional extra-adrenal involvement (PAL+). Among patients who had both CT and PET scans, 18FDG-PET revealed extra-adrenal involvement not detected by CT scan in 9/18 cases (50%). The most common clinical manifestations were B symptoms (55%), fatigue (45%), and abdominal pain (35%). Endocrinological assessment was often inadequate. With a median follow-up of 41.6 months, 3-year progression-free (PFS) and overall (OS) survival rates in the entire cohort were 35.5% and 39.4%, respectively. The hazard ratios of iPAL for PFS and OS were 40.1 (95% CI: 2.63-613.7, P = 0.008) and 2.69 (95% CI: 0.61-11.89, P = 0.191), respectively. PFS was much shorter in iPAL vs PAL+ (median 4 months vs not reached, P = 0.006), and OS also appeared to be shorter (median 16 months vs not reached), but the difference did not reach statistical significance (P = 0.16). Isolated PAL was more frequent in females (OR = 3.81; P = 0.01) and less frequently associated with B symptoms (OR = 0.159; P = 0.004). Conclusion: We found unexpected heterogeneity in the clinical spectrum of PAL. Further studies are needed to clarify whether clinical distinction between iPAL and PAL+ is corroborated by differences in molecular biology.
Authors: F Ceccato; G Voltan; C Sabbadin; V Camozzi; I Merante Boschin; C Mian; V Zanotto; D Donato; G Bordignon; A Capizzi; G Carretta; C Scaroni Journal: J Endocrinol Invest Date: 2020-12-23 Impact factor: 4.256