Clinical spectrum of primary adrenal lymphoma: results of a multicenter cohort study

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Clinical spectrum of primary adrenal lymphoma : results of a multicenter cohort study. / Majidi, Fatemeh; Martino, Samuela; Kondakci, Mustafa; Antke, Christina ; Haase, Matthias; Chortis, Vasileios; Arlt, Wiebke; Ronchi, Cristina; Fassnacht, Martin; Laurent, Claire; Petit, Jean-Michel; Casasnovas, Olivier; Habra, Mouhammed; Kanji, Aleem; Salvatori, Roberto; Ho, An Thi Nhat; Spyroglou, Adriadni; Beuschlein, Felix; Villa, Diego; Limvorapitak, Wasithep; Engelbrekt Wahlin, Bjorn; Gimm, Oliver ; Rudelius, Martina ; Schott, Matthias; Germing, Ulrich; Haas, Rainer; Gattermann, Norbert.

In: European Journal of Endocrinology, Vol. 183, No. 4, 10.2020, p. 453–462.

Research output: Contribution to journalArticlepeer-review

Harvard

Majidi, F, Martino, S, Kondakci, M, Antke, C, Haase, M, Chortis, V, Arlt, W, Ronchi, C, Fassnacht, M, Laurent, C, Petit, J-M, Casasnovas, O, Habra, M, Kanji, A, Salvatori, R, Ho, ATN, Spyroglou, A, Beuschlein, F, Villa, D, Limvorapitak, W, Engelbrekt Wahlin, B, Gimm, O, Rudelius, M, Schott, M, Germing, U, Haas, R & Gattermann, N 2020, 'Clinical spectrum of primary adrenal lymphoma: results of a multicenter cohort study', European Journal of Endocrinology, vol. 183, no. 4, pp. 453–462. https://doi.org/10.1530/EJE-19-0506

APA

Majidi, F., Martino, S., Kondakci, M., Antke, C., Haase, M., Chortis, V., Arlt, W., Ronchi, C., Fassnacht, M., Laurent, C., Petit, J-M., Casasnovas, O., Habra, M., Kanji, A., Salvatori, R., Ho, A. T. N., Spyroglou, A., Beuschlein, F., Villa, D., ... Gattermann, N. (2020). Clinical spectrum of primary adrenal lymphoma: results of a multicenter cohort study. European Journal of Endocrinology, 183(4), 453–462. https://doi.org/10.1530/EJE-19-0506

Vancouver

Author

Majidi, Fatemeh ; Martino, Samuela ; Kondakci, Mustafa ; Antke, Christina ; Haase, Matthias ; Chortis, Vasileios ; Arlt, Wiebke ; Ronchi, Cristina ; Fassnacht, Martin ; Laurent, Claire ; Petit, Jean-Michel ; Casasnovas, Olivier ; Habra, Mouhammed ; Kanji, Aleem ; Salvatori, Roberto ; Ho, An Thi Nhat ; Spyroglou, Adriadni ; Beuschlein, Felix ; Villa, Diego ; Limvorapitak, Wasithep ; Engelbrekt Wahlin, Bjorn ; Gimm, Oliver ; Rudelius, Martina ; Schott, Matthias ; Germing, Ulrich ; Haas, Rainer ; Gattermann, Norbert. / Clinical spectrum of primary adrenal lymphoma : results of a multicenter cohort study. In: European Journal of Endocrinology. 2020 ; Vol. 183, No. 4. pp. 453–462.

Bibtex

@article{e34008cd7bc94d4eb7c6f36a07e9f31f,
title = "Clinical spectrum of primary adrenal lymphoma: results of a multicenter cohort study",
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: 97 patients from 14 centers in Europe, Canada and the United States were included in this retrospective analysis between 1994 and 2017.Results: Of 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 versus 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.",
author = "Fatemeh Majidi and Samuela Martino and Mustafa Kondakci and Christina Antke and Matthias Haase and Vasileios Chortis and Wiebke Arlt and Cristina Ronchi and Martin Fassnacht and Claire Laurent and Jean-Michel Petit and Olivier Casasnovas and Mouhammed Habra and Aleem Kanji and Roberto Salvatori and Ho, {An Thi Nhat} and Adriadni Spyroglou and Felix Beuschlein and Diego Villa and Wasithep Limvorapitak and {Engelbrekt Wahlin}, Bjorn and Oliver Gimm and Martina Rudelius and Matthias Schott and Ulrich Germing and Rainer Haas and Norbert Gattermann",
year = "2020",
month = oct,
doi = "10.1530/EJE-19-0506",
language = "English",
volume = "183",
pages = "453–462",
journal = "European Journal of Endocrinology",
issn = "0804-4643",
publisher = "BioScientifica",
number = "4",

}

RIS

TY - JOUR

T1 - Clinical spectrum of primary adrenal lymphoma

T2 - results of a multicenter cohort study

AU - Majidi, Fatemeh

AU - Martino, Samuela

AU - Kondakci, Mustafa

AU - Antke, Christina

AU - Haase, Matthias

AU - Chortis, Vasileios

AU - Arlt, Wiebke

AU - Ronchi, Cristina

AU - Fassnacht, Martin

AU - Laurent, Claire

AU - Petit, Jean-Michel

AU - Casasnovas, Olivier

AU - Habra, Mouhammed

AU - Kanji, Aleem

AU - Salvatori, Roberto

AU - Ho, An Thi Nhat

AU - Spyroglou, Adriadni

AU - Beuschlein, Felix

AU - Villa, Diego

AU - Limvorapitak, Wasithep

AU - Engelbrekt Wahlin, Bjorn

AU - Gimm, Oliver

AU - Rudelius, Martina

AU - Schott, Matthias

AU - Germing, Ulrich

AU - Haas, Rainer

AU - Gattermann, Norbert

PY - 2020/10

Y1 - 2020/10

N2 - 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: 97 patients from 14 centers in Europe, Canada and the United States were included in this retrospective analysis between 1994 and 2017.Results: Of 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 versus 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.

AB - 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: 97 patients from 14 centers in Europe, Canada and the United States were included in this retrospective analysis between 1994 and 2017.Results: Of 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 versus 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.

UR - http://www.scopus.com/inward/record.url?scp=85086868776&partnerID=8YFLogxK

U2 - 10.1530/EJE-19-0506

DO - 10.1530/EJE-19-0506

M3 - Article

VL - 183

SP - 453

EP - 462

JO - European Journal of Endocrinology

JF - European Journal of Endocrinology

SN - 0804-4643

IS - 4

ER -