Treatment of Refractory Adrenocortical Carcinoma with Thalidomide: Analysis of 27 Patients from the European Network for the Study of Adrenal Tumours Registry

Matthias Kroiss; Timo Deutschbein; Wiebke Schlötelburg; Cristina Lucia Ronchi; Ségolène Hescot; Daniela Körbl; Felix Megerle; Felix Beuschlein; Bruno Neu; Marcus Quinkler; Eric Baudin; Stefanie Hahner; Anke Heidemeier; Martin Fassnacht

doi:10.1055/a-0747-5571

Experimental and Clinical Endocrinology & Diabetes, Table of Contents

Exp Clin Endocrinol Diabetes 2019; 127(09): 578-584
DOI: 10.1055/a-0747-5571

Article

Treatment of Refractory Adrenocortical Carcinoma with Thalidomide: Analysis of 27 Patients from the European Network for the Study of Adrenal Tumours Registry

Matthias Kroiss^*

¹Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany

,

Timo Deutschbein^*

¹Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany

,

Wiebke Schlötelburg

²Department of Radiology, University Hospital Würzburg, University of Würzburg, Germany

,

Cristina Lucia Ronchi

¹Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany

,

Ségolène Hescot

³Gustave Roussy, Université Paris Sud, France

,

Daniela Körbl

¹Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany

,

Felix Megerle

¹Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany

,

Felix Beuschlein

⁴Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, Munich, Germany

⁵Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, Universitätsspital Zürich, Zürich, Switzerland

,

Bruno Neu

⁶Second Department of Medicine, Acedemic Teaching Hospital Landshut Achdorf, Germany

,

Marcus Quinkler

⁷Endocrinology in Charlottenburg, Berlin, Germany

,

Eric Baudin

³Gustave Roussy, Université Paris Sud, France

,

Stefanie Hahner

¹Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany

,

Anke Heidemeier

²Department of Radiology, University Hospital Würzburg, University of Würzburg, Germany

,

Martin Fassnacht

¹Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany

⁸Comprehensive Cancer Center Mainfranken, University of Würzburg, Germany

› Author Affiliations

Abstract

Objective Adrenocortical carcinoma (ACC) is a rare malignancy with a dismal prognosis. In advanced stages, tumour control by mitotane and cytotoxic chemotherapy is often temporary and salvage treatments are warranted.

Methods Retrospective cohort study of participants in the prospective European Networks for the Study of Adrenal Tumours (ENSAT) registry. Main outcome measures were best response during treatment, progression-free survival (PFS), both measured according to RECIST 1.1 by two blinded radiologists, and overall survival (OS).

Results Twenty-seven patients (13 males; median age 44.1 years) progressing after mitotane and a median of 4 further systemic treatments were included. Thalidomide was administered as tolerated with a starting dose of 50 mg and target dose of 200 mg /d. The median interval between treatment initiation and first imaging was 10.5 (4.4-17.5) weeks. The best response to treatment was stable disease (SD, n=2) and progressive disease (n=25), with a median PFS of 11.2 weeks and a median OS of 36.4 weeks. The first patient with SD discontinued treatment due to mild epistaxis and diarrhea after 22.3 weeks. The second patient had SD at the second treatment evaluation after 25.2 weeks and continued thalidomide but then had clinical progression and deceased after 54.3 weeks. In general, thalidomide induced only mild or moderate adverse effects (mainly fatigue and gastrointestinal complaints).

Conclusion Thalidomide was overall well tolerated but resulted in disease control in only 2/27 (7.4%) patients. In the absence of predictive response markers, thalidomide should only be considered in exceptional cases as a salvage therapy in ACC.

Key words

adrenal - adverse events - cancer - efficacy - follow-up - outcome - progression - side-effects - staging - toxicity - treatment

Full Text

References

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