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Exp Clin Endocrinol Diabetes 2019; 127(09): 578-584
DOI: 10.1055/a-0747-5571
Article
© Georg Thieme Verlag KG Stuttgart · New York

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

Matthias Kroiss   *
1   Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany
,
Timo Deutschbein   *
1   Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany
,
Wiebke Schlötelburg
2   Department of Radiology, University Hospital Würzburg, University of Würzburg, Germany
,
Cristina Lucia Ronchi
1   Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany
,
Ségolène Hescot
3   Gustave Roussy, Université Paris Sud, France
,
Daniela Körbl
1   Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany
,
Felix Megerle
1   Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany
,
Felix Beuschlein
4   Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, Munich, Germany
5   Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, Universitätsspital Zürich, Zürich, Switzerland
,
Bruno Neu
6   Second Department of Medicine, Acedemic Teaching Hospital Landshut Achdorf, Germany
,
Marcus Quinkler
7   Endocrinology in Charlottenburg, Berlin, Germany
,
Eric Baudin
3   Gustave Roussy, Université Paris Sud, France
,
Stefanie Hahner
1   Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany
,
Anke Heidemeier
2   Department of Radiology, University Hospital Würzburg, University of Würzburg, Germany
,
Martin Fassnacht
1   Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Germany
8   Comprehensive Cancer Center Mainfranken, University of Würzburg, Germany
› Author Affiliations
Further Information

Publication History

received 12 July 2018
revised 20 August 2018

accepted 19 September 2018

Publication Date:
14 November 2018 (online)

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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

* M.K. and T.D. contributed equally to this work


 

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