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Sebastian Wortmann, Marcus Quinkler, Christian Ritter, Matthias Kroiss, Sarah Johanssen, Stefanie Hahner, Bruno Allolio, and Martin Fassnacht

Objective

No standard therapy for advanced adrenocortical carcinoma (ACC) is established by any randomized trial but a consensus conference 2003 recommended mitotane as monotherapy or combined with etoposide, doxorubicin and cisplatin or with streptozotocin as first-line systemic therapy. However, there is no evidence for any therapy beneficial in patients failing these therapies. Therefore, we evaluated the effects of the anti-VEGF antibody bevacizumab plus capecitabine as salvage therapy in ACC.

Methods

Patients registered with the German ACC Registry with refractory ACC progressing after cytotoxic therapies were offered treatment with bevacizumab (5 mg/kg body weight i.v. every 21 days) and oral capecitabine (950 mg/m2 twice daily for 14 days followed by 7 days of rest) in 2006–2008. Evaluation of tumour response was performed by imaging according to response evaluation criteria in solid tumours every 12 weeks.

Results

Ten patients were treated with bevacizumab plus capecitabine. None of them experienced any objective response or stable disease. Two patients had to stop therapy after few weeks due to hand-foot syndrome, and three patients died on progressive disease within 12 weeks. Other adverse events were mild (grade I–II). Median survival after treatment initiation was 124 days.

Conclusions

Bevacizumab plus capecitabine has no activity in patients with very advanced ACC. Hence, this regimen cannot be recommended as a salvage therapy.

Free access

Stephan Petersenn, Paul-Ajoy Richter, Thomas Broemel, Christian O Ritter, Timo Deutschbein, Frank-Ulrich Beil, Bruno Allolio, Martin Fassnacht, and for the German ACC Study Group

Objective

Thresholds of 2–20 hounsfield units (HU) in unenhanced computed tomography (CT) are suggested to discriminate benign adrenal tumors (BATs) from malignant adrenal tumors. However, these studies included only low numbers of adrenocortical carcinomas (ACCs). This study defines a HU threshold by inclusion of a large cohort of ACCs.

Design

Retrospective, blinded, comparative analysis of CT scans from 51 patients with ACCs (30 females, median age 49 years) and 25 patients with BATs (12 females, median age 64 years) diagnosed during the period of 2005–2010 was performed.

Methods

Tumor density was evaluated in unenhanced CT by two blinded investigators.

Results

Median tumor size was 9 cm (range 2.0–20) for ACCs vs 4 cm (2.0–7.5) for BATs (P<0.0001). In ACCs, the median unenhanced HU value was 34 (range 14–74) in comparison with 5 (−13 to 40) in BATs (P<0.0001). ROC analysis revealed a HU of 21 as threshold with the best diagnostic accuracy (sensitivity 96%, specificity 80%, and AUC 0.89). However, two ACCs that were 5 and 6 cm in size would have been missed. Setting the threshold to 13.9 allowed for 100% sensitivity, but a lower specificity of 68%.

Conclusions

This first large study on ACCs confirmed that the vast majority of ACCs have unenhanced HU >21. However, to avoid misdiagnosing an ACC as benign, a threshold of 13 should be used.