Searchable abstracts of presentations at key conferences in endocrinology

ea0049ep179 | Endocrine tumours and neoplasia | ECE2017

Serum succinate: investigation of its putative role as a new biomarker in malignant SDH-x mutated pheochromocytoma-paraganglioma patients?

Lamy Constance , Hadoux Julien , Mercier Lionel , Bailleux Dorian , Hescot Segolene , Paci Angelo , Baudin Eric , Broutin Sophie

Introduction: Malignant paraganglioma/pheochromocytoma (MPP) are very rare neuroendocrine tumors with heterogeneous prognostic and no gold-standard treatment. MPP can be associated with germline mutations at SDH-x genes which encode for the succinate dehydrogenase that catalyzes the oxidation of succinate to fumarate. SDH-x mutations lead to inactivation of the enzyme and thus accumulation of succinate.Aim: This project aims to evaluate...

ea0022oc4.4 | Adrenals | ECE2010

Combined anticortisolic therapy by metyrapone, ketoconazole and mitotane: an alternative to adrenalectomy in severe Cushing’s syndrome

Kamenicky Peter , Droumaguet Celine , Baudin Eric , Salenave Sylvie , Trabado Severine , Cazabat Laure , Chanson Philippe , Young Jacques

Context: Mitotane due to its adrenolytic action is highly effective in long-term management of ACTH-dependent Cushing’s syndrome (CS). However, the slow onset of its anticortisolic effect makes its use problematic in severe CS, when very rapid therapeutic response is required. Association with metyrapone and ketoconazole, rapidly acting steroidogenesis inhibitors, could warrant CS control while waiting for the full efficiency of mitotane and thus avoid urgently performed ...

ea0032p516 | Endocrine tumours and neoplasia | ECE2013

Comparison of two mitotane starting dose regimens in patients with advanced adrenocortical carcinoma

Kerkhofs Thomas , Baudin Eric , Terzolo Massimo , Allolio Bruno , Chadarevian Rita , Leboulleux Sophie , Mantero Franco , Haak Harm , Fassnacht Martin

Introduction: Current medical treatment of adrenocortical carcinoma (ACC) is based on mitotane alone or in combination with cytotoxic chemotherapy. However, very little is known about the pharmacokinetic properties of mitotane and dosing schedules are based on clinical experience only. The aim of this study was to investigate the relationship between mitotane dose and plasma concentration comparing two pre-defined treatment regimens. Secondary objectives were to evaluate safet...

ea0026oc2.1 | Neuroendocrinology | ECE2011

Etoposide, doxorubicin, cisplatin, and mitotane versus streptozotocin and mitotane in adrenocortical carcinoma: preliminary results from the first international phase III trial: the FIRM-ACT study

Fassnacht Martin , Terzolo Massimo , Allolio Bruno , Baudin Eric , Haak Harm , Berruti Alfredo , Mueller Hans-Helge , Skogseid Britt

Background: No randomized trials have been conducted in adrenocortical carcinoma (ACC) patients. Treatment recommendations for this rare but aggressive disease have been based on data from small phase II trials. We have now performed the first randomized phase III trial, comparing etoposide, doxorubicin, cisplatin, plus mitotane (EDP-M) against streptozotocin plus mitotane (Sz-M).Methods: Three-hundred and four chemotherapy-naive patients with ACC not am...

ea0022p418 | Endocrine tumours &amp; neoplasia (<emphasis role="italic">Generously supported by Novartis</emphasis>) | ECE2010

Adrenocortical carcinoma: is the surgical approach a risk factor of peritoneal carcinomatosis?

Leboulleux Sophie , Deandreis Desiree , Ghuzlan Abir Al , Auperin Anne , Dromain Clarisse , Young Jacques , Schlumberger Martin , Baudin Eric

Context: Peritoneal carcinomatosis (PC) is a rare site of distant metastasis in patients with adrenocortical cancer (ACC). One preliminary study suggests an increase risk of PC after adrenalectomy through laparoscopic approach of ACC.Objective: The objective of the study was to search for risk factors of PC including surgical approach. This was a retrospective cohort study conducted in an institutional practice.Patients: Sixty-four...

ea0063p826 | Adrenal and Neuroendocrine Tumours 3 | ECE2019

The French COMETE-Cancer network for adrenal cancer: 10 years of activity as part of a national plan for clinical care of rare cancers

Libe Rossella , Tabarin Antoine , Chabre Olivier , Laboureau Sandrine , Goichot Bernard , Vezzosi Delphine , Lefebvre Herve , Verges Bruno , Niccoli Patricia , Vanthyghem Marie-Christine , Baudin Eric , Bertherat Jerome

Introduction: The French National Institute of Cancer (INCa) launched supported by the Ministry of Health in 2008 a program for the recognition of national networks for the management of rare cancers. Among the 23 selected networks COMETE-Cancer was recognized by INCa in 2009 for Adrenocortical carcinoma (ACC) and malignant pheochromocytoma/paraganglioma (MPP). At that time 60–120 new ACC/year and 30 new MPP/year were expected at the national level....

ea0032p544 | Endocrine tumours and neoplasia | ECE2013

Multimodal combination of interferon and loco-regional treatment for disease control in progressive metastatic pheochromocytoma/paraganglioma patients

Hadoux Julien , Deandreis Desiree , Caramella Caroline , Leboulleux Sophie , Al Ghuzlan Abir , Chougnet Cecile , Dumont Frederic , Deschamps Frederic , Schlumberger Martin , Baudin Eric

Interferon-α (IFN-α) has shown some activity in neuroendocrine tumors with disease stabilizations. Malignant pheochromocytoma and paraganglioma (MPPGLs) have a heterogeneous behavior with a slow progression rate, most of the time and a high frequency of bone metastases. Stabilizing disease and preventing skeletal-related events are two goals to achieve in the management of MPPGLs patients.This retrospective study evaluated a multimodal strategy...

ea0026p27 | Adrenal cortex | ECE2011

Do mitotane levels impact on the outcome of patients treated adjuvantly following radical resection of adrenocortical cancer (ACC)?

Ardito A , Al Ghuzian A , Fassnacht M , Daffara F , Leboulleux S , Wortmann S , Zaggia B , De Francia S , Volante Marco , Gonzaga Luigi , Berruti Alfredo , Allolio Bruno , Baudin Eric , Terzolo Massimo

We have demonstrated that adjuvant mitotane prolong recurrence free survival (RFS) in patients with radically resected ACC. Aim of the present study was to correlate mitotane levels with patient outcome in an adjuvant setting in 3 different referral centres in Europe. There were 120 patients (45 W, 75 M, median age 44 years, range 16–76) radically resected for ACC who were treated adjuvantly with mitotane from 1996 to 2010. ACC was stage I in 10 cases, II in 73, III in 31...

ea0022p29 | Adrenal | ECE2010

Benefit to risk ratio of mitotane high starting dose strategy in 22 patients with adrenocortical carcinoma (ACC): a prospective evaluation

Mauclere-Denost Sophie , Leboulleux Sophie , Borget Isabelle , Al Ghuzlan Abir , Young Jacques , Drouard Laurence , Paci Angelo , Chanson Philippe , Schlumberger Martin , Baudin Eric

Background: The benefit to risk ratio of mitotane high starting dose regimen in ACC remains unknown.Methods: To evaluate a high starting dose strategy, we performed a single-center, prospective study with three main objectives: 1) to assess the time taken to reach a mitotane plasma level above 14 mg/l (benefit); 2) to evaluate mitotane tolerance (risk) in the first 3 months of treatment and 3) to analyse the correlation using a regression analysis test b...

ea0022p424 | Endocrine tumours &amp; neoplasia (<emphasis role="italic">Generously supported by Novartis</emphasis>) | ECE2010

Serum concentration of o,p’DDD (mitotane), o,p’DDA and o,p’DDE as predictors of tumour response in adrenocortical carcinoma: results of a retrospective European Network for the Study of Adrenal Tumors (ENS@T) multicentre study

Hermsen Ilse , Fassnacht Martin , Terzolo Massimo , Houterman Saskia , Hartigh Jan den , Leboulleux Sophie , Daffara Fulvia , Allolio Bruno , Berruti Alfredo , Chadarevian Rita , Haak Harm , Baudin Eric

Introduction: O,p’DDD is the drug of choice for patients with adrenocortical carcinoma (ACC). Monitoring o,p’DDD serum level has been proposed as predictor of tumour response. Measurement of o,p’DDD metabolites involved in the active pathway may provide an improved prediction of o,p’DDD activity. The objective of our study was to (1)to confirm the currently used threshold o,p’DDD serum level of 14mg/l for achieving a tumour response and (2)compare the ...