Endocrine Abstracts

Pheochromocytomas and paragangliomas are rare neuroendocrine chromaffine tumors located in the adrenal or extra-adrenal sites, respectively. Malignant pheochromocytomas or paragangliomas represent 10–20% (MPP) of these patients and are defined by the presence or occurrence of metastasis, in non chromaffin organs. MPP are characterized by their heterogeneous presentation as testified by their variety in primary locations, levels and type of hormone secretions, percentage o...

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Medullary thyroid carcinoma (MTC) early spreads to cervical lymph nodes and to distant sites: mostly to the liver, lungs or bones. Unusual sites such as breast, skin or orbit have been also described. The aim of the study was to evaluate the frequency, localisation of unusual metastases of MTC and their impact on survival.Eligibility criteria for this study were the presence of a pathological confirmed diagnosis of MTC with distant metastases (other than...

Mitotane (o,p’DDD) represents the most effective treatment of adrenocortical carcinoma (ACC). However, molecular mechanisms of mitotane action remain poorly understood. Mitochondrial (mt) impact of mitotane has previously been suggested but not yet fully validated. We investigated functional consequences of mitotane exposure on mitochondrial steroidogenesis, respiratory chain activity and biogenesis, using human adrenocortical secreting H295R and non-secreting SW13 cells....

Background: Refractory hypoglycemia in patients with metastatic insulinomas (RHMI) is an important cause of morbidity and mortality and everolimus could be a new therapeutic option.Methods: We conducted a restrospective multicentric study within the French group of endocrine tumors to evaluate the time to first symptomatic recurrence (TSR) in patients with RHMI after everolimus initiation. Ongoing hyperglycemic medical options and safety were recorded.</...

Malignant pheochromocytoma and paraganglioma (PPGLs) are rare diseases with a heterogeneous behaviour. 131I-MIBG therapy and the cyclophosphamide-dacarbazine-vincristine chemotherapy regimen (CVD) constitute the most popular options in the metastatic setting. We have investigated the antitumor effect of temozolomide (TMZ), in patients with metastatic PPGLs. Efficacy was primary endpoint, safety and identification for prognosis factors of response were secondary endpoints.<...

Introduction: Adrenocortical carcinoma (ACC) is a rare tumor of the adrenals with poor prognosis (survival rate for metastatic patients <15% at 5 years). Mitotane (o,p′-DDD) is the main therapeutic option with up to 66% objective response rates in patients with serum levels between 14 and 20 mg/l. Its two main metabolites are o,p′-DDA and o,p′-DDE which plasma levels are not correlated to therapeutic response. Mitotane is a lipophilic drug that accumulate...

Background: Mitotane antitumor efficacy is related to plasma levels. Objective responses have been associated with mitotane plasma levels >14 mg/l. However, high plasma levels >20 or >30 mg/l are at higher risk of toxicity, especially neurotoxicity. National-based survey is lacking to evaluate the frequency and severity of high mitotane plasma levels. Our aim was to retrospectively describe serious adverse events (SAE).Methods: Mitotane plasm...

Pheochromocytoma and paraganglioma are defined as malignant by the occurence of metastasis in extra paraganglionic sites. The natural evolution of these tumors remains unknown.The aim of our retrospective study was to define the progression-free survival (PFS) of untreated Malignant Pheochromocytoma and Paraganglioma (MPP) at 12 months (12 m-PFS) and to look for prognostic markers of 12 m-PFS. We analyzed clinical parameters of patients followed within 8...

Objective: To assess outcome of stage III–ENSAT adrenocortical carcinoma (ACC) patients after complete macroscopic resection and prognostic factors for disease-free survival (DFS) and overall survival (OS).Methods: A retrospective review of 68 stage III ACC patients treated surgically between 1994 and 2009 in the French COMETE network was performed. Characteristics of patients were reviewed by a single investigator as well as the Weiss score by expe...