Endocrine Abstracts

In the gastroentero-pancreatic tract, a spectrum of neuroendocrine tumors (NET) exists, including low grade tumors (carcinoid), intermediate grade malignant carcinoid, and high grade poorly differentiated carcinomas of the small and large cell types. In year 2000, the WHO presented a classification scheme for all NETs, but mostly applied to gastroentero-pancreatic tumors. This term carcinoid was replaced by (neuro)endocrine tumor, malignant carcinoid by well differentiated (ne...

Objective: In vitro data suggest that somatostatin receptors (SSTR) and mTOR pathways might represent reasonable targets for novel therapies, since are involved in growth of adrenocortical carcinoma (ACC). However, in vitro analysis of combination treatments, targeting both mTOR and SSTR as compared to mitotane, and detailed tissue typing mTOR/SSTR pathway are poorly explored in ACC.Methods: This study was designed to characterize the t...

Background and objectives: The role of adjuvant mitotane therapy after radical surgery for adrenocortical carcinoma (ACC) is still debated. The aim of our study is to assess the effects of adjuvant mitotane on recurrence free survival (RFS) and overall survival (OS) in non-metastatic ACC patients and to evaluate prognostic factors in adjuvant setting.Methods: Retrospective data were collected from 152 patients affected by ACC (stage I-III) referred to ou...

Background: Adrenocortical carcinoma (ACC) is a rare cancer associated with hereditary syndromes in 10% of cases. However, data on germline variants (GVs) in adult patients with sporadic ACC are limited.Methods: We analyzed germline DNA from 150 adult patients with sporadic ACC sequentially referred to our centers between 1998-2019. We designed a custom panel of 17 genes potentially involved in the pathogenesis of ACC: AIP, APC, ARMC5, ARNT, BRCA1, BRCA2...

Mitotane is the only approved drug for the treatment of advanced adrenocortical carcinoma (ACC) and we recently demonstrated that a high expression of cytochrome P450 2W1 (CYP2W1) correlated with response to mitotane. The association between CYP2W1 alleles and a generally increased cancer risk is under debate. Aim of the study was to evaluate the frequency of CYP2W1 polymorphisms and its correlation with the response to mitotane treatment in ACC patients.<p class="abstext"...

Parathyroid carcinoma (PC) is a rare neoplasia responsible for about 1% of primary hyperparathyroidism (PHPT). Differently from patients with its benign counterpart, the phenotype of these patients is characterized by severe PHPT and hypercalcemia. The aim of this study was to describe a series of PC casesrecorded in the regional cancer network of Piedmont and Valle d’Aosta, Italy (Rete Oncologica del Piemonte e della Valle d’Aosta) from 2007 to 2017, including 25 ...

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...

Objective: Various features have been identified as predictors of relapse after complete resection of pheochromocytoma, but a comprehensive multivariable model for recurrence risk prediction is lacking. The aim of this study was to develop and internally validate an integrated predictive model for post-surgical recurrence of pheochromocytoma.Methods: The present research retrospectively enrolled 177 patients affected by pheochromocytoma and submitted to ...

Adrenocortical carcinoma (ACC) is a rare tumor with poor prognosis and the only approved drug for advanced disease is mitotane. The cytochromes P450 (CYP) 2W1 and 2B6 are proposed predicting markers of sensitivity to mitotane treatment. Aim of the study was to evaluate the relationship between CYP2W1 and/or CYP2B6 polymorphisms and response to mitotane in ACC.Methods: We performed a multicentric retrospective study including 182 ACC patients (F/M=121/61)...

Background: The human cytochrome P450 (CYP) 2W1 is involved in the metabolism of several endogenous substrates, including lysophospholipids and fatty acids. Using a polyclonal antibody, we previously demonstrated a high CYP2W1 immunoreactivity in adrenocortical tumors, particularly in those secreting steroids. The aim of the study was to better elucidate the relationship between CYP2W1 and steroid secretion in adrenocortical carcinoma (ACC) H295R cell line and in a larger seri...