TY  - JOUR
A1  - Canu, Letizia
A1  - Puglisi, Soraya
A1  - Berchialla, Paola
A1  - De Filpo, Giuseppina
A1  - Brignardello, Francesca
A1  - Schiavi, Francesca
A1  - Ferrara, Alfonso Massimiliano
A1  - Zovato, Stefania
A1  - Luconi, Michaela
A1  - Pia, Anna
A1  - Appetecchia, Marialuisa
A1  - Arvat, Emanuela
A1  - Letizia, Claudio
A1  - Maccario, Mauro
A1  - Parasiliti-Caprino, Mirko
A1  - Altieri, Barbara
A1  - Faggiano, Antongiulio
A1  - Modica, Roberta
A1  - Morelli, Valentina
A1  - Arosio, Maura
A1  - Verga, Uberta
A1  - Pellegrino, Micaela
A1  - Petramala, Luigi
A1  - Concistrè, Antonio
A1  - Razzore, Paola
A1  - Ercolino, Tonino
A1  - Rapizzi, Elena
A1  - Maggi, Mario
A1  - Stigliano, Antonio
A1  - Burrello, Jacopo
A1  - Terzolo, Massimo
A1  - Opocher, Giuseppe
A1  - Mannelli, Massimo
A1  - Reimondo, Giuseppe
T1  - A multicenter epidemiological study on second malignancy in non-syndromic pheochromocytoma/paraganglioma patients in Italy
JF  - Cancers
N2  - No studies have carried out an extensive analysis of the possible association between non-syndromic pheochromocytomas and paragangliomas (PPGLs) and other malignancies. To assess >the risk of additional malignancy in PPGL, we retrospectively evaluated 741 patients with PPGLs followed-up in twelve referral centers in Italy. Incidence of second malignant tumors was compared between this cohort and Italian patients with two subsequent malignancies. Among our patients, 95 (12.8%) developed a second malignant tumor, which were mainly prostate, colorectal and lung/bronchial cancers in males, breast cancer, differentiated thyroid cancer and melanoma in females. The standardized incidence ratio was 9.59 (95% CI 5.46–15.71) in males and 13.21 (95% CI 7.52–21.63) in females. At multivariable analysis, the risk of developing a second malignant tumor increased with age at diagnosis (HR 2.50, 95% CI 1.15–5.44, p = 0.021 for 50–59 vs. <50-year category; HR 3.46, 95% CI 1.67–7.15, p < 0.001 for >60- vs. <50-year). In patients with available genetic evaluation, a positive genetic test was inversely associated with the risk of developing a second tumor (HR 0.25, 95% CI 0.10–0.63, p = 0.003). In conclusion, PPGLs patients have higher incidence of additional malignant tumors compared to the general population who had a first malignancy, which could have an impact on the surveillance strategy.
KW  - pheochromocytoma
KW  - paraganglioma
KW  - epidemiology
KW  - genetic analysis
KW  - mortality
KW  - surveillance
Y1  - 2021
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-250148
SN  - 2072-6694
VL  - 13
IS  - 22
ER  - 
TY  - JOUR
A1  - Basile, Vittoria
A1  - Puglisi, Soraya
A1  - Altieri, Barbara
A1  - Canu, Letizia
A1  - Libè, Rossella
A1  - Ceccato, Filippo
A1  - Beuschlein, Felix
A1  - Quinkler, Marcus
A1  - Calabrese, Anna
A1  - Perotti, Paola
A1  - Berchialla, Paola
A1  - Dischinger, Ulrich
A1  - Megerle, Felix
A1  - Baudin, Eric
A1  - Bourdeau, Isabelle
A1  - Lacroix, André
A1  - Loli, Paola
A1  - Berruti, Alfredo
A1  - Kastelan, Darko
A1  - Haak, Harm R.
A1  - Fassnacht, Martin
A1  - Terzolo, Massimo
T1  - What is the optimal duration of adjuvant mitotane therapy in adrenocortical carcinoma? An unanswered question
JF  - Journal of Personalized Medicine
N2  - A relevant issue on the treatment of adrenocortical carcinoma (ACC) concerns the optimal duration of adjuvant mitotane treatment. We tried to address this question, assessing whether a correlation exists between the duration of adjuvant mitotane treatment and recurrence-free survival (RFS) of patients with ACC. We conducted a multicenter retrospective analysis on 154 ACC patients treated for ≥12 months with adjuvant mitotane after radical surgery and who were free of disease at the mitotane stop. During a median follow-up of 38 months, 19 patients (12.3%) experienced recurrence. We calculated the RFS after mitotane (RFSAM), from the landmark time-point of mitotane discontinuation, to overcome immortal time bias. We found a wide variability in the duration of adjuvant mitotane treatment among different centers and also among patients cared for at the same center, reflecting heterogeneous practice. We did not find any survival advantage in patients treated for longer than 24 months. Moreover, the relationship between treatment duration and the frequency of ACC recurrence was not linear after stratifying our patients in tertiles of length of adjuvant treatment. In conclusion, the present findings do not support the concept that extending adjuvant mitotane treatment over two years is beneficial for ACC patients with low to moderate risk of recurrence.
KW  - mitotane
KW  - adjuvant treatment
KW  - adrenocortical cancer
KW  - recurrence
KW  - recurrence free survival
KW  - timing
Y1  - 2021
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-236507
SN  - 2075-4426
VL  - 11
IS  - 4
ER  -