Oops, you're using an old version of your browser so some of the features on this page may not be displaying properly.

MINIMAL Requirements: Google Chrome 24+Mozilla Firefox 20+Internet Explorer 11Opera 15–18Apple Safari 7SeaMonkey 2.15-2.23

Poster display session

2330 - Activity of temozolomide (TMZ) in patients (PTS) with malignant pheochromocytoma or paraganglioma (MPP): a mono-institutional retrospective study


10 Sep 2017


Poster display session


Giuseppe Lombardi


Annals of Oncology (2017) 28 (suppl_5): v142-v157. 10.1093/annonc/mdx368


G. Lombardi1, M.A. Ferrara2, A. Pambuku1, M. Lorusso2, F. Boaretto2, F. Schiavi2, L. Biasini3, F. Azzolin2, G. Opocher4, S. Zovato2, V. Zagonel1

Author affiliations

  • 1 Clinical And Experimental Oncology, Medical Oncology 1, Veneto Institute of Oncology, 35128 - Padova/IT
  • 2 Familial Tumor Unit, Veneto Institute of Oncology, Padova/IT
  • 3 Immunology And Molecular Oncology Unit, Veneto Institute of Oncology, 35128 - Padova/IT
  • 4 Iov, Veneto Institute of Oncology, 35128 - Padova/IT


Abstract 2330


MPP are very rare neuroendocrine tumors and, currently, there is no standard chemotherapy for their treatment. TMZ showed some benefit in digestive neuroendocrine tumors. We investigate TMZ activity in PTS with MPP.


Retrospectively, we evaluated TMZ activity in MPP PTS treated at our oncological center, Veneto Institute of Oncology, from January 2015 to March 2017. The inclusion criteria were: pathological diagnosis of MPP, ECOG PS 0-2, no haemopoietic, renal and hepatic abnormalities. TMZ schedule was 150-200mg/m2 for 5 consecutive days every 28 days until progression disease or unacceptable toxicity. CT scan and urinary concentrations of metanephrines were performed every 12 wks; evaluation of tumor response was performed according to RECIST 1.1 criteria. Germinal mutational analysis of the genes of susceptibility to pheochromocytoma/paraganglioma (SDHx, MAX, TMEM127, RET, VHL, FH) was performed. Aberrant hypermethylation of MGMT promoter was analyzed on DNA obtained from surgical tissue. Median OS and PFS were estimated by the Kaplan-Meier method. Toxicity was evaluated by CTCAE v.4.


We enrolled 12 consecutive PTS; 7 were males; ECOG PS was 1 and 2 in 9 and 3 PTS. MAX gene was mutated in 1 PT. SDHB gene was mutated in 2 PTS. MGMT promoter was methylated in 1 patient. No other genetic mutations were found. 5 PTS were already treated with a prior chemotherapy (3 sunitinib, 1 capecitabine, 1 dacarbazine) and 4 with a prior MIBG. 9 PTS were evaluable for response: 2 PTS had a partial response, 5 stable disease, 2 progressive disease. Median follow up was 9.2ms (range 1.1- 28ms). 2 PTS received TMZ for more than 2 years and other 2 PTS for more than 1 year. Median PFS and OS were not reached (95% CI = 3.4ms-n.a.; 6.2ms-n. a, respectively). Urinary metanephrines levels seem to correlate with response. Hypertension descreased significantly in 5 PTS during TMZ treatment. No grade 3-4 toxicity was recorded.


TMZ is an active and safe treatment for MPP, regardless of previous treatment. A prospective phase II study is ongoing.

Clinical trial identification

Legal entity responsible for the study

Giuseppe Lombardi




All authors have declared no conflicts of interest.

This site uses cookies. Some of these cookies are essential, while others help us improve your experience by providing insights into how the site is being used.

For more detailed information on the cookies we use, please check our Privacy Policy.

Customise settings
  • Necessary cookies enable core functionality. The website cannot function properly without these cookies, and you can only disable them by changing your browser preferences.