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Poster display session

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

Date

10 Sep 2017

Session

Poster display session

Presenters

Giuseppe Lombardi

Citation

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

Authors

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
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Resources

Abstract 2330

Background

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.

Methods

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.

Results

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.

Conclusions

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

Funding

None

Disclosure

All authors have declared no conflicts of interest.

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