Vol 70, No 4 (2019)
Case report
Published online: 2018-10-25

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Capecitabine plus temozolomide in well- or moderately-differentiated primary atypical neuroendocrine tumours — single-centre experience of two cases

Anna La Salvia1, Maria Pia Brizzi2, Leonardo Muratori1, Elena Trevisi1, Massimo Di Maio2, Giorgio Vittorio Scagliotti1
Pubmed: 30359462
Endokrynol Pol 2019;70(4):380-383.


Introduction: Neuroendocrine neoplasms (NENs) are a rare and heterogeneous group of tumours, with a variety of primary origins and variable aggressiveness. NENs with an atypical primary origin, such as breast and retroperitoneal NENs, are extremely rare. As a consequence, an established diagnostic and therapeutic strategy in this particular subgroup is lacking. The combination of capecitabine and temozolomide, called CAPTEM regimen, has produced promising response rates in patients with grade 1 or 2 neuroendocrine tumours of multiple origins.

Case presentation: The first is a case of a 68-year-old woman with a metastatic primary breast neuroendocrine tumour, treated with cisplatin plus etoposide as first line, followed by CAV scheme (cyclophosphamide, doxorubicin, and vincristine), and subsequently treated, in third line with the CAPTEM regimen, obtaining radiological response and good tolerance. The second is the case of a 66-year-old woman affected by a metastatic primitive retroperitoneal NET G2. The patient progressed after a somatostatin analogue-based first line, whereas the CAPTEM regimen led to a partial and durable response with a favourable safety profile.

Conclusions: CAPTEM chemotherapy has been shown to be an active and safe therapeutic option in advanced, metastatic G1/2 atypical primary NENs.

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  1. Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003; 97(4): 934–959.
  2. López-Bonet E, Alonso-Ruano M, Barraza G. Solid neuroendocrine breast carcinomas: incidence, clinico-pathological features and immunohistochemical profiling. Oncol Rep. 2008; 20(6): 1369–1374.
  3. Dehal A, Kim S, Ali A. Primary Epithelial Neuroendocrine Tumors of the Retroperitoneum. Perm J. 2015; 19(4): 71–75.
  4. Kotteas EA, Syrigos KN, Saif MW. Profile of capecitabine/temozolomide combination in the treatment of well-differentiated neuroendocrine tumors. Onco Targets Ther. 2016; 9: 699–704.
  5. Ramirez RA, Beyer DT, Chauhan A. The Role of Capecitabine/Temozolomide in Metastatic Neuroendocrine Tumors. Oncologist. 2016; 21(6): 671–675.
  6. Abbasi S, Kashashna A, Albaba H. Efficacy of capecitabine and temozolomide combination in well-differentiated neuroendocrine tumors: Jordan experience. Pancreas. 2014; 43(8): 1303–1305.
  7. Fine RL, Gulati AP, Krantz BA. Capecitabine and temozolomide (CAPTEM) for metastatic, well-differentiated neuroendocrine cancers: The Pancreas Center at Columbia University experience. Cancer Chemother Pharmacol. 2013; 71(3): 663–670.
  8. Saranga-Perry V, Morse B, Centeno B. Treatment of metastatic neuroendocrine tumors of the thymus with capecitabine and temozolomide: a case series. Neuroendocrinology. 2013; 97(4): 318–321.
  9. Strosberg JR, Fine RL, Choi J. First-line chemotherapy with capecitabine and temozolomide in patients with metastatic pancreatic endocrine carcinomas. Cancer. 2011; 117(2): 268–275.
  10. Crespo G, Jiménez-Fonseca P, Custodio A. Capecitabine and temozolomide in grade 1/2 neuroendocrine tumors: a Spanish multicenter experience. Future Oncol. 2017; 13(7): 615–624.
  11. Uri I, Avniel-Polak S, Gross DJ. Update in the Therapy of Advanced Neuroendocrine Tumors. Curr Treat Options Oncol. 2017; 18(12): 72.
  12. Angelousi A, Kaltsas G, Koumarianou A. Chemotherapy in NETs: When and how. Rev Endocr Metab Disord. 2017; 18(4): 485–497.
  13. Campana D, Walter T, Pusceddu S, et al. Correlation between MGMT promoter methylation and response to temozolomide-based therapy in neuroendocrine neoplasms: an observational retrospective multicenter study. Endocrine. 2018; 60(3): 490–498.
  14. Murakami J, Lee YJ, Kokeguchi S. Depletion of O6-methylguanine-DNA methyltransferase by O6-benzylguanine enhances 5-FU cytotoxicity in colon and oral cancer cell lines. Oncol Rep. 2007; 17(6): 1461–1467.
  15. Kunz P, Catalano P, Nimeiri H, et al. A randomized study of temozolomide or temozolomide and capecitabine in patients with advanced pancreatic neuroendocrine tumors: A trial of the ECOG-ACRIN Cancer Research Group (E2211). Journal of Clinical Oncology. 2018; 36(15_suppl): 4004–4004.
  16. Zhu Y, Li Q, Gao J. Clinical features and treatment response of solid neuroendocrine breast carcinoma to adjuvant chemotherapy and endocrine therapy. Breast J. 2013; 19(4): 382–387.
  17. Wang J, Wei B, Albarracin CT, et al. Invasive neuroendocrine carcinoma of the breast: a population-based study from the surveillance, epidemiology and end results (SEER) database. BMC Cancer. 2014; 14: 147.
  18. Steinhof-Radwańska K, Barczyk A, Powązka P, et al. Primary neuroendocrine carcinoma of the breast — the report of four cases. Endokrynol Pol. 2017 [Epub ahead of print]; 68 (5): 597–602.
  19. Stupp R, Mason WP, van den Bent MJ. Radiotherapy plus concomitant and adjuvant temozolamide for glioblastoma. N Engl J Med. 2005; 352: 987–996.