@article{3183278, title = "Unmet Needs in Appendiceal Neuroendocrine Neoplasms", author = "Toumpanakis, Christos and Fazio, Nicola and Janson, Eva Tiensuu and and Horsch, Dieter and Pascher, Andreas and Reed, Nicholas and O'Toole, and Dermot and van Dijkum, Els Nieveen and Partelli, Stefano and Rinke, Anja and and Kos-Kudla, Beata and Costa, Frederico and Pape, Ulrich-Frank and and Grozinsky-Glasberg, Simona and Scoazec, Jean-Yves and Detlef, Bartsch and and Rudolf, Arnold and Eric, Baudin and Lisa, Bodei and Ivan, Borbath and and Jaume, Capdevila and Martyn, Caplin and Jie, Chen and Frederico, and Costa and Anne, Couvelard and Jaroslaw, Cwikla B. and de Herder, Wouter and W. and Massimo, Falconi and Jenny, Falkerby and Nicola, Fazio and Diego, and Ferone and Andrea, Frilling and Rocio, Garcia-Carbonero and Simona, and Glasberg and Vera, Gorbunova and Ashley, Grossman and Dieter, Hoersch and and Robert, Jensen and Gregory, Kaltsas and Guenter, Kloeppel and Peter, and Knigge Ulrich and Beata, Kos-Kudla and Guenter, Krejs J. and Eric, and Krenning and Matthew, Kulke and Steven, Lamberts W. J. and Elisabeth, and Nieveen van Dijkum and Juan Manuel, O'Connor and Dermot, O'Toole and and Ulrich-Frank, Pape and Stefano, Partelli and Ellen, Pavel Marianne and and Marc, Peeters and John, Ramage and Nicholas, Reed and Guido, Rindi and and Anja, Rinke and Philippe, Ruszniewski and Halfdan, Sorbye and Anders, and Sundin and Jean-Yves, Scoazec and Babs, Taal G. and Eva, Tiensuu Janson and and Christos, Toumpanakis and Juan, Valle and Marie-Pierre, Vullierme and and Staffan, Welin and Bertram, Wiedenmann and ENETS 2016 Munich and Advisory Board", journal = "ISRN Neuroendocrinology", year = "2019", volume = "108", number = "1", pages = "37-44", publisher = "Karger", issn = "2090-7982", doi = "10.1159/000493894", keywords = "Appendix; Carcinoids; Appendiceal neuroendocrine neoplasms; Appendicectomy; Right hemicolectomy", abstract = "Appendiceal neuroendocrine neoplasms (ANEN) are mostly discovered coincidentally during appendicectomy and usually have a benign clinical course; thus, appendicectomy alone is considered curative. However, in some cases, a malignant potential is suspected, and therefore additional operations such as completion right hemicolectomy are considered. The existing European Neuroendocrine Tumour Society (ENETS) guidelines provide useful data about epidemiology and prognosis, as well as practical recommendations with regards to the risk factors for a more aggressive disease course and the indications for a secondary operation. However, these guidelines are based on heterogeneous and retrospective studies. Therefore, the evidence does not seem to be robust, and there are still unmet needs in terms of accurate epidemiology and overall prognosis, optimal diagnostic and follow-up strategy, as well as identified risk factors that would indicate a more aggressive surgical approach at the beginning and a more intense follow-up. In this review, we are adopting a critical approach of the ENETS guidelines and published series for ANEN, focusing on the above-noted grey areas." }