Well-differentiated digestive neuroendocrine tumors (NET) are a heterogeneous group of neoplasms usually associated with slow growth but a high rate of metastases, including peritoneal carcinomatosis (PC). Herein, we aimed to comprehensively review the current knowledge of PC in terms of implications for the management and prognosis of patients with NET, including the latest studies and expert statements. NET-derived PC concerns about 17% of NET patients and up to 30% of those with small intestine primary NET. It has an independent pejorative prognostic impact. The extent of PC in NET patients and its severity can be expressed by analogy to other malignancies. However, it must be placed in the context of NET disorders, which usually vary from other PC-related malignancies. Recently, a gravity PC score was proposed by a consensus European Neuroendocrine Tumor Society (ENETS) expert group, but it requires validation. In addition, the form of peritoneal involvement (nodular or fusiform/infiltrative) might influence its prognosis and management. Aggressive surgical management seems justified for subsets of NET-related PC but requires careful selection of the candidates most likely to benefit. Cytoreductive surgery prolongs survival, especially when the peritoneal lesions are completely resected. Too little is known about the benefit of hyperthermic intraperitoneal chemotherapy for NET-derived PC, but if it confers an advantage, it would have to be counterbalanced by its high morbidity.
© 2015 S. Karger AG, Basel.